Change of Heart: Yale and the Evolution of Congenital Cardiac Care

Name: Change of Heart: Yale and the Evolution of Congenital Cardiac Care
Uploaded: 2022-06-06T20:27:17.413Z
Duration: 4 h 14 min 38 s
Description: June 3, 2022

June 06, 2022

June 3, 2022

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ID: 7899
To Cite: DCA Citation Guide

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00:03I guess it's being recorded.
00:06I I actually feel kind of
00:07bad interrupting everybody.
00:08It's great to see everybody like
00:10sort of getting to see each other
00:12again and talking, and it's great.
00:13So I want to say thank you very
00:16much to Jen DeSantis and the
00:18Chair's office and Lauren Perry
00:20for helping us put this on.
00:22Really, it could not have
00:23come together without them.
00:30I also want to be sure to recognize
00:32that it's Rob Wilder's birthday
00:34and Despite that he's here.
00:39It's it's, I think, just the vibe
00:41in the room is speaks a lot about
00:43the history of the section and the
00:45history of congenital cardiology here,
00:47because I think what what you
00:49come away with is that this place
00:50has always been kind of a family,
00:52and I think it's stayed that way
00:54and I think we'll we'll feel that
00:56as we go through this history,
00:58part of part of the plan for today
01:00was to talk about the history
01:01of the section in the history of
01:03congenital cardiology here at Yale,
01:05which is a really incredibly rich history.
01:07And then look at where is the field going?
01:10And and what can we expect over the
01:14over the next decade along the way?
01:17Ohh, along the way,
01:19we're going to hear from Mike
01:21Alfieri who's a wonderful gentleman
01:23and a patient of our program who
01:26spent his life in and out of the
01:29program at various points in time.
01:31And it's going to provide us with a
01:33perspective of a patient who's who's been
01:35part of our family for for many many years.
01:38So I think I'm going to jump right in.
01:39Unfortunately, Cliff Bogue,
01:40who's our chair is not able to be here today.
01:44He's at his son's graduation at Oxford, so.
01:48He he recorded a message for us.
01:54I think I'm doing the wrong thing up.
01:55There we go.
01:57So.
01:57I have not yet seen this.
01:59I have no idea what he's going to say so.
03:23Right?
03:26So with that I'm going to just make a couple
03:29of announcements so there's coffee outside.
03:31That'll stay there for anybody
03:33who needs it, including myself.
03:35There's a there are restrooms on
03:38the sort of 2nd floor, so you can
03:40either go out the back door that way,
03:42or out that way and up the stairs.
03:44And there are restrooms back up that way.
03:47I think those are the only
03:49announcements I need to make.
03:50All right, so we're going to start with a
03:53brief history of of congenital cardiology.
03:55Yale and I have to say that it's not
03:58easy to put this together because there
04:00are lots of different sort of sources
04:02and memories and things out there.
04:04And I learned this morning from from
04:06Doctor Kim that there are some people
04:08who I never stumbled across their names.
04:11So it's it's really a fascinating history.
04:13Who has moved in and out of out of
04:15the halls here and and what became
04:18of them and what became of us so.
04:20I'm going to walk through some of that.
04:22I think the theme here that that I
04:24came away with was that we really stand
04:27on the shoulders of giants and that's
04:29really a very cliche thing to say,
04:31but I think as you'll see it,
04:32it really is true.
04:35I don't have any relevant disclosures
04:36about the history of Yale.
04:42So I think people know that
04:45Ruth Whittamore really founded
04:47pediatric cardiology here at Yale.
04:49Ruth was a resident here from 1942 to 1944.
04:53She then went down to Hopkins
04:55where she was Doctor Tausik,
04:57senior Fellow in pediatric cardiology,
05:00and she spent three years there.
05:02She was news to me,
05:04but she took care of the first
05:07child who had the Blaylock.
05:09Like Thomas Shunt and was the sort
05:11of there the postop night and so
05:13this was a quote from her I thought
05:16was really interesting so she
05:17says it was a girl Eileen Saxon.
05:19She came out of surgery with
05:21numerous postoperative complications.
05:23I had to stick needles into both
05:25sides for chest to draw off air
05:27that was compressing her lungs.
05:28We didn't have a way to monitor
05:29the pressure on her lungs,
05:30so I rigged up a way to monitor it
05:32without having to stick her repeatedly.
05:34I was definitely.
05:35It was definitely a Rube Goldberg affair,
05:37but the baby came through it.
05:39As for me,
05:40I fell into pediatric cardiology and
05:42I couldn't get out and I think you
05:44know it really speaks to what life
05:46was like and I think like what life
05:49felt to a lot of us like as first,
05:51second and third year fellows sometimes.
05:54But the last piece of this that
05:55she fell into pediatric cardiology
05:57and couldn't get out.
05:59I think it really is probably true
06:00for most of the people in this room,
06:03and I think you sort of get stuck in
06:04it and love it so. There you have it.
06:09So Ruth came back to New Haven in
06:121947 and really came back to start a
06:15rheumatic heart disease program for
06:17the state and and along the way she
06:19also began taking care of congenital
06:21heart disease patients in the state.
06:23Around 1950 she started having
06:25trainees come to work with her
06:28and it's hard to date exactly
06:30when the fellowship here started,
06:32but it seems to be that that was
06:35the beginning of the fellowship.
06:37She also went on to study the.
06:39Inheritance of congenital heart
06:40disease and Tina Brechner's going
06:41to talk a good bit about this,
06:42so I'm just going to skim over it,
06:44but really,
06:45she was quite a giant in in starting
06:48to think through the inheritance
06:50of of the diseases that we deal
06:52with on a regular basis.
06:54She also was an early adult
06:56congenital heart disease specialist.
06:57And really, you know,
06:58wrote one of the first papers on the
07:00impact of congenital heart disease
07:01on pregnant women, and this is,
07:02you know,
07:03going back quite some time before
07:05adult congenital heart disease was
07:07really thought of as a as a field,
07:08so she was really quite a remarkable woman.
07:13In 1960 Norm Towner was recruited
07:17by Dave Cook who is the chair of
07:19Pediatrics at the time and Norm was
07:22he had trained at the Cardiovascular
07:24Research Institute in San Francisco
07:26which is where Abradolf and Julian
07:28Hoffman were The Who are huge
07:30figures in the field in terms of
07:33the early understandings of cardiac
07:35Physiology and so norm came and
07:38really brought a whole new era to
07:40to the section to the hospital.
07:43And to the care of of patients
07:44with heart disease,
07:45catheterization was a relatively
07:47new and growing area and really was
07:50just about angiography at that time.
07:53And he brought a focus on the
07:55Physiology of congenital heart disease
07:56and in particular the Physiology
07:58of of what we consider congestive
08:00heart failure for congenital
08:01heart disease and really sort of.
08:04Pivoted the section in a lot of ways.
08:09Norm recruited this gentleman.
08:12Mike Berman. Mike had been doing
08:14pediatric cardiology at the NIH and
08:17he was really focused on cardiac
08:19catheterization and he added a lot
08:21to our understanding of the human
08:23dynamics of complex surgeries.
08:25This was one of the papers that
08:28they wrote together about the
08:30mustard operation and about the
08:32identification of obstruction or
08:34blockage between the pulmonary veins
08:36and the and the right ventricle.
08:38And and what that looked like in the
08:41Cath lab and how to understand that
08:42you have to remember this is at a
08:44time when there was no echo, right?
08:46So so you couldn't put the probe
08:48down and sort of look and say,
08:49oh that's where this was all moving
08:51catheters around and trying to
08:53understand where you were by injecting
08:55contrast and measuring pressures.
08:57And Mike went on to invent 2 specific
09:00pediatric specific catheters.
09:01The Berman catheter and the wedge
09:04catheter will talk a little
09:05bit about that later.
09:07So the Physiology and and the Cath lab
09:10really became a huge focus for the program.
09:13And really landmark studies came
09:15out of out of the lab here.
09:17This was a study that showed the
09:19impact of raising blood pressure
09:21on patients with tetralogy.
09:23So they brought patients to the Cath lab,
09:26increased their systemic blood
09:27pressure using phenylephrine and
09:29measured what happened to their
09:31oxygen content and oxygen delivery.
09:33And remember there were no
09:34pulse ox probes that you had to.
09:37Get a blood gas and we had to run
09:38a blood gas and this and that.
09:40And I was amazed at the saturations
09:42of some of the patients that
09:44they brought to the lab.
09:45I have not taken the patient
09:47to the lab with the stat of 55,
09:50but quite remarkable and the other
09:51thing I thought was really interesting.
09:53When you go back through some of these
09:55historical papers is apparently Hippo
09:57was not a thing and you could just
09:59use your patient's initials in the paper,
10:02which I thought was really interesting.
10:05And they also went on to describe what
10:07happens to the ductus arteriosus and
10:10what the impact of the ductus arteriosus
10:13is in coarctation of the aorta.
10:15So this was not known at the time it
10:17was known pathologically, but really,
10:19it was not understood that,
10:21as as the ductus,
10:22which is this normal connection between
10:25the aorta and the pulmonary artery
10:27which is supposed to narrow after birth,
10:30what happens if it narrows and
10:31causes a constriction or a narrowing
10:33in the aorta itself?
10:35And this was a landmark paper that said,
10:37you know,
10:38it's it's this constriction that
10:40results in in postnatal coarctation.
10:42So and again, no echo,
10:44all angiography and hemodynamics
10:46really quite amazing.
10:48And this is stuff that we now like.
10:50This is just what we talk about, right?
10:51It's not we don't question it at all,
10:53and this is this came out of here.
10:58So then in 1970 Bill Hellenbrand
11:01came to Yale as an intern I.
11:04I'm really sad that bill can't
11:06be here today and he is as well,
11:08but Bill is at 0 to Jazzy's son's wedding.
11:12So you know what are you going to do?
11:15So so Bill came as an intern in 1970
11:17and in talking to him it sounds like
11:19he immediately started spending time
11:21in the Cath lab with Mike Berman.
11:23Even as an intern.
11:24And then he went on to become a fellow here.
11:28Between 72 and 73,
11:29and then he went into the army as part
11:31of the Berry Plan and he was at Walter
11:33Reed after one year of fellowship.
11:35As the pediatric cardiologist between
11:381973 and 1975 and had a got, you know,
11:41an incredible education on the ground.
11:44And then he came back here in 75 and he
11:47was allowed to complete his fellowship
11:49and be an attending at the same time so
11:52so he could sit for the boards so it will,
11:56we'll hear a little bit more about.
11:58Till later.
12:00So this was the section in 1976 and there
12:05are remarkable giants in this photograph.
12:09So this is Mike Berman.
12:11This is Norm Towner.
12:13And important people who who don't always
12:17get recognized or in this photo as well.
12:21So this is Alice Rollins,
12:23she was the.
12:26Sort of Cath lab.
12:28Tech extraordinaire from many,
12:30many years and worked with Mike and Norm
12:33on all of the studies that got done and
12:36really was a driving force in the lab.
12:38I unfortunately never had
12:39a chance to work with her.
12:40I had a chance to meet her at a reunion,
12:43but but never worked with her.
12:45This person over here is Scotty,
12:48who ran the office and also was
12:50responsible for getting the manuscripts
12:51out the door for all of these papers.
12:54So she really played an important role
12:56in the development of the section
12:57because without her I don't think.
12:59Anybody would have published anything?
13:00From what I understand.
13:03Up here is Ruth Whittemore.
13:07This is Catherine O'Brien
13:09who was recruited by Ruth.
13:12Here in the middle is Bill Hellenbrand.
13:17And there are a bunch of other people.
13:19This doctor Delois Schenberg,
13:20who is a cardiologist here
13:22for a number of years.
13:23There are some fellows and
13:25some other folks as well.
13:26I am particularly fond of this
13:28gentleman that I don't know who he is.
13:34So really again,
13:35Giants and and this is sort of
13:38where the section came from.
13:41In in the late 1970s,
13:43Charlie Kleinman was recruited by
13:45Doctor Towner and was really a
13:48pioneer in echocardiography and
13:50particularly in fetal echocardiography
13:52and is sort of thought of as the
13:54father of fetal echo and fetal
13:56cardiology and this was the Seminole
13:58paper that was published back
14:00in 1980 talking about the fact
14:03that you could do echocardiograms
14:05in fetuses so again huge huge
14:08landmark things happening here.
14:11That really set the course
14:13for for the field at large.
14:16A little bit later,
14:17George Lister,
14:17who wasn't part of the section
14:19of Cardiology officially,
14:20but essentially was came.
14:22He was started off as a medical student.
14:25George is an unbelievably
14:28smart guy and George is thesis,
14:31which all yell Med students are
14:32required to do really described
14:34the development of measurement
14:36of oxygen consumption.
14:37So he described the system for
14:39the continuous measurement of
14:41oxygen consumption and measuring
14:43carbon dioxide production, I mean.
14:45He was immense student,
14:47so he went on to do his pediatric
14:51residency here and then he went
14:53out to San Francisco to the to the
14:55Cardiovascular Research Institute and
14:57really became interested in critical care,
15:00which was an evolving field at the
15:02time and came back here as the
15:03Director of Pediatric Intensive Care.
15:07George, while he was out in San Francisco,
15:10took his medical school thesis
15:12and working with these two giants,
15:15Julian Hoffman and Abe Rudolph,
15:17actually built.
15:18What he had described and built the
15:201st VO2 machine and this became sort
15:23of the standard way that you measured
15:26oxygen consumption and that allowed us
15:28to really accurately do hemodynamics in
15:31the Cath lab and understand blood flows.
15:34If we can understand how the body
15:36is absorbing oxygen and how much
15:37oxygen in the body is taking in,
15:39we can use oxygen as a way of measuring
15:42flow in in the in the vasculature so
15:45incredibly remarkable and John tells
15:47me that when when George came back.
15:50They sort of jury rigged a
15:52couple of these machines,
15:53one for the animal lab
15:54and one for the Cath lab,
15:55and we're using these machines regularly.
16:00So then we move to the
16:02late 70s and early 80s.
16:04Disco was raging and there were continued
16:07growth and contributions from the section.
16:10So in in the early 1970s was
16:13the prostaglandin study.
16:14So imagine being on call at night and
16:17not being able to start prostaglandin.
16:20So that was the life until
16:22the mid 70s, right?
16:23And so there was a multicenter trial
16:25run out of the out of the Research
16:27Institute in San Francisco that
16:29looked at the use of prostaglandin.
16:31And those patients came to the Cath lab.
16:34Had an angiogram shot that showed they
16:36needed prostacyclin and they had a ductal
16:39dependent lesion and so prostaglandin.
16:41For those of you who don't know is a
16:43medication that we can give that allows
16:45us to maintain the patency of the
16:47ductus and the ductus in these patients.
16:49Allows us to have either blood flow
16:51to the body or blood flow to the
16:54lungs that wouldn't otherwise be
16:55there so we could go to the Cath lab.
16:57Take a picture,
16:58decide that it was a ductal dependent
17:00lesion that would be able to be survived.
17:03If the.
17:04If the.
17:04Doctors were open and then prostaglandin
17:06was started in the Cath lab.
17:08After the angiogram and at that
17:10time the thought was you had to
17:12actually infuse the prostaglandin
17:13at the doctor so you had to have
17:15an arterial catheter and infuse it
17:17right at the duct and then you had
17:19to take a follow up angiogram to
17:20show what happened to the doctors.
17:22So this was a pretty involved study again.
17:25No echo and that was a huge contribution
17:29and really changed the entire field
17:31and the entire outlook for our.
17:34Patients.
17:37Yeah,
17:37it was part of the New England
17:38regional infant cardiac program.
17:40This was a program that gathered
17:42information about infants with
17:43congenital heart disease from
17:45all across the region and really
17:47allowed there to be a database of
17:49information from which understanding
17:51congenital heart disease understanding
17:53of it was developed,
17:55that more recently has kind of
17:57restarted in a way as the New England
18:00Congenital Cardiology Association.
18:02Which we've been an important part.
18:05Collaborations with surgical colleagues
18:07that we'll hear about Doctor Stansell,
18:09Dr Lax doctor Kopp all went
18:12on during this time.
18:13And I forgot to include Doctor
18:14Glenn because I know you're
18:15gonna talk a lot about it.
18:19And the Physiology of congenital heart
18:21disease stayed a major focus as did pre and
18:25post operative management and fetal echo.
18:28In 1988, John opened the exercise lab here.
18:33Pediatric exercise labs were few and
18:35far between and at that point in
18:37time patients were going over to the
18:39adult hospital for their exercise,
18:40stress testing and that was not
18:43felt to be adequate.
18:44This is Rita, who was the exercise
18:48and EKG guru at the time.
18:51This is the first exercise bike
18:53John tells me that that came in
18:55in 1988 and important papers.
18:56Came out of the lab and an understanding
18:59of what the Physiology for for
19:01our patients during exercise were,
19:03and John, I think we should get
19:05this bike for the next.
19:06The next one that looks really cool.
19:11And then Tina Bruckner came as
19:13a cardiology fellow in 1987.
19:15Sorry Tina, I had to do it.
19:21So Tina came in 1987 and and
19:25to her to to hear her tell it,
19:28she she literally stood on the
19:29shoulders of giants as well,
19:31and she encountered Rich Lifton
19:33and Arthur Horwich and they.
19:36Sort of sparked her interest in genetics,
19:39and she went on to become a towering
19:42figure in in cardiac development.
19:45She joined our faculty in 1991,
19:47and she jointly developed the congenital
19:50cardiac genetic service here.
19:52And I think it's really cool
19:54that your lab has a coat of arms.
19:57That's awesome.
20:00I think we need a coat of
20:01arms in this section,
20:03so her work in the early 19
20:06early late 90s really led to
20:09the understanding of left,
20:12right asymmetry and and how that
20:14happens and what the what the role
20:17of Celia is in that development.
20:19And you know,
20:20publishing in nature in 1997 and 19 in 2002,
20:25these were major major papers and the
20:27understanding of cardiac development.
20:30She went on to continue to contribute
20:32in in the genetics of congenital
20:35heart disease here and continues
20:37very active lab and has been our
20:40API and now for several years in
20:42the Cardiac Genomics Consortium
20:44and bringing our understanding
20:45of genetics to the bedside.
20:49So a large cast of characters
20:52ensued overtime. Some of these
20:54people, you'll know. Yeah.
20:59I'll point out a few, so here's
21:03Al he he came in high school.
21:07That's pretty clear.
21:10John Love, who I didn't have
21:11that chance to work with,
21:12but I hear he was a force
21:15Tom Doyle who is Tom here.
21:18I didn't see him OK.
21:23This is Ann Dubin and her husband Rosenthal,
21:25who's first name I can never,
21:27David Rosenthal, and they're both out
21:30in San Francisco and Stanford now.
21:33Tom Doyle again, this is Elkanah
21:36Bruckheimer who's a pediatric
21:37interventional cardiologist in Israel.
21:39This gentleman is in the audience here.
21:42Where are you?
21:44John love, not John, I'm sorry.
21:48Nancy, where are you?
21:57Alice. Lord fight.
22:02Who's sharing a few beers with?
22:04Ruth, it was always a
22:07fun time in this section.
22:09Al came back for his bar mitzvah.
22:13For us
22:19let's see we got Emily, where are you?
22:23Rob's not a graduate,
22:25but we'll we'll take you on.
22:28Tina Christopher George.
22:34I don't know where you all
22:36were, do you remember?
22:41And and Scotty so.
22:46Over time there was this huge
22:48shift in the demographics
22:49of congenital heart disease,
22:50and if you look at what
22:53happened to the population,
22:55the adult patients really began
22:57to take over the practice and
22:59that was because of all of
23:01the successes that were coming
23:02out of the clinical practice.
23:04So in 2004 we started the adult
23:06General Heart disease program here
23:08and with John as its director.
23:12So what began looking like
23:15this in 1967? Or 76 sorry.
23:20Looked like this during the pandemic.
23:24Was a miserable time and now
23:26finally looks like this.
23:28So we all gathered last night for the
23:30graduation of our most recent fellow
23:32grads and had a bunch of you with us,
23:34which was terrific so.
23:37Thank you all for coming.
23:38I'm really excited to move through the day.
23:44All right, so I'm going to introduce Rob,
23:48who's going to introduce Mike.
23:50So Rob elder. I think many,
23:52almost everybody in the room knows,
23:53is the director of our adult
23:55congenital heart disease program.
23:57He's a wonderful person and also
24:00our fellowship program director,
24:02and I'm you don't need any
24:04more introduction than that.
24:12I will adjust the microphone
24:14for Michael and myself.
24:16So good morning everybody.
24:17It's so fun to be here,
24:19what a great turnout both last
24:20night and here and I appreciate
24:22in particular all the alumni that
24:23are here from around the country.
24:25So thank you guys for being here as Jeremy
24:28and I were having coffee at Blue State,
24:30which is where all important meetings happen.
24:32As many people know and
24:34thinking about this symposium,
24:36we thought well it would be important to
24:38think about the patient perspective and
24:40immediately I thought of Mr Mike Cloud Fairy.
24:42So Michael has been a patient of
24:45ours and really a friend of mine.
24:48Since I arrived here and has
24:50been a wonderful addition,
24:52he's been I'm going to let him tell
24:54you his patient experience story,
24:56but from my perspective I know
24:58him as a businessman.
25:00As a successful entrepreneur,
25:01as a philanthropist and as someone
25:03who has really been a champion
25:05for congenital heart disease.
25:06He is the person who is at
25:08all of our heart walks.
25:09He is the person who we had
25:12coffee together and we said,
25:13well,
25:14what do you need for the program
25:15to be successful and to grow?
25:17He sits on the board.
25:18Of the Children's Heart Association
25:22or or the Friends of Pediatric
25:24Cardiology and the Children's Hospital
25:26here at Yale and has always been
25:29a great supporter of our program.
25:31And so it's my honor and privilege to
25:33welcome him up here to tell his story
25:35and his experience being a patient.
25:37So thank you Michael for being here.
25:46Thank you Rob, I appreciate that.
25:49All right, so these are my disclosures.
25:51Jennifer said I had to have this slide.
25:54So we'll bypass that.
25:58As as you know, now my cloud fury and
26:01I'm going to share my perspective in
26:04terms of being a patient here at Yale
26:08and the things that it has meant to me.
26:11I will say that I am extremely flattered
26:14to to be in front of all of you,
26:16and it's an honor to stand here.
26:22I think the Giants that you spoke about are.
26:26I am largely the beneficiary of those giants.
26:29I can say that with no doubt I did not share
26:32my presentation ahead with with Doctor Asnes,
26:35but you'll see some overlap.
26:36Some of the photos will
26:38be a little more recent.
26:39As well, but let me take you back.
26:43To the world in 1963,
26:45some of you might remember the world in 1963.
26:48As I look out here,
26:50many of you would not.
26:54But this was what the
26:55world looked like in 1963.
26:57I won't go through any detail,
26:58but it was that year during the Kennedy
27:02Kennedy administration that I was born.
27:04And it was. This is an old photo.
27:07It was right here at Yale,
27:09New Haven Hospital and it was
27:11called Grace New Haven Hospital.
27:15So that's where life started for me. And.
27:20I had my first lesson early in my life.
27:24It's a word I am certain everyone in this.
27:28Room knows. And word that I came to know
27:32quite well, and I'll put it up here.
27:34This is just out of the peers in the
27:36dictionary as I prepared my remarks,
27:38I looked in the dictionary and you
27:40see the word congenital existing
27:42at birth and this is the example.
27:44They used a malformation of the heart
27:46I'm going to come back to this word.
27:49Later, but it's stuck because I was
27:52diagnosed with tetralogy of the love
27:54that was about three or four months old
27:57and that diagnosis came here at Yale.
28:05And. I learned that there were
28:08some important people in my life
28:10that I didn't know at the time.
28:11Some of them were already mentioned today,
28:14but some of the exact photos that
28:17you'll see Doctor Arthur fellow.
28:20Obviously, the person that came up
28:22with these, I believe it's four,
28:25although sometimes I've heard 54
28:27characteristics of tetralogy of fellow.
28:30Walt Lillehei I I certainly.
28:34Read about and many of you,
28:36I'm certain have heard of his tremendous
28:39advances for cardiac here when he came
28:42up with, and I'll never forget one of
28:44the doctors here at Yale described it to me.
28:46The only procedure that had a 200% mortality
28:50rate because it had crossed circulation.
28:53Initially, before the heart lung machine.
28:57And then of course, Doctor Helen,
28:59Brooke, Taussig, who is the mother of.
29:02See now of pediatric cardiology.
29:07As she teamed up with.
29:09Doctor Blaylock and Vivian Thomas
29:12for the operation that was already
29:15mentioned down at Johns Hopkins.
29:18What I found interesting about this photo
29:20is some of the folks that were in the photo.
29:22I don't know if anyone has seen
29:26this photo before and identified,
29:29but I'll use the pointer here.
29:32You probably know the positions
29:33of the doctors.
29:34Doctor Blaylock standing over the patient.
29:36This is Vivian Thomas, who at the time.
29:40Is not allowed to touch the patients
29:43because he was a black man and was then
29:47honored by Johns Hopkins later in his life.
29:51And then I found it interesting across
29:53from the patient as Doctor Denton Cooley.
29:56Who am I understanding was one of the
29:59residents or fellows I should say of
30:02Doctor Blaylock and in this photo,
30:05doctor Tausik could not be identified
30:09as one of the women because she
30:11always wore glasses.
30:12So it's suspected she's this person
30:16that was walking to the side of the.
30:20Operating area.
30:22But those names were not as important
30:26as those that I did come to know,
30:29and those that I came to know.
30:31Were were mentioned and they truly were
30:34giants and these are giants that touched me.
30:38These were my doctors,
30:40Doctor Ruth Whittemore,
30:41who you already know came as one of the
30:46proteges of those folks that were down
30:48in Baltimore and then Doctor Marie Brown.
30:51She actually I had a different photo.
30:54She was on the right hand
30:56side of Doctor Asnis photo,
30:57but I circled her here and
31:00you'll see next to her is.
31:04Ruth Whittamore I think she had this same
31:06hairstyle the entire time she was here.
31:10But Doctor Brown was instrumental
31:12in my health and my care.
31:14She saw me as a patient for over 2 decades
31:17from my birth until I was about 21 years old.
31:21And, you know,
31:23I really admired everything that she
31:26did for for me and for patients.
31:30I was actually in touch with her later
31:32in her life before she passed away,
31:34and it was very touching to be
31:36in a in in touch with the doctor
31:38that late in their life.
31:40In 2006, when I reached out to her
31:44and recalled our relationship from
31:47decades before and then finally.
31:52Doctor Horace stands.
31:53All this is actually a newspaper
31:55clipping that my parents obviously
31:57took out of the books called the
31:59New Haven Register back then,
32:01but Doctor Horace Stanzel,
32:03who did join the team with
32:07William Glenn at the time and
32:11helped lead the pediatric.
32:14Cardiology surgical staff at the time.
32:19And I'll take you to the summer of 1969.
32:23Still a lot of you don't remember
32:25that summer, but the summer of 69,
32:27I always think there was two
32:29events that people recognize.
32:30I'll just put the important one up here.
32:32Me and land landed on the moon
32:34and I share that to under score
32:37the gravity of where I was,
32:39where the world was,
32:41where medicine was in 1969,
32:43but it was in the summer of 1969.
32:47Shortly just three weeks after that.
32:51At this little boy.
32:53Here had his heart repaired and I know the
32:57term now is repaired by Doctor Stanzel here.
33:03Umm?
33:04The other thing that Doctor Stanzel
33:06had done prior to my full repair
33:09was a BT shunt and I remember
33:11coming back here later in life.
33:14I guess at the time was a BT shunt and I
33:16mentioned to doctor Asnis when he came
33:18back a few years later it was called.
33:20Many years later.
33:21I should say a classic BT shunt.
33:24So then I felt like an old GT Pontiac.
33:28So boy,
33:28the world has changed.
33:29When you hear that as a patient.
33:34And that was. You know,
33:36in 1969 I did go through that 70s and
33:3980s period that was mentioned, but.
33:42Pediatric cardiology was
33:44very different back then.
33:47It was annual visits and then a
33:50promotion to every other year.
33:52So it came and I walked those halls
33:55here at Yale and there was little
33:57yellow lines had to follow to get
33:59up to the pediatric department and
34:01I finally graduated to every two
34:04years and then finally as a send
34:07off in 1984 when I was 21 years old.
34:10I had a cardiac Cath and I met a young
34:12fellow sitting right here and the
34:14cardiology group and I still remember it.
34:16To this day,
34:17as he was leaning over my
34:19groin to stop the bleeding.
34:23So you know my hats off to you, John.
34:25In terms of the relationship
34:27that we have had as well.
34:32But as I said. It was a time.
34:36That was very different.
34:37You know you brought a note to every
34:40physical education teacher that
34:41you had to kind of get out of gym,
34:43which you know maybe it was
34:44a good in some regards.
34:46It was caution coupled with Wari it
34:49couldn't be in competitive leagues.
34:52You know very different than the
34:54things that you today tell patients.
34:57I'm certain is very very different based
34:59on what I now know and what you now know.
35:02But I then became what I now know today.
35:07Lost to care.
35:09I was an adult.
35:11I was carefree and I did not see
35:16a cardiologist for many years
35:18after my 21st birthday and after
35:22meeting Doctor Fahey.
35:27And in that time I won't
35:28bore you with my lifetime.
35:32I did a lot before.
35:34Reemerging at the adult adult
35:37Congenital heart program.
35:38I got graduated from college
35:40actually twice I married only once.
35:45I had a boy I had a girl there they are
35:48Michael and Jocelyn and my daughter's
35:51name is Jocelyn Brooke out here.
35:55After doctor. Towns it.
35:59They aren't together.
36:00I bought a farm which is another story,
36:01but we won't go into that here.
36:05You know I went to Disney.
36:08Doesn't everyone do that?
36:09And they learned to ski as an adult.
36:12And I still remember
36:14don't go out in the cold.
36:16It could be bad for you.
36:18So that was very,
36:20very important to me.
36:21As an adult that I got to do,
36:22some of these things.
36:25You know my family life was great.
36:27I I could not say enough.
36:30About
36:33you know the cure that I had and what
36:35it you know gave me the ability to do,
36:38and really, because of those giants
36:40because of the backs of those folks
36:43that you come on come behind.
36:48But then it was time to
36:50come back to cardiology.
36:52And I call this cardiology take 2.
36:56I've seen here and at the time.
37:01Say he was here. Doctor Perry I I
37:06have him as the production director.
37:08Here was the I I think at
37:10that point was the director of
37:13the Adult Congenital program,
37:14Nancy I first met this evening.
37:17It was almost 16 years of the day.
37:19It was on June 6, June 9th, 2006.
37:25I entered the Children's Hospital
37:27through the ear through the ER,
37:29and I believe my my pulse was 44 and
37:34they asked are you a runner and I said no.
37:37I just feel tired.
37:41So I learned a lot since that point
37:44in time and obviously have had a lot
37:46of here since that point in time,
37:48diagnosed with radial cardia
37:50and complete heart block,
37:52I came through the ER as I mentioned,
37:54they admitted me immediately.
37:57And I had a pacemaker place.
38:01Now this is my perspective of of a pacemaker.
38:04I did travel a tremendous amount.
38:07In my career, an airport nearly every
38:12day at times, certainly every week,
38:15but these are the things you get asked.
38:17You do get to go to the head
38:18of the line if you say,
38:18have a pacemaker of that little
38:20card that Medtronic gives you.
38:22But my favorite response
38:23to the questions they ask,
38:24which is always do you need a private screen?
38:26Do you have any sensitive areas
38:27and can I use any the back of my
38:29hand and they always say to them
38:31I don't need a private screen.
38:32I have no sensitive areas and you could
38:34use any side of your hand that you'd like.
38:37So that's my perspective of the pacemaker.
38:44Shortly after that placing of the pacemaker,
38:47I received a call actually was from the ANC.
38:50She couldn't remember placing.
38:51It was on my birthday.
38:52As I said, was August 1st and she
38:55said Michael they can do this medical
38:59procedure that they had a cancellation.
39:01I was actually having dinner with my wife,
39:03but to come to the hospital on August 3rd,
39:072006, I came to the hospital.
39:10And I think she may have been said.
39:13Just pull into the little circular area.
39:16They have valet parking.
39:18So you know you're nervous.
39:21I drove the car myself.
39:22I said my wife said you need to drive.
39:24I said what do I need to drive?
39:25I said no, I can drive so I pull
39:27into the circle at the Children's
39:30Hospital and the valet immediately
39:31said it looks me directly in the eyes.
39:34Look at the window down.
39:35He's 18 inches away.
39:36Get the baby out of the car
39:38and head up to admitting.
39:39And I said you're looking at the base.
39:42Yeah,
39:42so I got out of the car and I had
39:45it up to to admitting and that day
39:49Doctor Koff and Doctor Mello performed
39:51what I now know is called an arvat
39:54procedure to you know further correct
39:56some of the malformations that have
39:58built up in my heart and hoping some
40:02some narrowing and they actually saved
40:06the valve which I'll get to in a minute.
40:10But you know,
40:11I still remember meeting Doctor
40:15Cough in 1984 at that cap because
40:18he had actually come in and gave
40:22me some discussion about possibly
40:24needing a procedure in the future.
40:27And, you know,
40:28at that point,
40:29those 20 odd years later,
40:32I couldn't believe he would be the
40:34man standing and doing this procedure
40:36on me right back here at Yale.
40:39So another great.
40:40Saw Doctor Koss name there and
40:42Doctor Mello I understand is back
40:45in Connecticut somewhere but he
40:47came down for the procedure as well.
40:49The other thing is I had to
40:51start blood thinners.
40:52I think blood thinners have
40:53changed over the year.
40:54But blood thinners, as a patient,
40:56are a little different as well.
40:57You really get to know your lab technician
41:00very well in terms of INR levels,
41:02you never have to worry about
41:05finishing your spinach.
41:06But I know that has changed with
41:09some of the more recent developments
41:11on blood thinners.
41:13And, you know, I had continued here.
41:16I do have a new appreciation for
41:18bovines because I came back several
41:20years later and doctor Asnis and
41:21Doctor Hellenbrand placed one of
41:24these valves in the pulmonary position
41:27through a transcatheter procedure as well,
41:30so those are just, you know,
41:32some of the things that I've had here,
41:34as I saw some of the reunions going on.
41:36With some of the other doctors
41:39that are here today,
41:40you know there was a number of
41:44other procedures that I've had.
41:46You know each of them.
41:47You know.
41:48I clearly remember,
41:49you know the some of the commentary
41:51that you know I've learned,
41:53and I will tell you as you go through this,
41:56you realize you're going to have this.
42:00You know condition for the for your life.
42:02You do learn about it.
42:04So I think one of the things that you
42:06will see is as you as you transition from
42:09pediatric patients to adult patients.
42:11The real learning that happens
42:13because you're really facilitating the
42:15parents in those early years to their
42:18understanding and the need for tear.
42:20As I said, I had that big gap,
42:22but the learning that I have
42:24to now today is tremendous.
42:25And it was a little bit of catch up.
42:27Hopefully people don't have to have that.
42:30In the future.
42:34Even I'll mention this less to do with
42:37the perspective in terms of bedside,
42:40but even my interest in reading
42:43habits have changed over the years.
42:45These are some of the things that I now read.
42:47I remember the doctor, Katie said,
42:50make the doctor, Katie,
42:51the head of I think it's ahead of the
42:54surgery or pediatric surgery here,
42:55he said Mike, you know,
42:57what are you reading?
42:58I said I'm reading a John Grisham novel,
43:00he said as a surgeon.
43:01I always read about surgeon.
43:04So I read the knife man,
43:06which just was an amazing historical
43:08perspective of how surgery developed
43:11and those very early years.
43:13I was kind of spellbound.
43:16And I read this and you hear a surgeon say,
43:19you know,
43:20do surgeon surgery all day and then
43:22I go home and read about it actually
43:24met him a couple weeks ago at another.
43:29Another session or meeting
43:31affiliated with the hospital.
43:32Of course, he gave me more
43:34reading lists to to take care of,
43:36and I put some of the highlights up here.
43:37One of the books that I just found
43:40very interesting was healing hearts.
43:42It's by a cardiothoracic
43:45surgeon that's a woman,
43:47and her name is Kathy Migliaro.
43:49I believe she still practices on the
43:51West Coast and really talked about her
43:55passion in terms of helping cardiac.
43:58Patients and I was overwhelmed by
44:01the amount of passion that she put
44:04into the book she talked about.
44:05I think it's called the Greenfield
44:07filter or something like that.
44:09Went up to me Doctor Greenfield in Michigan,
44:11but to see that passion by someone
44:14and how they practiced it every
44:17day was absolutely amazing.
44:20I did read King of Hearts.
44:22Some of you are many of you may
44:25know that is the story about
44:27about Doctor Walt Lillehei and.
44:29To me it was,
44:30you know,
44:30the most intriguing thing to know
44:34that I have been through.
44:36Some of these heart surgeries
44:37and to see what he had done for
44:40the field of heart surgery was
44:42absolutely absolutely amazing.
44:44And one other that I received
44:46from Doctor Elder,
44:47he gave me this book and I
44:50read it cover to cover.
44:51I think I read it in one day
44:53because I found it so compelling.
44:54And thank you Rob for giving it to me.
44:57But Doctor Denton Cooley who wrote 1100
45:00thousand hearts and it's really about
45:03the 100,000 patients and the 100,000
45:05hearts that he touched as a surgery
45:08and of obviously say Denton Cooley.
45:10Everyone knows that name.
45:12But as I read the book and
45:14the perspective I had,
45:15this was the one line that stuck
45:17with me and the entire book is
45:20on page 163 and I want to sound
45:23like citation English teacher.
45:24But I'll read it because I think
45:27it's that important for each of
45:29you to understand what it means to
45:32the patient that is being touched.
45:34At the same time that I touched their hearts.
45:37They also touched mine.
45:41I found that so so compelling.
45:46The last thing in terms of our
45:48reading habits had to do with kind of
45:51staying up on all these acronyms that
45:53I use every time I come to clinic.
45:55It's amazing, you know you.
45:59I I think you all talk in acronyms and you
46:01know know what each other is talking about.
46:03But as a patient and doctor
46:04Elder knows this about me,
46:05I tried to keep pace to some degree
46:09so I did reach out to. I did read.
46:12I don't know if I've ever said
46:15this to the doctors that know me.
46:17I don't think I had,
46:18but they always said it home, Mike,
46:20you seem to know a little bit about this.
46:21I did find out that the father of adult
46:24congenital heart disease was Doctor
46:26Joseph Perloff, so I bought his book.
46:29Now this one I could not read in one day
46:31because I'm not as smart as all of you,
46:34but I took my time and I read
46:37through it and it just.
46:39Again,
46:39it was compelling to see someone
46:42an individual developed this whole
46:45entire practice around the disease
46:48that I had and helping people like me.
46:53So I couldn't help myself and I wrote
46:56to Doctor Perloff and I just wrote
46:59to thank him for what he's done for.
47:02This profession and he got back
47:04to me right away. I put up here.
47:09His quote and I'm just going to read the.
47:14The part in green here that he mentioned
47:17back to me once and it's really about
47:19this field of adult congenital heart disease.
47:23Once the truth is known,
47:24the fool sees it,
47:26but the early days of development
47:29of congenital heart disease and
47:31adults were a struggle.
47:33And that just stuck out to
47:35me that this person.
47:38And in his career has affected
47:40so many so many people.
47:42I mean,
47:43hear the numbers today of millions
47:46of adult congenital heart patients.
47:49So there was, I thought,
47:50a very touching message in terms of.
47:56His message immediately back to me.
47:58Of course,
47:59he gave me a little more reading to do,
48:01read the 6th edition Michael
48:03and you'll find out about some
48:06of the historical background.
48:08But I mentioned that in terms
48:10of these habits,
48:11you know it wasn't really
48:13reading that changed in my life.
48:14But my perspective on life changed
48:17because of the relationship I have and
48:20the care that I had right here at Yale.
48:27These are my latest friends and true heroes.
48:32I'm going to actually show more recent
48:34pictures, but each of these folks
48:36have had a hand in touching my heart.
48:39Some of them truly physically Doctor Fahey,
48:43who I'm first met in 1984.
48:45Doctor Jim Perry,
48:46who I mentioned was that the director
48:49of the Adult Congenital program.
48:51Nancy Rollinson,
48:52who I personally want to give
48:54another round of applause for.
49:05And then Doctor Koff and Doctor
49:08Mello I mentioned did my I guess.
49:11Third open heart surgery.
49:14And then the Doctor Elder,
49:15who have become very,
49:17very well acquainted with over the years.
49:19Doctor Fish Burger, who is now,
49:22I think down to Miami,
49:23although used to be here in Miami,
49:25used to ask them how the
49:27how's that flight situation.
49:28I know he did.
49:30That doctor, doctor Marieb who.
49:35I couldn't believe how much
49:36he writes on his scrubs,
49:38but I believe it's now elsewhere and
49:41taught me a little bit about Figure 8
49:44circuits and things like that Doctor Gruber.
49:48And then Doctor Hillenbrand,
49:52doctor asnes and Jacoby.
49:54I think he's not here at Yale anymore,
49:58but Doctor Jacobi, Dr Beach,
50:00and Doctor Vinikour,
50:02all of you truly have touched my heart
50:06in a fashion that it will never forget.
50:09And truly, our heroes,
50:11I remember, I think,
50:13was the last clinic I came to.
50:15And I was outside walking to my
50:20appointment and doctor Alder passed me
50:22and the on the street in front of the.
50:25The parking lot and I looked
50:27over my shoulder and it said and
50:30it still says heroes work here.
50:32And I I know that was put up
50:36more recently for COVID and.
50:38You know to really shed light on what
50:41you all have done during this pandemic,
50:44but it hit me that there's always been
50:48heroes in these buildings and you know,
50:51that wasn't something that was new.
50:54So so to me.
50:56You know that notion of heroes working
50:59here has been here since August 1st, 1963,
51:02when it was Grace New Haven Hospital.
51:05We can't say enough.
51:09And in terms of.
51:13Recognition that.
51:17This hospital has given me so much.
51:19Rob has mentioned this.
51:20There is no better gift than
51:22paying it forward and giving back.
51:25I've had you know such a long relationship
51:28with the hospital and I have only been
51:31able to give back a very small portion
51:34of what you have all given to me.
51:37So I am part my wife and I were founding
51:39members of the Children's Hospital Council,
51:42which is the philanthropic.
51:45Engine of the hospital.
51:46There are many other
51:48hearings of philanthropy,
51:49but my wife and I joined as founding
51:51members and worked with some of
51:53the senior leadership over the
51:55years on that Council in terms of
51:58developing philanthropic endeavors,
52:00my heart is more closely aligned
52:04to the adult and pediatric.
52:07Entities and most of my donations
52:10have gone towards that.
52:13They featured us in a giving
52:15from the heart not too long ago.
52:19Which you know was unbeknown,
52:21really.
52:22I did not know that I would be
52:24featured in terms of giving.
52:25It is truly something that is a
52:27privilege and I don't look for recognition,
52:29but they actually presented us
52:31with the the magazine that was
52:34published and the article that
52:36was published about my life.
52:37And I you know,
52:39I found that again just rewarding
52:41in terms of the recognition
52:43that came with that recognition
52:45that I was not looking for.
52:46I'm looking to recognize all of you.
52:49Up there.
52:52And I'm going to just mention you
52:53know two other things.
52:54As I conclude my remarks here and
52:57I'll go back to the lesson in
52:59Latin that you have taught me,
53:02because I think the definition of congenital,
53:05a congested US or congenital as
53:07we know it has changed for me.
53:10And this is the same word.
53:12It's still an adjective,
53:14but existing at birth has changed
53:17from me to existing throughout life.
53:21And I think that.
53:23Is solely because I had a congenital
53:27yellow haven heart entirely.
53:33And one final message, I'll leave you with.
53:37It's something that I recognized.
53:39You do every day and I think it's all
53:41the professionals that technicians,
53:43the nurses, the doctors,
53:46everyone here at Yale.
53:48Particularly the folks that
53:50that I know in this section and.
53:55You know it's a lesson about.
53:59What you do,
54:00and you should recognize you do every day.
54:03And I truly believe that you have the
54:07ability to change the trajectory of
54:11someone's life every day you come to work.
54:15And I thank you for that.
54:17I'm certainly happy to answer any questions.
54:40Thank you so much that that was
54:42really just a a tremendous talk.
54:44I really appreciate it.
54:48Hard to follow. So if there's
54:51anybody who can follow it.
54:56So I'm happy to introduce
54:58and reintroduce Rick Kim,
55:00who many of you know,
55:02so Rick did his thoracic surgery
55:05fellowship at Penn was a pediatric
55:08cardiac surgery fellow at Chop
55:10and returned to Yale,
55:12where he had done surgery
55:14back when did you come back?
55:202007 so we overlapped for three years
55:23and then left us to head out to LA
55:27where he was at CHL A for a number
55:29of years and then recently took over
55:31as the director of the Warren Family
55:33General Heart Center at Cedar Sinai,
55:35where he's the also the chief
55:37of pediatric cardiac surgery.
55:39So Rick's going to talk to us about the the
55:42history of congenital heart surgery at Yale,
55:45and again I think a perspective that's
55:47really a telling about this. Nice.
55:57Great it it's it's actually
55:59wonderful to be back.
56:00It's it's very difficult
56:02to follow that that talk.
56:04Michael's talk was was pretty amazing
56:06and and I've just been thrilled already
56:08in the in the short time that I've been back,
56:11you know they always say it's it's.
56:14It's the same, but it's not the same.
56:19Let me let me go back for
56:21just just for a minute.
56:22You know I'm going to talk a lot
56:24about about Doctor Glen and and
56:26it's great that Michael put some
56:28of the pictures up and I think I
56:30can actually fill in a little bit.
56:32Kind of some of the details
56:34surrounding some of those pictures.
56:36I I just wanna say first though
56:40that the that the.
56:43I mean the when when I think about
56:46congenital heart surgery at Yale.
56:48Actually I I think about first
56:51doctor Gary Cough. I don't.
56:53I don't know the Garry's here, but.
56:58You know,
56:59I first met Gary in the late 1990s
57:03when I was a general surgery resident,
57:06and Gary and Johnny were like
57:09the kings of the hospital.
57:12They they're like they're so cool,
57:15and but, but I was a I was a third year.
57:18I would never.
57:19I would never kind of dare to talk to
57:22to Gary and but I was my my I was a
57:26third year general surgery resident and.
57:29I was in the in the ICU taking
57:33call at night and you know,
57:35as they do a heart a cardiac patient
57:38comes back and starts exsanguinating.
57:42And and I have no idea, right?
57:45But but I, I was a very good student,
57:48as as all as all Koreans in particular,
57:52so so I had read a lot of
57:55the books and and the book.
57:57For some reason that had
57:58really stuck my mind.
57:59Was this little white book and
58:02there's this picture of how you do
58:05a reduced anatomy and and all they
58:07did is they opened the very bottom.
58:10They just took out one wire.
58:12And they put the retractor in,
58:13and it was so perfect and beautiful,
58:16and so here I am.
58:17And and I never even seen a chest close
58:21before, and I just remember this.
58:23This picture is so indeed and perfect.
58:25And so I got OK, I know what to do I.
58:30OK, I opened this little incision.
58:33And of course, you can't see anything,
58:35and so I put the retractor and and
58:37I tore the whole sternum apart.
58:39It's like bleeding everywhere.
58:41I don't Gary comes in.
58:43He doesn't know who I am,
58:45but but Gary comes in and stops the bleeding,
58:49puts everything back and he hasn't
58:51spoken a word to me this entire time.
58:53Not one, not not anything.
58:57And then finally,
58:58he's about to walk out the door
59:01again and Jerry goes he goes.
59:05Next time,
59:05just take out all the wires that
59:08was it and I go.
59:09I go this guy's like the coolest,
59:11the coolest person and so.
59:14And so I went off to.
59:15I went off to Philadelphia and and
59:18when I came back as an attending.
59:21You know Gary was the guy Gary.
59:26You know when you when you first
59:27come out you think you know but
59:29but as they always like to say,
59:30you don't know what you don't know
59:32and the the wonderful thing about
59:34Gary is that he would always make
59:37me feel like I knew what I was
59:39doing when of course I didn't.
59:42And and and Gary was the guy
59:45who really kind of got me over
59:47those those first few few things.
59:49Actually it was a great time.
59:51Jeremy was there,
59:52Tina al everyone and and and you know,
59:57I would come out of the OR and and
59:59I would have these big residual
01:00:00VC's would be like don't worry
01:00:02about that would be fine.
01:00:04I'd like I'd like have a you know a
01:00:08you know branch PA something Jeremy.
01:00:11Don't worry don't worry so it was.
01:00:14It was a really great time. OK.
01:00:15Anyway I'm going to talk about.
01:00:17I just have to say that about
01:00:19Gary because I haven't seen him.
01:00:20And and. And he he really,
01:00:22he really meant a lot to me.
01:00:25Umm?
01:00:27So I'm going to talk.
01:00:28Actually, instead of what?
01:00:29What Jeremy did and talk about everybody.
01:00:31I really want to talk about about
01:00:34Doctor Glenn because Glenn is really
01:00:37kind of the the spirit of of of
01:00:40how Yale cardiac surgery develops.
01:00:42Umm? And. The Glen that we see
01:00:48today and this this is one of mine
01:00:50is is of course very different now
01:00:52it's it's like a staging operation.
01:00:54You know we use it really commonly
01:00:56for single reason.
01:00:57This is at children's with John John
01:00:59Wood who I worked with for a decade or
01:01:03more actually and we think of it as
01:01:05it as a very straightforward operation.
01:01:07It's a bidirectional operation and
01:01:10of course the operation that Glenn
01:01:12is known for is very different.
01:01:15It was a. It was a classic.
01:01:17Granny classic Glenn and and it's
01:01:21a unilateral glend to to one side
01:01:24and and of course it was done for.
01:01:27For very different things, right?
01:01:29Yes, it was done for tricuspid
01:01:31atresia and things like that,
01:01:32but it's also done for tech.
01:01:34PS You know it's done for
01:01:36corrected transposition.
01:01:37It's done for Epstein's and and the
01:01:40first time they did it clinically
01:01:42was in 1958.
01:01:43But he had been thinking about
01:01:46it since 1948 and he had been
01:01:48working on it through the lab.
01:01:49And of course he.
01:01:50He did it for a single VPS's like the
01:01:53perfect patient to do it on the the.
01:01:56The thing I love about this.
01:01:58Initial description here though
01:01:59is is is when you look at it,
01:02:03you immediately know why a classic
01:02:06lennis to one side is because you had,
01:02:10you know there's no cardio pulmonary
01:02:12bypass support for for us,
01:02:13it's easy because we're draining the
01:02:15head and we're kind of bypassing
01:02:17that whole area,
01:02:18but he had to put a clamp on it so
01:02:20you know you you put a clamp on the
01:02:22SVC and a synodic patient that that
01:02:25patients headsets are going to go to 0.
01:02:28So the other the other reason
01:02:30why I love this is that it.
01:02:32There's the the technique involved so.
01:02:36If you ask most.
01:02:39Pediatric heart surgeons in the world today,
01:02:43they said,
01:02:43how do you technically do your Glen
01:02:45and they say, oh, I just run it?
01:02:47No problem.
01:02:48I just I just run run means you
01:02:50just do a continuous suture over and
01:02:52over around the new to S Madonna.
01:02:54That's so so The thing is is what
01:02:59I what I found, at least for me,
01:03:01is that if I was truly,
01:03:02truly honest with myself when those
01:03:05patients came back for their Prefontaine cat.
01:03:09Two more,
01:03:10often more often than you'd like, you see,
01:03:13like a little narrowing and and the
01:03:15cardiologists of course be very nice to you,
01:03:17and they'll be like, oh,
01:03:18don't worry about it, you know,
01:03:19do dilate at the time or do something,
01:03:21but you you have like this little
01:03:23tiny pinch in there.
01:03:25And I said, well.
01:03:27You know,
01:03:28I so so we tried everything.
01:03:30We kind of like pull it open we're
01:03:32very cognizant we're trying not to.
01:03:34We call this a purse string.
01:03:37And I said, you know something so about
01:03:393-4 years ago I said I'm going to run the
01:03:42back and interrupt the front and and when
01:03:45you run the back and you interrupt the front.
01:03:48Like it essentially takes it away,
01:03:50and so you you get a really kind of
01:03:53nice anastomosis and then and then.
01:03:55Sure enough I look at this and
01:03:58so this is in 1958.
01:03:59This is only 60 years ago.
01:04:02Glenn runs the back and interrupts the front.
01:04:06So so it took me took me a long time,
01:04:09but but you know,
01:04:11I've kind of I've kind of gone back to that.
01:04:14OK here. Here's the other major
01:04:16thing that he's known for.
01:04:17And really, this is a you know he.
01:04:20He worked with a Yale medical student
01:04:22on his on his graduation thesis,
01:04:24which was the.
01:04:25This it everybody calls it a pump,
01:04:27but really it was a total artificial heart.
01:04:29It was a.
01:04:31It was famously modeled on
01:04:34an erector set and and. Umm?
01:04:39There's no oxygenator for it
01:04:40and and Glenn never used it.
01:04:42There's only one,
01:04:43actually a commercial model ever made.
01:04:46Never used on a human.
01:04:47They did a coronary blood flow study on it.
01:04:51But it's it's important to note that
01:04:54when Debakey put in the first bed,
01:04:56actually he used a bad.
01:04:58That was a ventricular assist device.
01:05:00It was exactly the same kind
01:05:03of model that that Glen used.
01:05:08But when you think about those two things,
01:05:13those two accomplishments that that
01:05:16everybody talks about. It doesn't.
01:05:19It doesn't really tell the story of Glen,
01:05:22you know, it tells two things and what
01:05:25I found really interesting about when
01:05:27I started to kind of put this whole
01:05:30thing together is is when I think about
01:05:33the time and I think about the person.
01:05:35I had this one image in my mind of
01:05:38of who Glenn was and then once it
01:05:41started learning about Glenn I I
01:05:43realized I had it completely wrong.
01:05:45And that's it.
01:05:46The image in my mind of Glenn
01:05:49was was not the same person who
01:05:51really started the department here.
01:05:53And the big lesson to me was is
01:05:57those characteristics that Glen had,
01:05:59and that that he started this department
01:06:03with those characteristics are like
01:06:05a the the the paradigm of charisma.
01:06:08And they're the they're those
01:06:10important characteristics that you
01:06:12would look for in a leader.
01:06:14And they're obviously just as relevant.
01:06:16Today, as they were back then.
01:06:18So, So what was the origin
01:06:21story of the department so?
01:06:24So Glen.
01:06:26When he was asked to come back to Yale,
01:06:29this is this is 1948,
01:06:31so he he had already finished his
01:06:34his training at at the MGH and
01:06:37he was back in in Philadelphia
01:06:39and Philadelphia at Jefferson,
01:06:42actually working with with John Gibbon,
01:06:44I guess they called him Jack Gibbon,
01:06:45who who famously,
01:06:46you know,
01:06:47developed that was one of the developers
01:06:50of the cardio pulmonary bypass circuit.
01:06:53But.
01:06:54So so Glenn agrees to come back and and
01:06:58or come here in 1948 and he immediately
01:07:02walks into like a big problem.
01:07:04And the big problem.
01:07:06It's actually 2 problems is not
01:07:09starting pediatric heart surgery.
01:07:13Because contrary to what?
01:07:16What people believed pediatric heart
01:07:18surgery 1948 New Haven was the the
01:07:22place to be it it had already it.
01:07:27It was at the very forefront.
01:07:29They were doing closed heart
01:07:31operations for pulmonary stenosis.
01:07:33They were doing blailock operations.
01:07:37They were doing, you know,
01:07:39coarctation they were doing PDA.
01:07:41Everything that. You could you could do.
01:07:44They were already doing here.
01:07:46Ruth Whittemore was already here
01:07:48and she had a vibrant practice.
01:07:51I was talking earlier with Jeremy.
01:07:53There's a, you know,
01:07:54one of the fathers of pediatric
01:07:56cardiac catheterization.
01:07:57Paul Lurie was here and and Paul
01:08:00Laurie actually went out to to CHL
01:08:03and was there for for many many years.
01:08:06So the problem was not starting the
01:08:08program the the problem when he got
01:08:11here and the two major problems one
01:08:13is is that the person who started
01:08:16this guy named Harry Schumacher.
01:08:18Harry Schumacher had just left and
01:08:21Harry Schumacher was one of the only
01:08:24people and I'll get to him in a second.
01:08:27Who really knew how to do or had any
01:08:30experience to any of these operations.
01:08:31And then the second major
01:08:33problem that that Glenn had is.
01:08:35Glenn had absolutely zero training
01:08:38in cardiac surgery like none
01:08:41like Nada. He he he,
01:08:44he hadn't done anything.
01:08:46And and so, so the story of of of
01:08:49Harry Schumacher is is that Harry
01:08:52was with Blaylock with with denting
01:08:55Kuli and with another kind of West
01:08:58Coast surgery named Bill Longmire.
01:09:03And he had been there and and but
01:09:06Harry wanted to kind of start his
01:09:10own thing and and so Harry had come
01:09:13up to to Yale to start the program.
01:09:17And he started the program
01:09:18and he was in two years.
01:09:20He was already really successful and.
01:09:25But as it is the those kind
01:09:28of people are prone to to be.
01:09:31Harry wanted to be chair,
01:09:34and if you know anything about
01:09:37the history of of Yale surgery,
01:09:41Harry didn't become chair.
01:09:42A guy named Gus Linscott became
01:09:46chair and so and so Harry left any
01:09:51and he left and he started Indiana.
01:09:55And so if you can just imagine the
01:10:00situation that Glenn walks into.
01:10:03So the first thing he's got to face is
01:10:06he's got to face the patients you know.
01:10:09So unlike it is today where there's there's,
01:10:13you know more than 100 places where
01:10:15you can get pediatric heart surgery.
01:10:17In those days, there were very few.
01:10:19There are only actually the
01:10:21places that were all descendants
01:10:23of the Blaylock trainees.
01:10:25And on top of that,
01:10:26you know as as we learned from from Ruth
01:10:30Whittemore and Tina Adult or congenital
01:10:32heart disease is the most common
01:10:35form of congenital disease out there.
01:10:38But there's never been any surgery,
01:10:40so there's this huge backlog of patients.
01:10:43And after the Blaylock operation,
01:10:46within six weeks,
01:10:47you know congenital heart
01:10:49surgery became the OR.
01:10:51Hopkins became the center of the universe.
01:10:55For congenital heart patients,
01:10:56and Yale became one of those places also,
01:10:59and so so there's a huge volume of patients
01:11:02here and so and so so you can only
01:11:05imagine that Glenn's walking in there.
01:11:08And he's got to talk to these
01:11:10families of desperately ill patients,
01:11:13and there's tons of them around in New Haven.
01:11:16And he,
01:11:17he's got a he's got to face them.
01:11:19The other thing he's got to face
01:11:21is he's got to face Ruth Whitmore.
01:11:23And and if you read the things
01:11:26about about her,
01:11:27you know she's she's like a
01:11:29lovely woman and she's completely
01:11:31dedicated to her patients.
01:11:33But she is like no shrinking Violet.
01:11:35She is like a a really.
01:11:38Like determined like powerful woman.
01:11:41And actually there's a great story
01:11:45about denting Cooley and and about
01:11:48how denting Cooley got his his
01:11:52residency position at at at Hopkins.
01:11:55I'll bring it up because because
01:11:58Michael brought it up so.
01:12:00So I don't know if any of you
01:12:01have to have seen them to court,
01:12:03but he's a really handsome guy and
01:12:05he's like super well spoken and so
01:12:08he's an intern at Hopkins and he
01:12:10gets called into blaylock's office.
01:12:12This this from this is from
01:12:14Denton Chuy's mouth. And and.
01:12:19Blaylock goes to him.
01:12:20He goes Denton, he goes.
01:12:22I've heard that you are really popular
01:12:26with the women in this hospital.
01:12:29And he goes.
01:12:30I don't know Sir,
01:12:31but but maybe yeah,
01:12:33maybe.
01:12:36He goes and and black goes he goes.
01:12:39I've got a big problem.
01:12:42But the problem is,
01:12:44is that Helen Taussig and her and her
01:12:49residence and he lists off a bunch of them,
01:12:51including Ruth Whittemore.
01:12:54They are constantly on top of me.
01:12:57They are always telling me what to do.
01:12:59They're always doing this.
01:13:01He goes Denton.
01:13:03Do you think you can help
01:13:05me handle these women?
01:13:09And and Denton Cooley goes.
01:13:13I don't know, but I'll try and but anyway,
01:13:17that's the story and and I'm
01:13:20pretty sure that there's no.
01:13:22There's no controlling anybody,
01:13:23but that's how Denton Cooley
01:13:25got got his residency.
01:13:27According to Denton. Cool, OK,
01:13:30so so there's a Helen house, or there's a.
01:13:33Glenn Glenn comes to Yale.
01:13:35He's got a problem.
01:13:37He's never done any heart operations.
01:13:40Harry Schumacher the the only the
01:13:43only person who's who's really ever
01:13:45done any of these cases is now gone.
01:13:49Gus Lindskog he he's not.
01:13:51He's not stupid.
01:13:53He knows what a deficit it is at Yale to
01:13:58have to have Harry Schumacher leave and.
01:14:02And so you can only imagine he's
01:14:04putting some pressure on on on Glenn.
01:14:09And then there's the world with what's
01:14:12the world in the late 40s, early 50s?
01:14:15What's the world of of cardiac surgery?
01:14:17It's kind of.
01:14:18It's kind of this.
01:14:20And and Michael was saying about
01:14:22cross circulation and so so cross
01:14:24circulation actually is is they
01:14:26didn't haven't the the problem?
01:14:28Wasn't the pump the problem
01:14:30was the oxygenator.
01:14:30They couldn't get a good oxygenator
01:14:32and So what they would do is
01:14:34they'd hook the parent up.
01:14:36To the baby and they would pump assist,
01:14:40assist the pump but the the the
01:14:42parent was actually the oxygenator
01:14:44but I mean this was like crazy.
01:14:46Alright this this is like
01:14:49some original footage.
01:14:50OK so this is a BSD that's being closed.
01:14:54Umm no patch.
01:14:57Interrupted suture like this
01:15:00humongous incision in the RV.
01:15:03No cardioplegia, obviously, no arrest.
01:15:07No vents, no cardiotomy suction.
01:15:12No defibrillator, no pacemaker.
01:15:16But I mean, that's that's pretty.
01:15:21OK. So. But, and in the days of the
01:15:27Giants like it was bedlam right,
01:15:31so this this first one up on the upper left.
01:15:34Is is is Doctor Lewis who is closing an ASD
01:15:39he he closed it under deep hypothermia?
01:15:43You know, you know you know that is he.
01:15:45He takes a girl, he he puts her in a on
01:15:50top of a block of ice in a in a huge.
01:15:54It was a it was a it was a trough for
01:15:58feeding horses and they would blow.
01:16:00They would take a fan and they
01:16:02they cool the the the child off.
01:16:05Put her on the table.
01:16:08Open her up.
01:16:09It was a clamshell bilateral thoracotomy?
01:16:12Inflow occlusion,
01:16:13which means they basically clamp the KV,
01:16:17no blood flowing in there.
01:16:20Close at the best you can close
01:16:22it up and then rewarm her in.
01:16:24In warm water you can only imagine the
01:16:28sterility involved in this right and then
01:16:31of course this down here is is Lehigh.
01:16:34Right and and just from the
01:16:38number of pictures,
01:16:39the number of people around
01:16:41here that Donald Ross,
01:16:42who's famous for the the Ross procedure.
01:16:44He he, he writes.
01:16:47He goes.
01:16:48He goes when when he went in there
01:16:50he said it was like a circus.
01:16:51There's like 50 people kind of running
01:16:54around and you can imagine because what
01:16:57what kind of guy is doing this right?
01:17:01200% mortality.
01:17:03It's this guy walk little high,
01:17:06but look at him he.
01:17:07He's kind of like demanding
01:17:09attention this this.
01:17:11This is what loher here boom, right?
01:17:13It's pretty clear like he's like OK?
01:17:16John Lewis, you're doing this,
01:17:18but I'm better than you,
01:17:20he's like.
01:17:22And and so I I think it's important
01:17:25to help lay the scene also is
01:17:27because if you look at this picture,
01:17:29especially this picture down here,
01:17:32he's looking through the picture at you like.
01:17:36This is how intense this guy is and
01:17:40and Denton Cooley clearly marks in in
01:17:43addition to being this this kind of guy.
01:17:45He's like he's emotionally Hardy,
01:17:48basically because so many people died but.
01:17:52He could just keep going right?
01:17:54He changed the world but but,
01:17:56but also you know what kind of guys
01:17:58this the this is going to make.
01:18:00Tina cringe said.
01:18:01Avoid libraries and literature searches
01:18:04because that doesn't matter because
01:18:07let me just let me just go for it.
01:18:09OK, that and all that stuff doesn't matter.
01:18:13Right,
01:18:14But what does he do?
01:18:16He he gets pretty amazing results,
01:18:19but he goes through the entire
01:18:22learning curve in this thing like.
01:18:25Like you close the VSD.
01:18:27You you trap the air valve,
01:18:29it's right there you saw how big this
01:18:32this this whole incision on the RV is.
01:18:35There's he talks about residual
01:18:37VSD's because he sold to this
01:18:39limb of muscle tissue.
01:18:41Madonna knows that happens,
01:18:42that's that's easy to do right?
01:18:45And a third of all his deaths
01:18:46were complete heart block because
01:18:48he had no Pacers,
01:18:49right? There's no cross clamping
01:18:51and and actually when, when?
01:18:54When it comes down to it.
01:18:59There's there's a number of different things,
01:19:01number of different. Umm?
01:19:04Writings where where him and his associates
01:19:08minimize the risk to the donors.
01:19:11It really just said, uh,
01:19:12nothing's going to happen and and actually
01:19:14one of them had a had a big stroke.
01:19:20But it was a magical time, right? It?
01:19:24It was a time when the the heart lung
01:19:27machine kind of was coming on and everybody
01:19:30was like entranced with this whole thing.
01:19:33But these are the 1st 18 cases
01:19:36done on cardio pulmonary bypass.
01:19:3817 out of 18 died right it
01:19:42it changed the world right?
01:19:44But but this is the the scenario.
01:19:48It it's fascinating reading.
01:19:50What was going on in Minnesota at the time?
01:19:52Because everybody's entrance.
01:19:54Hundreds of surgeons are going up
01:19:57to Minnesota to learn about this,
01:19:59and they're they're looking at.
01:20:01There's 50 people in the room.
01:20:02They're coming all the time,
01:20:04and after their operation they
01:20:06would have lunch and then they
01:20:08would go down to the basement.
01:20:10And there's a guy named Vincent God.
01:20:13Who became head of of cardiac
01:20:16surgery at at Johns Hopkins?
01:20:19Vincent got another guy, DeWitt.
01:20:22They would.
01:20:23They would give him a demonstration
01:20:25about the DeWitt oxygenator,
01:20:28like it was the first.
01:20:29It was the 1st.
01:20:33Disposable oxygenator and and it
01:20:35really changed the world and and
01:20:37after this presentation after
01:20:39seeing little high there and this
01:20:42presentation by by DeWitt and and
01:20:45Vincent Cott they would have the
01:20:48opportunity to buy the tubing and
01:20:50to buy the the kind of whole thing.
01:20:53And of course everybody did.
01:20:54Everybody did this,
01:20:55they wanted to start this whole program.
01:21:00Umm? But yet this this is it.
01:21:03Every surgeon was self taught right by,
01:21:07by the way. But then you know,
01:21:09nowadays we get seven O needles.
01:21:11We use, you know, high,
01:21:13high magnification.
01:21:13There's none of that.
01:21:15You know, the the needles are kind of,
01:21:17you know, not, not kind of put on.
01:21:20And because heart surgery had such
01:21:23a high mortality, only the sickest
01:21:26people were were getting this done.
01:21:29And actually word of mouth suggested
01:21:31that the number of additional.
01:21:33There were a number of additional
01:21:35temps they just didn't report it
01:21:37because the deaths were like too high.
01:21:40So what what,
01:21:42what situation do we have for Glenn here,
01:21:46right? So Glenn,
01:21:48as he's trying to to build out this program.
01:21:53He's got this legacy from Harry Schumacher.
01:21:56He's got like a very tough
01:21:59pediatric cardiology group.
01:22:00They're generally very easy.
01:22:03Maybe not right?
01:22:05He's he's got all the hopes of
01:22:09the the hospital and and Gus
01:22:12Linscott and he's got this.
01:22:14You know this world of cardiac
01:22:16surgery that is opening around it,
01:22:18but it's a world that is really risky.
01:22:21It's risk personified,
01:22:23but it's entranced, everybody it's.
01:22:25It's entranced the world.
01:22:27And there's got to be tremendous
01:22:30pressure on Glen to bring, you know,
01:22:33cardiac surgery to the to the forefront.
01:22:37And So what happens?
01:22:40Well.
01:22:41So the easy thing would be is to assume
01:22:47that Glen was very much like lillehei, right?
01:22:52But he wasn't,
01:22:53that's that's that's not Glenn.
01:22:55Actually, you know and.
01:23:00Glen he does bring the oxygen.
01:23:04This is the first cardio pulmonary bypass
01:23:07circuit that came to Yale in 1959.
01:23:09This is her name is Marjorie St.
01:23:12John.
01:23:13She's she's a specialty nurse,
01:23:16but it only came after he did
01:23:19his own experiments and and
01:23:21he took a bubble oxygenator.
01:23:23What they were doing to the lab and
01:23:25he decided that it wasn't time and
01:23:27he waded into the late late 50s.
01:23:33Uh. And what does he do?
01:23:36He goes, we know, we, we.
01:23:38We know the history, he does.
01:23:42105 Operations his first 105 operations
01:23:45with a less than 5% mortality,
01:23:48and then he does 110 PDA's
01:23:51in a row with no mortality,
01:23:53which is like in incredible incredible.
01:23:57And at the end in 1959 Ruth
01:24:02Whittemore calls him the foremost
01:24:05cardiac surgeon in America.
01:24:08To his patient and and so how
01:24:10does he do it is is he like
01:24:13this kind of lillehei guy?
01:24:17Let me just give you an
01:24:19example of of of kind of what?
01:24:21What Glenn, what Glenn was like.
01:24:23So this is a great picture in front of
01:24:26333 Cedar St which is kind of right.
01:24:28There we pass it every day.
01:24:30This is the Department of Surgery.
01:24:31In 1959 Glenn is already the man right? He?
01:24:36He's like at the peak of his his powers.
01:24:39He he has reinvigorated the department,
01:24:43Yale, cardiac surgeries already.
01:24:44Kind of back to where it was.
01:24:47If you look at this picture.
01:24:50Everybody's everybody's looking at
01:24:51the camera everybody's like yes,
01:24:54I'm here for the camera this is Glenn.
01:24:56Glenn is the only guy who's
01:24:58not looking at he's shy.
01:25:00Maybe he's maybe,
01:25:01maybe he's even a little embarrassed
01:25:04he's kind of looking off to the
01:25:06side he's like that this this
01:25:08not see Walton lohi right?
01:25:13What is see? He's a lab rat, right?
01:25:17He? He spends many hours happily
01:25:20engaged in the laboratory,
01:25:22and actually, he still cautious, right?
01:25:25He wants the things to move forward,
01:25:27but but as as Bill Sewer said, you know,
01:25:31Bill Bill, he he said that you know,
01:25:33Bill remarked him that that he that Glenn
01:25:36was preventing him from moving ahead
01:25:38too fast and and Glenn suspects he goes.
01:25:41Yeah, you know, that's that's probably true.
01:25:44That's probably true.
01:25:46And and do you know what a
01:25:48A a happy Saturday is for?
01:25:50For Glenn, happy Saturday is going to the
01:25:54Sterling Memorial Library and reading
01:25:56the collected works of Ben Franklin,
01:25:59the Franklin Papers,
01:26:00and he loves the the the two
01:26:04virtues of industry and frugality.
01:26:11And so, and so this is this
01:26:14is Glenn and and I'm going to,
01:26:16you know, I'm I'm trying to.
01:26:19When I when I before I I heard
01:26:21this this interview I was I was
01:26:23thinking about all the pressure
01:26:25and I was like what kind of guy he
01:26:27is and just just like listen to
01:26:29kind of how he kind of comes off.
01:26:35Can you hear this?
01:28:07I I mean? What what what strike me
01:28:10struck me most about about this is that.
01:28:15And and these are the kind of takeaways,
01:28:17despite having never performed this case
01:28:19and and he really he kind of relished
01:28:23the opportunity and made the most of it.
01:28:25He was really grateful.
01:28:27He was grateful he felt fortunate and lucky
01:28:31to to kind of be in this place at this time,
01:28:34and kind of he saw he saw Helen,
01:28:37Taussig and and and and Bill Seward.
01:28:42They they were his team and and he felt.
01:28:45Empowered by them and and he empowered
01:28:48them also throughout his whole career
01:28:50and and then he believed in in industry
01:28:53and practice and learning and working
01:28:56problems out and and the you know,
01:28:59the the laboratory.
01:29:01As it turns out,
01:29:02would be really a lifelong source
01:29:04of strength for him.
01:29:05So.
01:29:08Did you know Yale? Since since that time,
01:29:12Yale kind of turned over and it
01:29:14and it's really led to to other
01:29:16great surgeons, including horror.
01:29:18Stancil, who, who most famously is,
01:29:21is known for a variation on on
01:29:24what we call a damask stanza,
01:29:26which is which which we use
01:29:28for Norwood modification.
01:29:30But he used that at that time,
01:29:32which is another story entirely for an
01:29:35extra cardiac repair of transposition.
01:29:37With great arteries, so young Hill lacks,
01:29:40and of course our own Gary cough.
01:29:44But but the the thing that I found
01:29:48most struggling is is that those
01:29:51characteristics that Glen embodied
01:29:53and and the way that he saw life the
01:29:56way that he treated other people,
01:29:59the the way that he saw life as
01:30:02an opportunity to be to be had,
01:30:05rather than rather than an obstacle.
01:30:08Those are the things that really
01:30:10made Glenn great.
01:30:11Not not a single procedure that has his name.
01:30:15And and I think it's really kind of it.
01:30:18It inspired the the Department of
01:30:20cardiac surgery here at Yale and and
01:30:23really is the the centerpiece of what
01:30:25makes Yale what it is, what it is today.
01:30:28And so and so I'm going to.
01:30:30I'm going to end there,
01:30:31but but I'm going to leave you with
01:30:34what I think is is a great great movie.
01:30:37Also a little video and it.
01:30:38It's a.
01:30:39It's a clip of of Harry Schumacher
01:30:43when when he was telling.
01:30:46Alfred Blaylock that he was going
01:30:47to come to Yale.
01:31:36All right, thank you.
01:31:47Thanks Rick, that was really tremendous.
01:31:50I think it's.
01:31:53It's so interesting to see how these
01:31:55stories overlap and interlace,
01:31:57and it's really fun to hear about.
01:32:02I think an interesting time.
01:32:07I'm gonna go through my next
01:32:10presentation fairly quickly.
01:32:17Well, maybe. Maybe not.
01:32:22Does anybody need coffee?
01:32:25So why don't we take five minutes
01:32:26and we'll grab a cup of coffee?
01:32:28We'll get this to work, and then
01:32:30we'll come back and and get going.
01:32:41Take your seats and settle in.
01:33:04So. An area that that Yale is incredibly
01:33:09well known for is fetal echocardiography
01:33:12and and fetal cardiology, and I.
01:33:17I'm really honored to have these
01:33:20next three folks talk about the
01:33:22history and the future of that.
01:33:24The going to really all Friedman
01:33:28needs no introduction around here.
01:33:31Having been here for his bar Mitzvah,
01:33:33high school graduation,
01:33:34and all the rest,
01:33:36so Al has been a member of
01:33:39the section since the 1980s.
01:33:41As a fellow and then subsequently
01:33:44an attending and then section
01:33:47chief more than once.
01:33:51Residency program director and
01:33:54now is our Chief Medical Officer,
01:33:57so he's had a tremendous rise through
01:34:00the ranks and is a wonderful friend and
01:34:03leader and clinician and and cardiologist.
01:34:06So with that Al, please take it away.
01:34:16Thank you. I can't, I really can't
01:34:22express how wonderful it is to see.
01:34:26All of these familiar faces,
01:34:28some of which I get to see almost every day,
01:34:32but some of you we haven't had a
01:34:34chance to connect in person for years,
01:34:37and it really is.
01:34:38It really is fantastic to be together.
01:34:44Before we move forward,
01:34:45I do want to introduce my two
01:34:49Co presenters Dr. Josh copell.
01:34:51I'm sure many if not all of you know Josh
01:34:55is a professor of obstetrics, gynecology,
01:34:58reproductive sciences and Pediatrics.
01:35:02And Josh is a vice chair in his
01:35:06department and I have the privilege
01:35:09of working with Josh in my new role.
01:35:13Because he is the assistant Dean for
01:35:16clinical affairs for Yale Medicine,
01:35:18so any of us who have spent any time
01:35:22at Yale know that we sort of straddle
01:35:25the two sides of Cedar and York Street
01:35:28between the hospital and the university,
01:35:30and it's kind of ironic that one
01:35:33of the people who I worked with
01:35:36early in the 90s when I arrived.
01:35:39Is now someone that I work with every
01:35:43day in in how we move our hospital
01:35:46and our medical practice forward and.
01:35:49Following Josh will be Dina Ferdinand,
01:35:53who is an associate professor of clinical
01:35:56Pediatrics in our Department of Pediatrics,
01:35:59and she is the director of our
01:36:02Pediatric Echo program and nothing.
01:36:05Gave me more happiness than being able to
01:36:10pass the Echo lab from me to Bevan to Dina.
01:36:17So it's wonderful to have Dina presenting
01:36:21with us today and talking about this.
01:36:22The current and future
01:36:24state of our fetal program.
01:36:30So Dina and I have no disclosures to make.
01:36:34I'm sure we wish we did, but we don't.
01:36:37Josh does do work for on an Advisory Board.
01:36:42It has absolutely no connection
01:36:45to today's presentation.
01:36:49So, as you've heard already,
01:36:50this morning we cannot begin the
01:36:54discussion of fetal cardiology and fetal
01:36:59echocardiography without beginning.
01:37:02With Charlie Kleinman and many in the
01:37:05audience had the pleasure and the privilege
01:37:08of working and learning from Charlie,
01:37:11some of you have only heard the stories.
01:37:15The stories about Charlie and
01:37:17the stories that came from
01:37:19Charlie that have been passed on.
01:37:22Charlie came to Yale in 1977.
01:37:27He was a resident and chief resident
01:37:30at Cornell in New York and then went
01:37:33to San Francisco for cardio pediatric
01:37:36Cardiology Fellowship where he studied
01:37:39with a Rudolph and Julian Hoffman
01:37:43and really became one of the leading
01:37:48fetal physiologists in the country.
01:37:52Charlie would also use the same
01:37:54quote that Jeremy did.
01:37:56He would tell you that he stood
01:37:59on the shoulder of giants,
01:38:01but I think Charlie would also say that his.
01:38:06His success in setting up the fetal
01:38:10program here in New Haven was in some
01:38:14ways a bit of luck and circumstance and fate.
01:38:20Charlie would all often tell me
01:38:24that he was in the right place.
01:38:27At the right time with the right people.
01:38:30I think that was true for Charlie in 1977.
01:38:34That was certainly true for me in 1991,
01:38:37and I'm sure that each of you can
01:38:40think that that was likely true
01:38:43for your arrival at Yale.
01:38:49So why did Charlie say that?
01:38:51Why? Why did he revert to
01:38:54that expression so often?
01:38:56Well, as you heard from Jeremy,
01:38:58Norm Towner was the section Chief
01:39:01of Pediatric Cardiology in the 70s
01:39:04and he recruited Young Charlie
01:39:07Kleinman from San Francisco as an
01:39:10echocardiographer and to begin research
01:39:13in the world of fetal Physiology.
01:39:17Norm was certainly one of the
01:39:19right people at the right place.
01:39:23Charlie always loved to be a collaborator.
01:39:27He loved to work.
01:39:29In the section in the department
01:39:32and across the department,
01:39:34I think Charlie would tell you
01:39:36what I would tell you, and that is.
01:39:38That is one of the jewels of being at Yale.
01:39:41There are no borders.
01:39:43There are no speed bumps between our
01:39:46sections between our departments,
01:39:48and even between our schools and
01:39:51our response to COVID is a perfect
01:39:54example of how the School of Medicine
01:39:56partnered with the School of Public Health.
01:40:00Which partnered with the School
01:40:02of Nursing and partnered with the
01:40:04hospital to have one of the lowest
01:40:07mortality rates for an academic
01:40:08Medical Center in the country.
01:40:10And.
01:40:11Norm.
01:40:12Towner brought John Hobbins who
01:40:15was the chief of maternal fetal
01:40:19medicine and a leader in OB GYN.
01:40:24To Charlie and establish this
01:40:27cross departmental relationship
01:40:29that Charlie so enjoyed,
01:40:31Charlie used the equipment
01:40:34in the OB department.
01:40:37He used the nursing staff.
01:40:38He used the sonographers and
01:40:42he was a wonderful partner.
01:40:45And just like in real estate
01:40:48and just like in comedy,
01:40:50fetal cardiology is also it.
01:40:55It is also true that timing is
01:40:58everything and just before Charlie
01:41:01arrived at Yale in the late 70s.
01:41:04A man by the name of a very
01:41:06young man at the time,
01:41:07by the name of Rick Morin,
01:41:09was a medical student here at Yale.
01:41:12And as we've heard several
01:41:14examples already this morning,
01:41:16Yale is for many people the right place.
01:41:21Because Yale requires a senior medical
01:41:25thesis before graduation and Rick Moran,
01:41:29who would go on to be a pediatrician and
01:41:32a neonatologist before becoming a Dean.
01:41:35At the University of Vermont,
01:41:38Rick wrote his senior thesis
01:41:40on how this new technology of
01:41:44ultrasound could be used,
01:41:46potentially to assess the
01:41:48function of the fetal heart.
01:41:51And so these three people on the
01:41:55right of the screen really were the
01:41:58right people in the right place at
01:42:01the right time for Charlie to take
01:42:04his expertise and fetal Physiology.
01:42:07And develop the field of fetal
01:42:10echocardiography and it didn't
01:42:11take Charlie long to publish.
01:42:13I think Jeremy showed this study which
01:42:15was published in Pediatrics in 1980
01:42:18and it really is the first clinical
01:42:21example of how fetal echo can be used.
01:42:24And I again I I'm taking some liberty here,
01:42:28but I have no doubt if
01:42:30Charlie were standing here,
01:42:31he would tell you what are the odds of a
01:42:34young pediatric cardiologist in a section.
01:42:37In the Department of Pediatrics to partner
01:42:41with an obstetrician and John Hobbins.
01:42:44A mentor in Norm Telnor,
01:42:47a radiologist in Carl Jaffe who
01:42:50used his radiologic skill to
01:42:52become an echocardiographer.
01:42:54He was not a cardiologist and a
01:42:58nurse who really functioned as the
01:43:01original sonographer and coordinator
01:43:03for the fetal program, and again,
01:43:06it's that ability to collaborate that so
01:43:09inspired Charlie and so many of us after.
01:43:12And if you read through this.
01:43:14Article and it's worth reading.
01:43:16It won't surprise you to learn
01:43:19that in that first paper.
01:43:21Patients with pulmonary atresia,
01:43:23you need ventricular heart,
01:43:25complete heart block atrial flutter
01:43:27are all described accurately
01:43:30and the field was born.
01:43:37Charlie soon thereafter set out
01:43:39to build his team. And in 1986,
01:43:44three 1983 Josh Copel came as a.
01:43:51Fellow in maternal fetal medicine and
01:43:56quickly became a student of Charlie's.
01:44:01A colleague of Charlies and a
01:44:04lifelong friend of Charlies.
01:44:06And you'll hear more about
01:44:07that in a few minutes.
01:44:09I do think it is worth taking just
01:44:13a moment to recognize that for
01:44:17any of us who were students of
01:44:20Charlies in the fetal Echo lab,
01:44:22who were students or colleagues of
01:44:26Charlies in pediatric cardiology or
01:44:29who were friends and colleagues with
01:44:31Charlie in the Department of Pediatrics.
01:44:34We learned so much more than the
01:44:38science of congenital heart disease,
01:44:40the science around imaging
01:44:43and around fetal cardiology,
01:44:46and you can see that here we are.
01:44:50Rounding in the old neonatal
01:44:52ICU which was on the 4th floor
01:44:56of the Children's Hospital.
01:44:58And you can see and Dubin in the
01:45:02sort of back left center who's
01:45:05the chief of the EPA at Stanford.
01:45:08David Rosenthal,
01:45:09her future husband sitting next to her
01:45:12who's the chief of heart failure at Stanford.
01:45:15John love with his omnipresence,
01:45:18smirk sitting right behind,
01:45:20Charlie and Ziad Bobel,
01:45:22who was a senior fellow when this
01:45:25picture was taken in the winter of 1994.
01:45:29And you can see that there was
01:45:33someone very much inspired by Charlie,
01:45:36who wanted to be as smart and
01:45:41as engaged and really wanted to
01:45:45practice like Charlie.
01:45:47I I have no no shame in admitting
01:45:51that I really wanted to follow in
01:45:53his footsteps and and take care
01:45:55of patients the way he did.
01:45:57And apparently I even wanted to sit.
01:46:00In the middle of rounds, exactly like he did.
01:46:04We always learned the patients were
01:46:07always 1st and we almost always
01:46:09were having fun.
01:46:13Little did we know that the way Charlie
01:46:16took care of patients and families,
01:46:19every patient, every time,
01:46:23was really foundational for for many
01:46:27of us I had the pleasure of taking over
01:46:31Charlie Clinic when he left Yale in 1999,
01:46:34and to this day some of his patients
01:46:38who I've provided care for over
01:46:40the last couple of decades will
01:46:42remind me that when Charlie was.
01:46:44In the room taking care of them.
01:46:48They felt like they were the only
01:46:51patient in the world and the only
01:46:54family that Charlie was seeing that day
01:46:57and that really gets to the comments
01:47:00that Mr Alfieri made earlier today.
01:47:02Charlie had.
01:47:04Unbelievable medical knowledge and
01:47:07exquisite technical and clinical skill.
01:47:11But what he had that separated him from
01:47:15so many other people was an ability
01:47:18to care for people and his patients.
01:47:22And so for many of us,
01:47:24that's really what was modeled to us.
01:47:27We had no idea what patient
01:47:29centered care was.
01:47:31It wasn't a term,
01:47:32it wasn't a thing that we taught.
01:47:35It wasn't an expectation that we held
01:47:38each other to back in the 90s the way
01:47:41it is part of our everyday activity today.
01:47:45But Charlie was practicing
01:47:47patient centered care,
01:47:49and he really was ahead of his time.
01:47:52In putting the patient in family in
01:47:55the center and making sure that every
01:47:58element of their care was provided.
01:48:01And he wasn't just ahead of his time.
01:48:03In my opinion,
01:48:03he was ahead of his time in the
01:48:06Institute of Medicines Opinion as well,
01:48:09because it wasn't until the early
01:48:122000s in 2001 in the crossing.
01:48:15The Quality chasm report.
01:48:17Where they promoted patient and
01:48:20family centered care and made
01:48:22it a part of what we do.
01:48:24So I'm going to close here with
01:48:27one of my favorite pictures again.
01:48:30Time back to Mr Alfieri who who referenced
01:48:35JFK earlier in September of 1997.
01:48:40I had the privilege of joining my friends
01:48:43and colleagues as a new faculty member
01:48:46when we went up to the JFK library
01:48:49as part of the very first picks Conference,
01:48:53John.
01:48:54And Bill performed caves all
01:48:56day here in New Haven that were
01:48:59televised to the picks audience.
01:49:02Charlie and I were at the head
01:49:03of the bed elbowing each other,
01:49:06trying to do TE at the same time and then
01:49:09through the graciousness of Tina Bruckner,
01:49:12who covered the service.
01:49:14The five of us got to drive
01:49:17up to Boston that night,
01:49:19and we had a great couple of days.
01:49:22I think in the end,
01:49:24what separates a great program
01:49:25and you know it's it's.
01:49:27It's wonderful to talk about
01:49:30the history of of cardiology,
01:49:32of congenital heart disease,
01:49:33of echo, of Cath of surgery.
01:49:35What we really should be doing
01:49:38and and what I can only hope that
01:49:41we've done as we as we have so
01:49:43many of our fellow graduates here
01:49:45is the history is important and
01:49:47we should acknowledge it.
01:49:50But but any good program developer,
01:49:53any good leader?
01:49:54Sets up the future and it's really
01:49:57the next 100 years that are far more
01:50:00important than the past 100 years.
01:50:02And I think Charlie again led by example.
01:50:06He followed this quote from Wayne Gretzky
01:50:09long before Wayne Gretzky ever said it.
01:50:13Charlie had a unique.
01:50:16And wonderful ability to know
01:50:19where the puck was going to
01:50:22be and to take us all there.
01:50:25So I'm going to.
01:50:27And the podium over to my friend
01:50:30and colleague Josh Copell,
01:50:32who will tell us what he and
01:50:36Charlie did with that puck through
01:50:38the last couple of decades.
01:50:49Thanks Sal, as as you were telling some of
01:50:53that I was reminded of an email that I sent.
01:50:56A couple months ago to Jessica Kleinman
01:50:59because I was doing a fetal scan.
01:51:02On a woman whose brother had congenital heart
01:51:06disease and she told me that her brother
01:51:09was born 38 years ago and she remembered
01:51:12Doctor Kleinman and the efforts that Doctor
01:51:15Kleinman took to save her brother's life.
01:51:19So she remembered his name 38 years later,
01:51:21and that that's a lot of
01:51:23what that patient centered.
01:51:25Thing was about now.
01:51:28Al told you part of the story of this paper.
01:51:33There are two other things that
01:51:34you need to know about this paper.
01:51:37One is that the way Charlie claims he got
01:51:39involved in the fetal scans was that he
01:51:42got a call from John Hobbins saying hey,
01:51:44would you like to work on a project together?
01:51:47And Charlie said,
01:51:48sure and John said,
01:51:50come over my office,
01:51:51which was on East pavilion.
01:51:533 And Charlie walked in and John
01:51:55handed him a huge cardboard box
01:51:57full of M modes and sit here.
01:52:00Do something with this.
01:52:03Charlie did. And we we are going
01:52:05to see some of the results.
01:52:10Al also talked about the
01:52:12importance of timing.
01:52:13And of course, being as an obstetrician,
01:52:16we want everybody who can to get pregnant
01:52:18as often as they want to be pregnant.
01:52:20And it worked out for me
01:52:23that Diana Lynch had,
01:52:25I believe it was her third baby
01:52:27just before I started my fellowship.
01:52:30So Charlie didn't have a sonographer.
01:52:32And I became his stenographer.
01:52:35So for the next, I think roughly
01:52:385 years I did all the fetal echos.
01:52:41If you want to learn how to do fetal echo,
01:52:43have Charlie Kleiman reviewing every video
01:52:46and critiquing everything you do and took.
01:52:49It took a good five years,
01:52:51so I'm I estimate roughly 2000
01:52:53or so fetal echoes before I was
01:52:55even allowed to do them on my own.
01:52:58So we've seen this paper.
01:53:00I'm going to review some of the
01:53:03other things that that that came
01:53:05out of the work here at Yale.
01:53:07Because the Charlie's next big splash
01:53:08paper actually is one of the things
01:53:10that inspired me to come here.
01:53:11Just before I started fellowship,
01:53:14this was around when we were
01:53:16interviewing and matching,
01:53:17and this was a much broader description
01:53:21that I think brought more attention,
01:53:23because knowing one journal obviously
01:53:25gets read by not just pediatricians.
01:53:28The rest of the world started to
01:53:29recognize what was what was happening here,
01:53:31and again we see the collaboration
01:53:34that AL talked about.
01:53:36Greg Devore was an MFM fellow,
01:53:39Diana was still here.
01:53:40**** Berkowitz was an MFM attending
01:53:43who waited,
01:53:44became chair at Mount Sinai and
01:53:46then moved over to Columbia.
01:53:49And then we started working together
01:53:52and described a number of things
01:53:54in this collaborative group with
01:53:56John Luigi P Lou,
01:53:57who is the professor in charge
01:53:59at the in Bologna.
01:54:01Now we reported on four Chamber
01:54:04view as a screening tool.
01:54:07Actually, even after that point,
01:54:09looking at the heart was not
01:54:11a part of routine obstet 12%.
01:54:13Identifying that the fetus was
01:54:15alive was part of the ultrasound,
01:54:17but structured looking at the heart was not.
01:54:21Since then,
01:54:22this has been expanded and we now,
01:54:24in our routine scans look at
01:54:26the outflow tracts as well,
01:54:28and that's led to a significant improvement.
01:54:32Not perfection,
01:54:33but an improvement in across the
01:54:36country in detection of heart disease.
01:54:39One of the things that we're
01:54:40about to start looking at.
01:54:41I don't think it's in Dinah's presentation,
01:54:43but we're going to be looking at the the
01:54:46incidence of of unexpected congenital
01:54:47heart disease in the nursery here,
01:54:49which I suspect is quite low.
01:54:53Umm? Now we're going to
01:54:56hear a talk that I had to.
01:54:58Good fortune to see the slides from
01:55:00but I I'm looking forward to seeing
01:55:02the recording of the talk from Martina
01:55:05Bruckner and in a little bit on.
01:55:07On the genetics of congenital heart disease.
01:55:10When we started working on this together
01:55:14with Jeremiah Mahoney from genetics.
01:55:17The frequency of aneuploidy in
01:55:19congenital heart disease in the
01:55:23pediatric literature was 5%.
01:55:25We know that's not true now because
01:55:27of the more advanced techniques,
01:55:28but nobody had looked at fetal heart
01:55:30disease and we found it was about 33%.
01:55:34Now, why would that be the case?
01:55:36What makes sense?
01:55:37We see a different population than you do.
01:55:40We see fetuses that don't survive to term.
01:55:43We see fetuses that because
01:55:45of the severity of disease,
01:55:47terminate or terminated,
01:55:48so they don't make it into
01:55:50pediatric databases.
01:55:52This hadn't been described before
01:55:53and now it's it's obviously even
01:55:54more important to be looking at
01:55:56genetics with some of the work
01:55:57that we'll hear about from Martina.
01:56:01After I finished my two year MFM fellowship,
01:56:03sorry Yale MFM Fellows.
01:56:04But it was two years back then.
01:56:07Now it's three years I did an additional
01:56:102 year funded research fellowship on
01:56:13fetal cardiology which I think was
01:56:15really a way for the Yale to get me
01:56:17to be an attending but not pay me.
01:56:19But anyway, so everybody shocked
01:56:21that would happen to GAIL.
01:56:24And and we were also one of the the
01:56:27original centers doing fetal injury during
01:56:30transfusions through the umbilical cord.
01:56:33So with a visiting cardiology
01:56:36fellow named Nagahama and some of
01:56:39the other names that are familiar
01:56:41already from previous slides,
01:56:43we looked at our.
01:56:44I assuming ice population before
01:56:46and after fetal transfusions.
01:56:48Because we we thought that we
01:56:50knew that kids became hydropic
01:56:51when they were severely anemic.
01:56:53We were learning that that.
01:56:54Happened when the hematocrit of
01:56:57the fetus was below 15 to 20.
01:57:00It should be similar to what you
01:57:02see in in the nursery and what
01:57:04we were able to show is that that
01:57:08the cardiac output of fetuses
01:57:11before transfusion was high.
01:57:14And it was largely due to contractility
01:57:17and that the day after transfusion
01:57:20already when the fetuses were repleted,
01:57:24the cardiac output dropped back
01:57:25towards a more normal level.
01:57:29And these are all the things I'm going
01:57:31through here are first, say Yale.
01:57:32Another first was looking at color flow
01:57:35mapping and I I know none of you that do.
01:57:38ECHO can imagine not using color
01:57:40flow mapping, but that was not a tool
01:57:43that was available when I started
01:57:45in fellowship in in 83 that they
01:57:48just it was some spectral Doppler.
01:57:51The machine for spectral Doppler was I
01:57:53think if you took those pictures that Rick
01:57:57Kim just showed of the of the perfusion.
01:58:00Machine stacked another one on top of it.
01:58:02Put that on wheels.
01:58:04That was the the spectral Doppler
01:58:07machine that we had.
01:58:09So as color became available,
01:58:10this was one of the first looks at what it
01:58:13does and and I think it's evolved since then.
01:58:16What we found at the time was that
01:58:19it helped us perfect diagnosis,
01:58:21but really the mainstay of diagnosis
01:58:24was the 2D imaging we found the VSD.
01:58:27We found the abnormally small blood vessels.
01:58:31With spectral Doppler.
01:58:32I'm sorry with with 2D,
01:58:33but with with color Doppler we
01:58:35were able to to do things more
01:58:37quickly and more accurately.
01:58:41This was the first report of trying
01:58:44to treat fetal heart block in utero.
01:58:48We had a patient who had a a
01:58:54fetus with second degree block.
01:58:56She had anti Rowan law and basically
01:58:59she said I'll try anything.
01:59:03And Charlie and I looked at
01:59:04each other and said, well,
01:59:06we could try steroids.
01:59:07It's an anti-inflammatory drugs.
01:59:09We could use dexamethasone because
01:59:12that'll cross the placenta.
01:59:14We've been using betamethasone and
01:59:16dexamethasone at that point for.
01:59:1915 plus years for enhanced fetal lung
01:59:22maturation and we thought that we saw
01:59:25some improvement in the heart block.
01:59:28We had a series of five patients.
01:59:30I'm not sure that that we
01:59:31made much of a difference,
01:59:33but we we thought we did the fetuses with
01:59:36complete heart block did not respond,
01:59:38but this led to a lot of work
01:59:41since then on the ability to treat
01:59:44first and second degree block and
01:59:45and now as we'll hear from Dina.
01:59:47There are other work going on here on that.
01:59:53When we started looking at this with Ann
01:59:56Tan who was visiting from Singapore I,
01:59:59I looked at the obstetrical literature
02:00:00and the pediatric literature
02:00:02and again for the fellows. Here.
02:00:04You have to remember that this
02:00:05is sort of Perry Internet days,
02:00:07so that meant going to the stacks in the
02:00:09library and looking through index Medicus on.
02:00:11And it was a printed book.
02:00:14I couldn't find any reports of improved
02:00:18outcomes of any prenatal diagnosis.
02:00:22Like with one exception,
02:00:24there was a series from Yale of
02:00:2710 cases of duodenal atresia,
02:00:28and the fetuses had fewer.
02:00:31The neonates were less likely to be
02:00:33alkalotic if there was a prenatal
02:00:36diagnosis because they didn't have to
02:00:38start barfing up all their stomach
02:00:40acid to have a diagnosis made,
02:00:42so that was it in the literature.
02:00:44And this paper we were able to show
02:00:47that there was a better survival and
02:00:50lower cost with a prenatal diagnosis.
02:00:53The then, with the postnatal diagnosis,
02:00:55at least in two ventricle repairs,
02:00:57I think our our failure to find a
02:00:59difference in in single ventricle
02:01:00repairs had more to do with the outcomes
02:01:03of the single ventricle repairs at
02:01:04the time then that there there isn't
02:01:06a difference and there's a lot of
02:01:08literature since then suggesting that,
02:01:09particularly with one ventricle
02:01:11repairs we have better outcomes.
02:01:16We have seen and continue to see a lot
02:01:18of fetuses with irregular heartbeats and
02:01:20up to this point nobody had actually
02:01:23looked at that in a structured way with
02:01:27the cardiology database that that was
02:01:30available in our prenatal information,
02:01:32we were able to show that the there was
02:01:35no increased risk of structural heart
02:01:37disease and that the kids were fine
02:01:39when they were irregular heartbeats,
02:01:40but that roughly 2% of them who were
02:01:42sent in for irregular heartbeats
02:01:44actually had very significant.
02:01:46Rhythmus that needed care.
02:01:50This is just to conclude a couple of
02:01:53slides where we were and where we are now.
02:01:56This is the original database that
02:01:58I started keeping as a fellow.
02:02:01This is HIPAA compliant because number one.
02:02:03You can't read my handwriting and
02:02:06#2 we use a totally different
02:02:08medical record number system now,
02:02:10so none of these actually are
02:02:11medical record numbers,
02:02:12but we've kept track of all of
02:02:15the patients that we've done
02:02:17fetal echoes on since the 80s,
02:02:20and at present we do it a little more.
02:02:24The little more contemporary method,
02:02:26but we've got all of these these patients.
02:02:32Collected, including some
02:02:33information about that.
02:02:35The biometry and the indications,
02:02:36and the findings and this has been
02:02:39been great for us to be able to to
02:02:42continue to do projects based on
02:02:44the the prenatal and and having the
02:02:46collaboration of pediatric cardiology.
02:02:48So we have the the postnatal outcome
02:02:50has has really kept us going all these
02:02:52years and will keep us going into the
02:02:54future and I think with that I'm going.
02:02:56Oh no. I have two more.
02:02:59One of these I think is a surprise
02:03:00because Tina changed the slide.
02:03:01After I made it,
02:03:03did you add just the numbers?
02:03:05Oh OK, so this is the number of
02:03:07fetal echoes that we've done by
02:03:09year when I started in fellowship.
02:03:11Doing the echoes for Charlie
02:03:13was 300 in a year.
02:03:14Grew slowly until about 2000,
02:03:16then really expanded over
02:03:19the next dozen years.
02:03:21And the number for 2021 I believe
02:03:25was right around the same as 2012.
02:03:29But Dina added the the pediatric
02:03:32cardiology fetal echoes,
02:03:33so we've continued to grow our population
02:03:37in this collaboration and and we're
02:03:40looking forward to continued growth.
02:03:43This is a comparison of the
02:03:46indications for fetal echo,
02:03:49the one that I think is the most interesting.
02:03:53In terms of changing proportions as diabetes,
02:03:56right?
02:03:56There are far more diabetics
02:03:59in the population now.
02:04:01We've had a lot more referred for because
02:04:03somebody thinks things don't look right,
02:04:05which is exactly what we want.
02:04:07The IVF population went from nothing
02:04:09to a good portion of our of our volume,
02:04:13so we continue to be tracking
02:04:16all this information over time.
02:04:19And I think that's those are my slides.
02:04:21I'll turn it over to Deanna.
02:04:21Thank you for your attention.
02:04:28Thank you Josh.
02:04:29So just a couple of minutes.
02:04:31I just want to sort of
02:04:32quickly go through sort of.
02:04:33Where are we at within the
02:04:34field for beetle cardiology,
02:04:36particularly for things that
02:04:38where we at here at Yale.
02:04:41So here at Yale,
02:04:42all clinical care for our sort of
02:04:44our fetal cardiac program is under
02:04:46the purview of the fetal care center,
02:04:49and this is a coordinated program
02:04:51between maternal fetal medicine
02:04:52and pediatric specialties,
02:04:53and I really sort of look at it as
02:04:55sort of the true intersection of
02:04:57our Children's Heart Center with
02:04:59maternal fetal medicine and obstetrics.
02:05:01You can see sort of here.
02:05:02Many of the components,
02:05:03but the real goal of the fetal
02:05:06care center is provide coordinated
02:05:07care for the the pregnant patient,
02:05:10the mother.
02:05:10The prenatal pediatric patient
02:05:12and the family and just sort of
02:05:14really helped guide that family
02:05:15through the transition from
02:05:17prenatal to post Natal care.
02:05:21It's really focuses on the
02:05:22patient centered model,
02:05:23which sort of I wanted to bring back.
02:05:24As you know, Doctor Friedman had mentioned,
02:05:27you know it's really.
02:05:28It's not just the legacy of
02:05:29the diagnosis and imaging
02:05:31that Doctor Kleinman left us,
02:05:32but it's really a sort of the true
02:05:34patient centered model and patient
02:05:35centered care that we do today.
02:05:39So since there we've had
02:05:42sort of foundational,
02:05:43we've seen the foundations of imaging
02:05:45come from here with Doctor Kleiman, Dr.
02:05:47Cappell and others.
02:05:47There's been served several advances.
02:05:49I'm just going to go through
02:05:51some brief examples of these.
02:05:52This is an advanced color Doppler
02:05:54signal showing a small muscular VSD.
02:05:57This is some what we call stick imaging.
02:05:59Looking at multi planner imaging to look
02:06:01at 3D structures in multiple views.
02:06:04When you sort of take that one step further,
02:06:06you can sort of use multiplanar
02:06:08imaging to sort of eventually get to.
02:06:10A live 3D imaging going to show
02:06:12some examples of that mentioned.
02:06:14These are all there we go.
02:06:16These are all from our own fetal
02:06:18Paco lab and I'd like to sort of
02:06:20give a special acknowledgement to
02:06:21Sharon Abraham who's our lead fields.
02:06:22Another was obtained.
02:06:23All the images I'm going to go through.
02:06:26This is a.
02:06:31So this is a fetus with a
02:06:33nature of ventricular canal.
02:06:34At mid gestation you can see the.
02:06:37Sort of the Multiplan
02:06:38review into the 3D view,
02:06:39as here a nicer image of that when
02:06:41you clean that up as shown here.
02:06:43This is a about 20 week fetus just to
02:06:47Orient you the apex of the heart is at
02:06:49the top with the actor at the bottom,
02:06:51so the light source is really
02:06:52at the crux of the heart and you
02:06:53can see how that sort of enters
02:06:55all four of the cardiac chambers,
02:06:56which is gives really gives you
02:06:58the diagnosis of the complete
02:06:59atrioventricular canal.
02:07:01You can also appreciate the
02:07:02single AV valve as well.
02:07:06Here's just an image of an right
02:07:08ventricular outflow tract with some good
02:07:10anatomy of the branch pulmonary arteries.
02:07:12And then lastly,
02:07:14a very normal and obstructed aortic arch.
02:07:17So serve as we've seen imaging advancements.
02:07:19You know what? What have we seen
02:07:21sort of for in utero management,
02:07:22so actually sort of what sort of has
02:07:25really been in place for decades,
02:07:26you know, as doctor Cappell and others
02:07:28that are described for a while,
02:07:30it's the medical therapy for
02:07:32fetal tachyarrhythmias, really,
02:07:34through maternal administration.
02:07:35For transplacental treatment,
02:07:36the therapies that we're giving
02:07:37are really sort of the same that
02:07:38have been in place for decades.
02:07:40The only thing that is being worked
02:07:41on now is sort of studies to look at
02:07:44optimal therapy depending on sort of
02:07:46what is the specific antiarrhythmic?
02:07:47Medication and really targeting
02:07:49towards the arrhythmia itself.
02:07:51I'll very briefly mention that in utero
02:07:54fetal cardiac catheter intervention
02:07:56is being performed at some centers.
02:07:58This is very, very,
02:07:59very specific subset of lesions
02:08:01and therefore only applies to
02:08:02a small subset of patients.
02:08:06So what's sort of the next sort
02:08:08of her on the horizon for sort
02:08:10of research within the field?
02:08:12Sort of goals moving forward and
02:08:13fetal cardiac research is really
02:08:15looking at variables relating to
02:08:17commuter progression as well as
02:08:18prediction of postnatal outcomes.
02:08:20Of course limitation.
02:08:21As we know congenital heart disease
02:08:22solutions can be rare diseases,
02:08:23and of course there's variable
02:08:25treatment strategies that can
02:08:27be very centered dependent.
02:08:29This is an editorial published
02:08:30in 2016 by the founding board.
02:08:32Members of the Fetal Heart society
02:08:34and sort of one of their primary
02:08:36aims is really to establish a
02:08:38platform to be able to promote
02:08:40multiple disciplinary collaboration
02:08:42and fetal cardiovascular research.
02:08:45So sort of what are we doing
02:08:46here at Yale now?
02:08:47Sort of to be part of these
02:08:49multi institutional studies,
02:08:49so I'm just going to sort of run
02:08:51through a couple of examples of things
02:08:53that were that are ongoing here.
02:08:55So within the fetal heart society itself.
02:08:56As I mentioned,
02:08:57there are several multi institutional
02:08:59studies we've contributed
02:09:00to several of these.
02:09:02Another one of their aims in
02:09:04addition to research has been
02:09:06educational so both our MFM faculty
02:09:08as well as Peach cardiology faculty
02:09:10have been involved in seminars
02:09:12as presenters and panelists.
02:09:15Sort of as as Josh mentions,
02:09:17the importance of sort of
02:09:19studying fetal heart block.
02:09:20So stop block is an important
02:09:22trial that we are part of.
02:09:24Yale is one of 31 institutions
02:09:26across the US and Canada.
02:09:27This is looking at women who are at high
02:09:29risk of fetal heart block where they're
02:09:31doing daily home Doppler screenings and
02:09:33really the goal is to pick up any screen,
02:09:35any Doppler signal that shows
02:09:38signs of developing heart block,
02:09:40treat that woman.
02:09:41Therefore treat that fetus with the goal to
02:09:43prevent progression to complete heart block.
02:09:45And then last but certainly not least,
02:09:47is the cardiac strain twin study,
02:09:50which is being led out of Yale.
02:09:51This is collaboration between
02:09:54cardiology led by Katya Kosiv
02:09:56and MFM from led by Emily Lee,
02:09:58and this is focused on Monochorionic
02:10:01diamniotic twin pregnancies,
02:10:03which are those who are at risk of
02:10:05twin twin transfusion syndrome and
02:10:06therefore at risk of cardiac dysfunction.
02:10:08And so the goal is by doing
02:10:10strain in these fetuses,
02:10:11are we able to pick up kind of early
02:10:13predictors of cardiac dysfunction?
02:10:16And then last just sort of and sort
02:10:18of where do we see sort of the field
02:10:20going in the next couple of years
02:10:22in terms of diagnostic standpoint,
02:10:24I think first trimester fetal
02:10:25echo is something that's growing.
02:10:27This is when a fetal echocardiogram
02:10:29is performed between 11 and 14 weeks,
02:10:31typically gestation,
02:10:32and certainly while there's sort
02:10:34of inherent limitations in the size
02:10:36of the fetal heart and structures.
02:10:37Many major lesions actually can
02:10:39be picked up at this time,
02:10:40and then fetal cardiac MRI,
02:10:42which also has many limitations.
02:10:44As you can imagine due to sort
02:10:46of the constantly.
02:10:47Shifting fetal heart position as well as
02:10:49high fetal heart rate makes it a challenge,
02:10:51but I think it is becoming a
02:10:53new modality for diagnosis.
02:10:55And I think with that we'd like to conclude.
02:11:00And say thank you and I just sort of
02:11:02wanted to add that I think it's been,
02:11:03you know,
02:11:03an honor to be part of an institution
02:11:05that's really had such sort of
02:11:07foundational contributions to the field.
02:11:09And I look forward to what's to come.
02:11:22I'd really like to thank the three of you
02:11:24so much for for bringing that to us, really.
02:11:27Fascinating to see the history and and
02:11:30the interweaving of things yet again.
02:11:33And for sure, through through AL and others.
02:11:37Charlie definitely lives on in this section
02:11:40without without a doubt. I'm going to.
02:11:43I'm not doing it, I'm going to.
02:11:47Jump backwards for a moment.
02:11:51You're doing it OK.
02:11:56I'm going to jump backwards for a moment
02:11:59and go very quickly through a history of
02:12:04congenital catheterization and intervention.
02:12:07The the goal had been to try and lay out
02:12:10a historical perspective and then look
02:12:13toward the future and I I still would
02:12:16like to sort of hone to that a bit so.
02:12:21But in the interest of time,
02:12:22we will go through this pretty quickly.
02:12:25So congenital cardiac
02:12:26catheterization at Yale.
02:12:28We sort of touched on before and
02:12:30and what the history looked like.
02:12:32And again, these were some some
02:12:35folks who were instrumental in
02:12:37the work that was done in the lab.
02:12:40And this is Alice Rollins.
02:12:42Again, I believe this person's
02:12:45name is Deborah Nixon and she was.
02:12:49She was working with Norm Towner in
02:12:52the lab early on and this person in
02:12:56the back is Joyce Sterling who was also
02:12:59an assistant to Doctor Towner early on.
02:13:04So the early days in the lab really
02:13:06in 1960s and 70s when Doctor Towner
02:13:09got here and and got Mike Berman
02:13:11up to Yale and again just to
02:13:14remind you there was there was no
02:13:16pulse ox or oximeter to rely on.
02:13:18There was no echocardiogram
02:13:20and there was no prostaglandin.
02:13:22So to hear the stories of those
02:13:25nights is really quite remarkable.
02:13:27If a child came in and was blue
02:13:29or had a murmur and was suspected
02:13:31to have major heart disease.
02:13:33Team came in,
02:13:34went to the Cath lab and either
02:13:37made a diagnosis which in in some
02:13:40cases from from what I've been told,
02:13:43the diagnosis might even get
02:13:44made in the NICU.
02:13:45They would inject contrast through
02:13:47the umbilical artery line if if
02:13:49hypoplastic left heart syndrome was
02:13:51diagnosed by that injection that
02:13:53no care was given that was in an
02:13:56era before we had surgery that that
02:13:57could bring those children through
02:13:59other babies would come down to the Cath lab.
02:14:02Have a picture taken.
02:14:03And if there was.
02:14:05Nothing that could be done to
02:14:07stabilize the patient.
02:14:08Then the patient would go to the
02:14:10operating room in the middle of the night.
02:14:12In 1965, Bill Rashkin came out
02:14:15with the rashkin procedure,
02:14:17the atrial septostomy,
02:14:18so that allowed some intervention to be
02:14:22done in the Cath lab for specific regions.
02:14:25So the Cath lab was able to provide
02:14:28diagnostic information and and soon
02:14:30thereafter soon after angiography came
02:14:33the the evaluation of human dynamics.
02:14:36So and this is baby with transposition
02:14:38and VSD you can see the pulmonary
02:14:40arteries coming off the left ventricle.
02:14:43Here this is a some form of single
02:14:45ventricle with transposition
02:14:46can see the aorta coming off
02:14:48anteriorly within battle line,
02:14:50septum and pulmonary atresia.
02:14:51So this is sort of the
02:14:53information that they had. And.
02:14:57This was the blood gas machine.
02:15:00Now it's the I stat right that
02:15:01you hold in your hand.
02:15:02It's basically your iPhone.
02:15:05This is a a green dye dilution curve so pre
02:15:11oximetry you would inject some green dye.
02:15:14You would sample the blood at
02:15:17a downstream site overtime.
02:15:19Multiple samples measure the
02:15:21concentration of the dye and you would
02:15:23look and see what happened to the dye.
02:15:25How did it first show up and then
02:15:27how did it go away in that sample
02:15:28over time and you could measure
02:15:30cardiac output from that.
02:15:31So that was sort of pre
02:15:33thermodilution prefix.
02:15:36It's doctor Lister's oxygen machine.
02:15:38As I like to call it.
02:15:40And then we talked about this paper earlier,
02:15:42so I'm not going to mention it again,
02:15:43these these two landmark papers
02:15:45that came out of the Cath lab.
02:15:47So Mike Berman's catheters that I mentioned
02:15:49briefly so there were no pediatric catheters.
02:15:51Obviously in the 1970s,
02:15:52so Mike Berman developed the Berman catheter,
02:15:56which is an angiographic catheter.
02:15:57So it had a balloon at the end
02:15:59and it had multiple holes before
02:16:00the end of the catheter,
02:16:02so that contrast could be injected.
02:16:04And he invented a small wedge catheter.
02:16:06So the story goes that most of
02:16:09the work was actually done.
02:16:11By Bill Helen Brandon, Joe Brennan Brenner,
02:16:14who was another pediatric
02:16:15cardiology fellow at the time.
02:16:17Mike got all the royalties
02:16:20and took Bill to dinner.
02:16:22Basically,
02:16:22sound Bill tells you that that's true,
02:16:24so the lab always came back to Physiology,
02:16:27and this was really an incredible
02:16:30paper in the history of understanding
02:16:33hemodynamics and congenital heart disease,
02:16:34and this was another New England
02:16:36Journal of Medicine publication,
02:16:37and you'll note Doctor Kleinman again,
02:16:39normal tallinder, George Lister,
02:16:41and Bill,
02:16:42and this is 1982 and really the the
02:16:45development of the understanding of
02:16:47how hematocrit impacted pulmonary
02:16:49vascular resistance and why infants.
02:16:52Presented with heart failure
02:16:53at the time that they do.
02:16:54And really you know,
02:16:56I think there was clinical
02:16:57understanding that this happened,
02:16:59but this was the Physiology and the lab
02:17:02continually came back to Physiology.
02:17:041980 saw really the development and
02:17:06the dawn of of more interventions
02:17:08other than raskins procedure.
02:17:10So so Bill Rashkin developed the
02:17:14first PDA device, the patent ductus,
02:17:17is this connection that we mentioned
02:17:19before between the aorta and the
02:17:21lung artery that in most infants
02:17:22closes within a few days of birth.
02:17:24But in some children stays open.
02:17:27On a personal note,
02:17:28my my mother-in-law had a ductus and
02:17:30was large enough that she was in bad
02:17:32heart failure at age 6 and was one of.
02:17:34Gross's first PDA ligations in Boston.
02:17:36She traveled from New Orleans to
02:17:38Boston to have product closed.
02:17:40She was in the hospital for a month.
02:17:43So now we send kids home from
02:17:44the Cath lab the same day.
02:17:46Think Britain somewhere scheduling
02:17:47a calf on a 1K baby with a
02:17:50ductus so it's really evolved.
02:17:55Raskins sort of concepts got translated,
02:17:58then into atrial septal
02:18:00defect closure devices.
02:18:01So an atrial septal defect is a hole
02:18:03in the wall between the top 2 chambers
02:18:05and that a crew of people who in
02:18:07the Cath world are sort of giants.
02:18:10So Bill Hellenbrand, Chuck Mullins,
02:18:13Larry Latson and Jim Lock and Bob
02:18:16Beekman descended on New Haven
02:18:18and we're in the Cath lab for
02:18:20the first day of disclosure.
02:18:22John tells me he got access.
02:18:24For the case as the fellow.
02:18:32So these these devices are, you know,
02:18:35they're for the history books,
02:18:36but this is where it all began.
02:18:40And the collaboration that Al and
02:18:43Josh Dina spoke about really started
02:18:45back then and in the Cath lab
02:18:49bill and Charlie and John started
02:18:52using echocardiography to help
02:18:54guide transcatheter interventions,
02:18:55and this was the first report
02:18:57of using Transesophageal Echo
02:18:59to help guide ASD closures.
02:19:01So this is the this is a rashkin
02:19:03ASD device that probably at this
02:19:05point was the 2nd generation device.
02:19:07You can see the that the echo
02:19:09probe I think was.
02:19:1014 millimeters in diameter so it can
02:19:12only be done in in larger adults and
02:19:15this was the image quality so not great.
02:19:17You certainly couldn't project it on
02:19:19the catch screen, so I don't know.
02:19:20We we keep complaining we can't do that,
02:19:22but back then I don't know how you
02:19:25must have turned a thing around or
02:19:27something and and so developed this sort
02:19:30of field of of interventional imaging.
02:19:32And again, back to the hemodynamics,
02:19:34right?
02:19:34So in the 1990s there was a big debate
02:19:37and it continues on now about when
02:19:39do you close a Fontan fenestration.
02:19:42So there were some papers coming
02:19:43out at the time,
02:19:44and the team here went back to the lab.
02:19:47Ziyad Hijazi was a fellow at
02:19:48the time John was junior.
02:19:50Attending Charlie's on this paper,
02:19:52Gary Koff as the surgeon and bill,
02:19:55and they really evaluated the hemodynamics.
02:19:58What happens when you test include the
02:20:00Fontan fenestration and showed that
02:20:01it may not be the best thing to do?
02:20:03For the patient, you may compromise
02:20:05your ability to deliver oxygen.
02:20:07Even though the saturations go up,
02:20:09so the debate continued and you
02:20:11kind of wonder well,
02:20:12So what does anybody even read this paper?
02:20:14It's circulation.
02:20:15It's not that great at JOURNAL,
02:20:18but if you look at the number
02:20:20of downloads for this paper,
02:20:22this is 2006 to 2022.
02:20:25The paper still being downloaded
02:20:27all the time.
02:20:28These are like, you know,
02:20:29major papers in this field,
02:20:31not you know it's not 50 a day,
02:20:33but it's congenital heart disease.
02:20:34You know they're like 10.
02:20:35You know not that many.
02:20:38Collaboration continued so similar study
02:20:40you know sort of cross pollinating between
02:20:43the operating room and the Cath lab.
02:20:45You know how did Fontaine fenestration impact
02:20:48survival in high risk Fontan patients,
02:20:51so this is Gary Kopf as the lead author,
02:20:53and Charlie and Ziad and John you
02:20:56know guys kind of recycled the paper.
02:20:58I think for another journal,
02:20:59but like this was the stuff that
02:21:02was going on.
02:21:03So this was the lab and you know,
02:21:05here's Scotty helping me get
02:21:06the papers out the door again.
02:21:08Mike Berman and now is making
02:21:10the to developing this catheter.
02:21:12This is Norm Towner and Alice
02:21:14at the dedication of the Towner
02:21:16Library and John nor crew.
02:21:20Bill receiving a lifetime achievement award.
02:21:22It picks and again these
02:21:24three gentlemen who who's met
02:21:26before. So that's the history.
02:21:31I think we're going to.
02:21:32We're going to move forward now.
02:21:36Let's see where we jumped
02:21:37around a little bit,
02:21:38so I'm going to bring Rob back
02:21:40up and we're going to talk about
02:21:43the evolution of congenital adult
02:21:44congenital heart disease here because,
02:21:46as we mentioned earlier,
02:21:48all of these developments and all
02:21:50of the work was really directed at
02:21:52improving outcomes for our patients.
02:21:54And we were successful.
02:21:57Now we need Rob.
02:22:08What a great morning of talks.
02:22:09I have learned a tremendous amount.
02:22:11Oops, I guess I'm I'm not control.
02:22:15You're starting my slides.
02:22:19I'll leave it alone.
02:22:22So thanks so much for the invitation.
02:22:23I get to talk a little bit about the
02:22:25past and a little bit about the future,
02:22:27and I'm going to try to do those
02:22:28and and move us forward with time.
02:22:32As we go through this talk,
02:22:34I have taken the opportunity to
02:22:36include a lot of pictures because,
02:22:39as you've heard, the theme of the morning,
02:22:41I'm so grateful to work with all of
02:22:43the people here in this auditorium.
02:22:45My colleagues, but I also want to give
02:22:47a particular shout out to the patients
02:22:49and the families that we take care of,
02:22:51because this really is a partnership
02:22:53with them and so you'll see some
02:22:55pictures sprinkled throughout here,
02:22:57including our staff.
02:22:58This is Kristen Cowenhoven,
02:22:59one of our wonderful nurses, and one of our.
02:23:02Patients are Dallas who recently had
02:23:04a a Fontaine revision or conversion.
02:23:06One of our last patients to
02:23:07have a classic Fontan,
02:23:08and I love her shirt here.
02:23:09Don't judge my disability by its
02:23:12visibility CHD, so you'll see a lot
02:23:14of these throughout the talk here.
02:23:16I have no disclosures.
02:23:19So we know everyone in this room knows that
02:23:22congenital heart disease is a big problem.
02:23:24It occurs about one in 100 live bursts.
02:23:26The most common birth defect and about
02:23:281/4 of those infants will require surgery
02:23:30within the first year of their life.
02:23:32And you heard some of this before that.
02:23:33The current estimates in the US is
02:23:35that there's about million children
02:23:36with congenital heart disease,
02:23:38but there's about 1.4 million.
02:23:40This is as of 2016 adults living
02:23:43with congenital heart disease.
02:23:45Many of those are severe,
02:23:47and what's changing over time is
02:23:49that the proportion of adults
02:23:50who have more complicated.
02:23:52Forms of congenital heart disease
02:23:54are growing tremendously,
02:23:55so Michael and I were talking
02:23:57about this this morning.
02:23:57We don't have really good access to
02:23:59data in this country because we don't
02:24:01have universal access to healthcare.
02:24:02It's a different talk of different
02:24:04problem for a different day.
02:24:06So we we rely on our neighbors
02:24:08to the North and Canada,
02:24:09so this is data that comes out of
02:24:12Quebec looking at the population there.
02:24:15And if you just pay attention to
02:24:16the bar graph here on the bottom,
02:24:18you can see that even as of 2000
02:24:20there were more adults living with
02:24:22congenital heart disease than children,
02:24:24and that proportion has just
02:24:26continued to increase so that by
02:24:282010 2/3 of those living in Quebec
02:24:30with penalties are actually adults,
02:24:32which changing over time again
02:24:35is the complexity of those.
02:24:37Another way of looking at this is to look
02:24:38at something a little more scary and morbid,
02:24:41but look at the the patients who
02:24:43died with congenital heart disease.
02:24:45If you look in the 1980s,
02:24:46you can see that this population
02:24:48skewed and there is a lot of
02:24:50people in this younger group here.
02:24:51These are the you heard about this
02:24:53before the the patients that we didn't
02:24:55have good care for the hypoplastic
02:24:57or the complicated forms of heart
02:24:59disease or didn't have options.
02:25:00If you looked at the curve now
02:25:03into more recently 2004 2005
02:25:05that has shifted to a much more.
02:25:07A typical looking curve for
02:25:09the general population,
02:25:10so we have moved.
02:25:11We have done such a tremendous job
02:25:12of caring for those in fetal land
02:25:14and pediatric land and neonates that
02:25:16the burden of much of this heart
02:25:18disease now falls into the adults
02:25:21with congenital heart disease.
02:25:23Here's a paper that came out by one
02:25:25of my colleagues, Sasha Okonski,
02:25:27and I think a medical student
02:25:29at the time in Boston looking
02:25:31at hospitalizations and again,
02:25:33if you look at the light grade,
02:25:34these are these are children
02:25:35under the age of 18.
02:25:36It's been relatively constant over time,
02:25:38but if you look at the dark
02:25:39Gray here on the bottom,
02:25:40the growth over time has increased
02:25:42substantially so that you have
02:25:44nearly a doubling in a decade
02:25:45of hospitalizations for adults
02:25:47with congenital heart disease.
02:25:48So these patients are out there
02:25:50and they need our care,
02:25:51and so this is our triumph.
02:25:53That these patients are surviving to
02:25:55adulthood and thriving and doing well,
02:25:57here's one of my patients,
02:25:57Chris with Don or wonderful social workers,
02:26:00medical student and me.
02:26:01And this is one of our patients.
02:26:03Well,
02:26:03who took his free time to to make huge
02:26:05donations to the Children's Hospital
02:26:07in terms of toys and gifts for the holiday.
02:26:10These patients are out there,
02:26:11they're doing, well,
02:26:13they're thriving.
02:26:14And I think many people have
02:26:15heard this story before.
02:26:17The triumph of Sean White,
02:26:18and I say to my patients,
02:26:19you could be Olympic snowboarder.
02:26:20Tina was whispering this to me,
02:26:22but I thought I would show you the
02:26:23triumph of one of our own patients here.
02:26:25Hope this video works.
02:26:26Is one of our patients.
02:26:28I think John knows that David.
02:26:31David has a raw procedure.
02:26:37So David is a champion barefoot water skier.
02:26:41And you can be anything you wanna be.
02:26:44You can be successful,
02:26:45so this is our triumph right?
02:26:47You could have complex
02:26:48congenital heart disease and be
02:26:49a champion barefoot water skier.
02:26:51Sorry, too loud, sorry about that.
02:26:54So we have our own version of
02:26:56Sean White right here in the
02:26:58adult congenital heart program,
02:26:59but as we have learned and heard and
02:27:01heard very eloquently from Michael,
02:27:03you know the way of saying,
02:27:05you know you have a discharge
02:27:06Cath when you're 18 or 20,
02:27:08and saying have a nice life
02:27:09doesn't really cut it and we know.
02:27:11And one of the mantras in my
02:27:13field of adults that aren't sees
02:27:15is that repaired is not cured.
02:27:16This is a patient, not my patient,
02:27:18but they tattooed a reminder on their
02:27:20chest of their of their tetralogy of flow.
02:27:23You can see the VSD,
02:27:24you can see the pulmonary.
02:27:25Analysis and overriding era,
02:27:26and even a little zipper here at the
02:27:28top of their scar just to remind people
02:27:30when they came into the hospital.
02:27:31That repaired is not cured.
02:27:34And so we we have these triumphs.
02:27:36But we have challenges.
02:27:37And so I think you know,
02:27:38we have the 30,000 feet view
02:27:40of the challenges.
02:27:41Do we have the resources to
02:27:43take care of these people?
02:27:44Do we have the experts who are knowledgeable
02:27:46about adult internal medicine and Canaries?
02:27:49Do we have the facilities and the surgeons?
02:27:51Do we have the quality metrics for the
02:27:53knowledge all the way down to the individual?
02:27:55Aspects of the patient.
02:27:56This is one of my patients with
02:27:58severe cyanosis and isometric.
02:27:59See the clubbing and her fingertips and
02:28:01we're dealing with patients who are
02:28:03living with the unforeseen complications
02:28:05of early surgical repairs procedures.
02:28:08We don't even do anymore that have a high
02:28:10risk of heart rhythm issues over time.
02:28:12Cyanosis,
02:28:12pregnancy.
02:28:13Many of my patients want to start
02:28:15families with their own and have
02:28:16families knows what does that mean
02:28:18for them long term and then some
02:28:19of the unforeseen complications
02:28:21from their early surgeries.
02:28:24So in 2004, yeah in 2004 or thereabouts,
02:28:32Doctor John Fahey was working on an
02:28:35with George Lister and realizing
02:28:37that this is a population that we
02:28:40need to take care of and put together
02:28:43a proposal to the Yale School of
02:28:45Medicine that we needed to have a
02:28:47dedicated program to take care of
02:28:49adults with congenital heart disease
02:28:50and right around that time Jim Perry
02:28:52came and together they established.
02:28:54One of the first programs in adults
02:28:57with the first integrated program
02:28:59in congenital heart disease here
02:29:01in the state of Connecticut.
02:29:02So this was in 2004 and I really like this.
02:29:05I I John shared with me some of the
02:29:08early literature and they really honed
02:29:10in on this concept of a medical home
02:29:13for these patients and I think that
02:29:15we have really tried to stay true to that.
02:29:17So the medical home was
02:29:18supposed to be patient,
02:29:19family centered.
02:29:20It was supposed to be comprehensive and
02:29:23so it would offer all of the services
02:29:27from electrophysiology to imaging,
02:29:29even including social work,
02:29:30which I thought was really revolutionary
02:29:32and important at the time and and.
02:29:33Realization that the adults were
02:29:35coming to our practice or dealing
02:29:36with their own set of issues,
02:29:38which may be complicated.
02:29:39They need wrap around support and
02:29:41really coordinated care to make
02:29:42their time and their effort to
02:29:44come to clinic very worthwhile.
02:29:45So I think it was a really
02:29:47revolutionary concept at the time.
02:29:50And and So what has happened over that time?
02:29:53So with the stewardship of John
02:29:55and Nancy Robinson and many
02:29:57other people in the group, here,
02:29:59there was the arrival of Dan Jacoby.
02:30:02One of our colleagues in adult cardiology
02:30:04who brought a different expertise
02:30:06about heart failure and transplant,
02:30:07was one of the early partners in
02:30:09the world of adults with digital
02:30:11heart disease with John.
02:30:12I came in 2013 after after
02:30:15training in adults 200 season.
02:30:17Here I am with one of my patients,
02:30:19Collette,
02:30:20who recently had a heart transplant
02:30:22and is doing great and actually came
02:30:24to our heart block and walk the the
02:30:275K distance and is doing quite well.
02:30:29We had our own fearless leader,
02:30:30doctor Jeremy Asnes,
02:30:31who decided that he would study
02:30:33hard and take the boards and
02:30:35adults with general heart disease
02:30:36because he was seeing more adults
02:30:38with general heart disease.
02:30:39Here he is working with Laura Matos
02:30:42and the Cath lab and then more
02:30:44recently the hiring of Jeff Vinokur
02:30:46who came from Rochester and had
02:30:48a wealth of expertise and caring
02:30:50for the increasingly complicated
02:30:52rhythm issues of our patients.
02:30:54And so we've had this wonderful growth
02:30:56of the program over time and here we are.
02:31:00Recently doing training,
02:31:01the team that trains together stays together,
02:31:03so this is a recent photo from our
02:31:06group where we went to do advanced
02:31:08cardiac life support and we have Jeff,
02:31:11Nancy, Amanda, one of our nurse,
02:31:13wonderful nurses, Cheyenne Beach,
02:31:15our electrophysiologist Britton Keeshan
02:31:17Jeremy or Phyllis leader myself.
02:31:19Jess Santora,
02:31:20our unsung hero of adult fiction
02:31:22artists who gets everything done for
02:31:24us and of course Doctor. John Fahey.
02:31:28And our team has grown overtime.
02:31:29So you heard about some of the
02:31:31people here Nancy, of course,
02:31:33the new hiring of Kingdom are wonderful.
02:31:35Nurse who's out here in the audience,
02:31:38somewhere Amanda and many of
02:31:39our nurses who help us out.
02:31:42Don who is our go to social worker
02:31:44who's so grateful to have as part
02:31:45of our program and has been such a
02:31:48tremendous asset to our patients,
02:31:49our families, our staff.
02:31:50I think that Don cares for us as
02:31:53a physicians and staff as much as
02:31:54she does the patients and families.
02:31:56Jest, of course,
02:31:57who I just turned to and say.
02:31:58Yes,
02:31:58please figure out a way to schedule
02:32:00this and she manages to get it
02:32:02done and then Steve and all of
02:32:04our wonderful echo texters too
02:32:06many names to
02:32:06put up there, but we really rely on your
02:32:08expertise and it's interesting because
02:32:10I have patients come in all the time who
02:32:12have gone to the adult cardiologists.
02:32:13They've had three or four or five
02:32:15tests that they probably need.
02:32:16All they really needed was a good
02:32:18quality congenital echo to be done
02:32:20to figure out what the diagnosis is,
02:32:21so I appreciate you guys and and you
02:32:23know you've heard about this too,
02:32:25but there really is a collaborative approach
02:32:27and one of the things I have learned.
02:32:29John Fahey is that how to reach
02:32:31across the aisle and get to know
02:32:33who the go to person is.
02:32:35So when you need surgery or
02:32:37Cath or an electrophysiologist,
02:32:39but even outside of those lines bounds when
02:32:41you need a nephrologist or hepatologist.
02:32:43And so I think one of the things that
02:32:44we have tried to do in that concept of
02:32:46medical home is to build a team and
02:32:48figure out who the resource and the
02:32:50referrals are for those individuals.
02:32:52And if you look at the growth of our program,
02:32:54this is again a little bit older data
02:32:56but from 2013 to 2016 looking at total.
02:32:59Because it's up over 1000,
02:33:01which I looked at a recent data
02:33:02where were around here.
02:33:03It's still growing in spite of the pandemic.
02:33:07And this is new visits to the adult
02:33:09congenital heart programs you can see here,
02:33:11right?
02:33:11We started out.
02:33:12We had our patient population,
02:33:13but we have been growing and we become
02:33:15a resource for people around the state
02:33:17and even beyond the borders of the
02:33:19state for new consults and complex
02:33:20forms of congenital heart disease,
02:33:22resulting in about 1/3 of the Cath
02:33:25lab volume and about a third of the
02:33:28surgical volume to the program.
02:33:30So this is an important and growing
02:33:32part of our business and our program.
02:33:36So if you look at our program now,
02:33:38we have our main location
02:33:40in New Haven of course,
02:33:42but we have clinics in Hartford and
02:33:45Greenwich and have become a resource for
02:33:49the for patients in those areas as well.
02:33:52From a national standpoint,
02:33:54what has been going on well nationally,
02:33:57there are adults with congenital heart
02:33:59disease resources and this specialty
02:34:01has been more formally recognized
02:34:02so that in 2015 board certification
02:34:04for adults with general heart
02:34:06disease became a reality through the
02:34:08American Board of Internal Medicine.
02:34:10This was something that was available
02:34:12for people that had either gone
02:34:14through a training pathway through
02:34:15pediatric cardiology and then a CHD
02:34:17or adult internal medicine, ADHD,
02:34:19and currently I looked this up a couple
02:34:21of nights ago in preparation of this talk.
02:34:23Just over 300 people that are board
02:34:26certified and adults anarchies,
02:34:28which sounds like a lot.
02:34:29But when you think about the
02:34:31numbers that I showed you at the
02:34:32beginning of talk where you have
02:34:33probably close to 2,000,000 adults,
02:34:34congenital heart disease,
02:34:35it's really not that much and I
02:34:37I heard a talk recently by Karen
02:34:39Stout who's estimating that only
02:34:41about 10% of adults with 200.
02:34:42These are actually getting care by someone
02:34:44who really understands their disease process.
02:34:47So although we have made efforts,
02:34:49there's a lot more work to do.
02:34:51The adult Congenital Heart Association,
02:34:53which is started as a patient
02:34:56advocacy organization,
02:34:56has really been at the forefront
02:34:59of ensuring quality.
02:35:00We are a part of that group.
02:35:01We participate in the national meetings
02:35:03and they have one of the most useful
02:35:05things they have put together is directory,
02:35:06so that if we have a patient that's
02:35:08moving to Hawaii or California,
02:35:10we can say, well,
02:35:10why don't you go find the expert there?
02:35:12And so that this is a recent list of
02:35:14places that have identified as expertise
02:35:17and adults with congenital heart disease.
02:35:19So there is a.
02:35:21There progress going on nationwide
02:35:24to improve the resources.
02:35:27This is again data from Montreal and
02:35:30looking at their population data and
02:35:33so the black line here is looking
02:35:35at referrals to adult congenital
02:35:38heart disease programs there,
02:35:40and they had the initial preparation
02:35:42of guidelines for how to manage these
02:35:44patients in 1996 and then publication 1998.
02:35:47You can see this inflection point
02:35:49here where they see the sharp
02:35:50inflection of increased referrals of
02:35:52the population to a specialized care
02:35:54for adults with Cajun heart disease.
02:35:56In the light Gray here is mortality
02:35:59of their patients. Again.
02:36:01They have universal access to care
02:36:02and can keep track of this population.
02:36:05And around 2000 you see this inflection
02:36:08of decreasing mortality for patients.
02:36:10So this was a very powerful paper in
02:36:12our field because this is published
02:36:14in circulation in 2014.
02:36:15This is some of the first data
02:36:17to suggest that specialized care
02:36:20for adults with disabilities.
02:36:22At a center where they know what
02:36:24they're doing can actually make
02:36:25a difference in their mortality.
02:36:27And so this is supported.
02:36:29Some of the national trends of of
02:36:33board certification and accreditation.
02:36:36And along that lines in the US we have
02:36:39had now two versions of guidelines.
02:36:42You know pediatric cardiology.
02:36:43We learned a lot from our mentors,
02:36:44but there's not a national guideline
02:36:46about what to do and adults
02:36:47with congenital heart disease.
02:36:48In 2018 they published the second
02:36:50version of these guidelines
02:36:51through major organizations,
02:36:53the American College of Cardiology
02:36:54and the American Heart Association
02:36:56to support what you should be doing,
02:36:58what the best evidence of practice is
02:37:00for adults with congenital heart disease.
02:37:02And if you look one of the main
02:37:04features that they always talk about,
02:37:05is that.
02:37:06Patients with complicated forms of
02:37:08Doctor John Hartsy should be managed
02:37:10in collaboration with an expert.
02:37:12We do that well here.
02:37:16One of the other triumphs is the
02:37:18growth of knowledge and publications.
02:37:20Is there so I did a pub Med search
02:37:22and looked, and maybe Ruth Whitmore's
02:37:25paper that Jeremy referenced was
02:37:27somewhere here in the 1960s or 1970s,
02:37:29but you can see that there's been
02:37:31this exponential growth that people
02:37:33studying adults with congenital
02:37:35heart disease with more to come.
02:37:37So you know, as a as a nation,
02:37:39we are making some improvements in strides
02:37:41to try to take care of this population.
02:37:44So I thought I would just close with
02:37:46a couple of the exciting things.
02:37:47And pivoting from the the past
02:37:49and looking toward the future.
02:37:50A couple of the exciting things
02:37:51going on in our program.
02:37:53I'm going to talk briefly
02:37:54about the fellowship.
02:37:55Think about our single ventricle
02:37:57comprehensive Fontaine clinic,
02:37:58our collaboration with our maternal
02:38:00fetal medicine colleagues,
02:38:01heart camp, which I have to mention,
02:38:04cardiac transplantation,
02:38:04and then some research.
02:38:06So we have had the great fortune
02:38:09of training outstanding fellows,
02:38:11and I want to in.
02:38:14This is one of our fellows,
02:38:16Abigail Simmons who did.
02:38:17Was a yell was it yell for a long time.
02:38:20She did a Med PEDs residency
02:38:21followed by pediatric cardiology
02:38:23and she said I want to stay here
02:38:24and we said we'd love to train you
02:38:26in adults with heart disease so
02:38:28around 2018 we created a fellowship.
02:38:31The first Advanced fellowship training
02:38:32here and she is now practicing as
02:38:35an AC HD faculty out in the West and
02:38:38then of course our own graduate this year,
02:38:41David Leoni,
02:38:42who's finishing his training in
02:38:44pediatric cardiology and headed off.
02:38:46We're sad to lose in Seattle,
02:38:47headed off to be.
02:38:48Adult control heart disease specialist
02:38:50to do his advanced training in Seattle
02:38:52so I'm very proud of our trainees.
02:38:54There are a few people across
02:38:55the country that you know,
02:38:56and I think there's on the order
02:38:57of 10 to 12 fellows going into this
02:38:59specialty across the country so the
02:39:01numbers are few and far between so
02:39:03we should take great pride and joy
02:39:05in each of the accomplishments of
02:39:08these individuals. We established with.
02:39:10With John's help, a Fontan,
02:39:12multidisciplinary clinic where we had
02:39:14the involvement of a hepatologist.
02:39:16We realized that although we were
02:39:18sending patients to see the liver specialist,
02:39:20it was a lot of back and forth.
02:39:21And so we brought all the specialty
02:39:23care to them.
02:39:24So they had a comprehensive day of testing,
02:39:26and that the cardiologists,
02:39:27the liver specialist,
02:39:28we've been able to do that and
02:39:30recently had new referrals to that
02:39:33group of complex patients with
02:39:35liver and heart issues,
02:39:37which has resulted in a number of.
02:39:39Of publications over time.
02:39:41Looking at the management of these patients.
02:39:46We have realized, as I mentioned before,
02:39:48you know you heard before about
02:39:50the fetal side, but many of my
02:39:52patients want to have families of
02:39:53their own and want to get pregnant,
02:39:55and so I've had the great fortune
02:39:57of working with some of my maternal
02:39:58fetal medicine colleagues.
02:39:59Kat Campbell in particular.
02:40:00I don't think she's here,
02:40:02but has really been a wonderful partner
02:40:03and we've been able to manage very
02:40:06complicated patients through their pregnancy.
02:40:07Again with a lot of good collaboration
02:40:09between the two groups, resulting in
02:40:11a number of publications over time.
02:40:13Looking at this group.
02:40:19And then of course, heart camp.
02:40:20So we kind of fell into this.
02:40:22But this is really a wonderful thing,
02:40:24and is someone who's interested in
02:40:27transition and successful adulting hartkamp.
02:40:30You know the hartkamp is
02:40:31supposed to be about fun,
02:40:32but the secret agenda is
02:40:35knowledge and empowerment and
02:40:36transition to successful adults.
02:40:38And so we started this program about
02:40:40five or six years ago and have offered
02:40:43free camp to any child with condolences
02:40:46between the ages of eight and 15.
02:40:48It really is a wonderful experience.
02:40:50See these kids come bond over these days.
02:40:53Go down to the waterfront,
02:40:54take off their shirts to go.
02:40:55You have a scar and I have a scar and
02:40:56sort of make these connections and
02:40:58eventually transition into adults.
02:41:00Many of them come back and they act
02:41:02as junior counselors before they
02:41:04go off and one of the wonderful
02:41:06things that we do that is I often
02:41:08and I always invite one of my
02:41:10patients back for Fireside chat,
02:41:12and so it's usually towards the end of camp.
02:41:14It's late at night,
02:41:15kids are all tired and they're
02:41:17excited and the the sun is setting
02:41:19this is this is an example.
02:41:21This is one of my patients.
02:41:22Indie game with Kristen
02:41:23sitting around a campfire.
02:41:24I just do a little interview
02:41:25and then I open up and the kids
02:41:27start to ask questions and they
02:41:29asked the most wonderful intimate
02:41:31questions that you can imagine.
02:41:32And and many of them say many of
02:41:34the my adult patients mechanism.
02:41:36I wish I had this experience
02:41:38when I was younger.
02:41:39I never knew anyone growing
02:41:40up with this experience.
02:41:41I hope that we are helping them to
02:41:43realize they're not alone in the world
02:41:45and there are resources out there for them.
02:41:47It's also been a great opportunity
02:41:48for our fellows to come.
02:41:49Usually our senior fellows come
02:41:51and participate in the camp,
02:41:52and we even had one of our fellows come
02:41:54back from California to be a part of camp.
02:41:56Laura maitoza.
02:41:57Here's Ben Eddleman,
02:41:58our other outstanding graduate.
02:42:02I also want to celebrate the
02:42:04collaboration we have had through
02:42:05Dan Jacoby's work and others with
02:42:07the adults Advanced Heart Failure
02:42:09Group and cardiac transplantation.
02:42:11I got this mug, which I love 500.
02:42:14Heart transplant,
02:42:14so Yale has celebrated their 500
02:42:17heart transplants on the adult side,
02:42:19and this has become an increasing
02:42:21resource for our complicated
02:42:23adult patients that need it.
02:42:24When I first got here,
02:42:26we had to send patients out
02:42:28for cardiac transplantation.
02:42:30There was.
02:42:33Hesitancy to take on some of
02:42:34the more complicated patients.
02:42:36But over my time here and in collaboration,
02:42:38working with some of the people you've heard,
02:42:40we have been able to do successful
02:42:42transplants which are life changing for.
02:42:44Fontan, patient the patient with the mustard,
02:42:47the patient with L transposition,
02:42:48which is really revolutionary.
02:42:49And here's Colette recently at the Heart Walk
02:42:52with her shirt powered by a donated heart,
02:42:54blocking her her five Ki.
02:42:56Think that's really remarkable testament to
02:42:58the the good work that's been done here.
02:43:01So what is on the horizon?
02:43:04Well, good things are coming.
02:43:06So we recently hired.
02:43:07Of course, Tony Pastor, who's just
02:43:10completed his PG Y 10 year in Boston.
02:43:13That's ten years of postgraduate
02:43:15training after medical school,
02:43:16and he's coming to join our
02:43:18faculty starting in August.
02:43:19Thank goodness Jess,
02:43:20because we need more,
02:43:21more places to schedule patients.
02:43:24We hired a woman named Sarah Goldstein
02:43:27who is an adult cardiologist who did
02:43:30a CHD training and his expertise
02:43:32in pregnancy and congenital heart
02:43:33disease and will be coming to be
02:43:35a partner with us and be a nice
02:43:37bridge between the two groups.
02:43:38And we have completed all of our
02:43:41paperwork and our scheduling our site
02:43:43visit for our accreditation for adulthood,
02:43:46causing heart disease in July.
02:43:47So I think that's hopefully soon
02:43:49to be coming down the Pike.
02:43:50So a lot of good things coming
02:43:52to this program.
02:43:53So you know another reminder
02:43:55of the repair does not cure.
02:43:57This is a ad campaign I think through
02:43:59mended little hearts also supported
02:44:00by the adult casual heart disease
02:44:02that talks about rocking your scar
02:44:03and they have this photo competition
02:44:05once a year for patients to show off
02:44:07their scars as a way of promoting advocacy.
02:44:10And I think it's a important reminder
02:44:12that from the majority of our patients
02:44:14prepared is not cured and they should
02:44:16be in care or the tattoo on your chest.
02:44:18But really, at the end of the day,
02:44:20this is a wonderful ability to
02:44:22be a partnership between.
02:44:24US as providers and cardiologists
02:44:26and the patients and families.
02:44:29And here is, of course,
02:44:31Michael and Lisa and Nancy and I at
02:44:33one of the heart blocks in the past.
02:44:35So thank you guys very much.
02:44:37It's been fun. I've learned a lot.
02:44:49That was tremendous. Thank you.
02:44:53That I think.
02:44:58Seeing the the numbers and knowing
02:45:00that we're sort of opening the
02:45:03doors in a lot of ways to make sure
02:45:07first of all that we don't have
02:45:09patients who wind up in that gap,
02:45:11but also that patients have a place to come
02:45:13back to should they wind up in that gap.
02:45:14Is is really great and it's
02:45:17great to see the work,
02:45:19so we're going to pivot a little bit now to
02:45:24looking more toward where the field is going.
02:45:28I am so happy to introduce Sean Lang Sean,
02:45:32where are you?
02:45:33I don't see there he is.
02:45:38So Sean was a fellow here and
02:45:40depending on who you asked,
02:45:42you graduated in 2013, you think?
02:45:46And after fellowship,
02:45:47Sean went out to Cincinnati
02:45:49and did an additional year of
02:45:52training in advanced imaging.
02:45:54So for us, advanced imaging is
02:45:56generally considered to be MRI
02:45:58of the heart and CT of the heart.
02:46:01And then Sean went to Little Rock,
02:46:03AR and attending for a couple of
02:46:05years and then recently returned.
02:46:06Or it's not that recently just Cincinnati
02:46:09is as the head of MRI imaging for Cincinnati,
02:46:12so it's great to have you back.
02:46:15We wish you would come back,
02:46:17but Cincinnati still you.
02:46:19So here he is, Sean.
02:46:31Right, OK, I'm doing it OK.
02:46:35Thank you Jeremy for inviting me.
02:46:37This is been just I like to
02:46:39echo everyone's comments.
02:46:40This has been such a great,
02:46:41amazing event and I'll say like you just
02:46:44never leave here because I went to Little
02:46:47Rock and I worked with Michelle Moss,
02:46:51a great critical care person who had trained.
02:46:54I think with you John and worked
02:46:56with like had plenty of stories
02:46:58about Bill and Norm and all those
02:47:01people and in Little Rock I got to
02:47:03meet Katya Kosaf who was a young.
02:47:05Trainee now a vibrant young
02:47:08faculty member and then went to
02:47:10Cincinnati where my colleagues are.
02:47:13David Spar and Eunice Han to residents
02:47:16here who you know are now plenty of time.
02:47:21We argue about which was the best pizza
02:47:23place to go to or you know and talk
02:47:24about all about the halls that we walk
02:47:26through here and then in Cincinnati.
02:47:28Also got to meet Jeremy Steele as a trainee
02:47:31who's now a vibrant faculty member here.
02:47:33So and I think.
02:47:35This kind of goes along with the
02:47:37whole thing about evolution,
02:47:39you know, and I hope through the
02:47:40talk to kind of talk about.
02:47:42Yeah, I have no disclosures.
02:47:43Forget it.
02:47:45How we got where we are.
02:47:47I even wanted to take it even back
02:47:49because we were talking about 100 years.
02:47:51You know where were we 100 years ago?
02:47:54How did we get from an imaging modality
02:47:57where the only way we could see the
02:47:59heart was in a surgical theater or an
02:48:02autopsy to now being able to see the
02:48:05heart and manipulate it even worlds away?
02:48:09So it's hard to talk about
02:48:12evolution without mentioning Darwin,
02:48:13so he was quoted as saying in the long
02:48:15history of humankind and animal kind.
02:48:17To those who learn to collaborate
02:48:20and improvise most effectively,
02:48:21have prevailed, and I really like the
02:48:23kind of mantra that's been, you know.
02:48:27Been the themes of this kind of 100
02:48:30years of Yale Pediatrics to heal,
02:48:32innovate and collaborate.
02:48:34And it's really those pillars that
02:48:37have advanced the field of imaging.
02:48:39You know, it's collaboration
02:48:41between scientists, clinicians,
02:48:42industry, all with like,
02:48:45disruptive, innovative thinking,
02:48:46all with the primary goal of patient care
02:48:49and how we improve the care of our patients.
02:48:51Which is never stopping
02:48:53and going to continue.
02:48:54Hopefully over the next 100 years.
02:48:57So this is an I borrowed
02:48:59this from Doctor Pearson's.
02:49:02Article that got published.
02:49:04This was the 3rd floor plan in the
02:49:07Grover Powers era in the 1920s.
02:49:11Back then, really like cardiac
02:49:14imaging was only a chest X ray.
02:49:17I had this joke that I was going to say,
02:49:18oh, this is John Fahey,
02:49:20but I'm still so afraid of you
02:49:22that I was like I don't know.
02:49:24I'm like ohhhhhh. I don't know.
02:49:27We just go for it.
02:49:27Let's just do it.
02:49:30There's just so many times in
02:49:31the Cath lab that was just like
02:49:33shaking and I think you probably
02:49:34had to hold pressure for patients
02:49:36because I was like on the floor.
02:49:40So quickly I realized that I was
02:49:42going to transition to more like
02:49:44non invasive sort of things.
02:49:49So ultrasound, you know.
02:49:51Ultrasound technology had been known
02:49:53since 1915 for underwater detection and
02:49:56sonar in World War Two is used by the
02:50:00Navy for identification of airplanes.
02:50:02But really, and at that time we're
02:50:04really just talking about a mode.
02:50:06You know where you could tell the
02:50:09different reflections and their
02:50:11distance from an emitting transmitter.
02:50:13KT Dusik used ultrasound to evaluate
02:50:16the ventricle of the brain.
02:50:18It's near and dear to my family's heart,
02:50:20my wife is on neuroradiologist.
02:50:23And then in the late 40s WD Keitel described
02:50:26the rhythmic ultrasound signals when
02:50:28you put the ultrasound probe on the chest,
02:50:31but was later quoted as saying,
02:50:32you know the the work involved
02:50:35was hardly that productive.
02:50:37So it really.
02:50:39Stalled cardiac imaging until really,
02:50:44these two gentlemen Dr Inga Elder
02:50:46and Carl Helmuth Hurst really were
02:50:49pioneers in the birth of what was
02:50:51called then ultrasound cardiography.
02:50:53Now Elder was director Edler.
02:50:56Sorry not elder.
02:50:58Was director of the Cardiovascular
02:51:00Lab in Lund, Sweden,
02:51:02where the physicians were perfecting the
02:51:04procedure of closed commissure automy for
02:51:07those patients with mitral valve stenosis.
02:51:10And so Elle Edler was tasked with the.
02:51:14The goal is to try to find the
02:51:16optimal surgical candidates.
02:51:17Those with just pure mitral stenosis
02:51:20and without significant insufficiency.
02:51:22So he enlisted the help of family,
02:51:24family of famous physicists.
02:51:26This one Carl Hellmuth hurts to try
02:51:29to investigate this non invasively
02:51:31and it was heard through his family
02:51:33connections who was able to borrow a
02:51:36machine from the Siemens company which
02:51:38was an ultrasound reflective scope.
02:51:40Now at that time,
02:51:41we realized that a mode in a
02:51:44moving heart was only so limited,
02:51:47so it was Edler who figured out that if
02:51:50he could pass film at a constant rate,
02:51:52he could create that motion image,
02:51:55which was what we now know as M mode.
02:51:57So that first M mode image was obtained
02:52:01and published in October 29th of 1953.
02:52:03Now it's easy to look back now and say,
02:52:06oh that was groundbreaking.
02:52:08And and I'm certain just accelerated
02:52:11the field, but actually.
02:52:14These two gentlemen of Edler and
02:52:16Hertz became really disenfranchised
02:52:18by lack of support and from their
02:52:20colleagues and also from grant funding.
02:52:22So Edler became a little hesitant
02:52:25to further publish,
02:52:26and Carl Hellmuth Hertz actually went
02:52:29into inkjet printing so it stalled for
02:52:32a little bit until this 1960s mid 60s,
02:52:36with Doctor Harvey Feigenbaum from Indiana,
02:52:39who's now kind of recognized as
02:52:42the father of echocardiography.
02:52:44He was training to be an electrophysiologist,
02:52:47then became an interventional cardiologist,
02:52:50but was very interested in trying
02:52:52to figure out non invasive ways
02:52:54to understand the dynamics of
02:52:57ventricular function.
02:52:58In 1963 he was the first to document
02:53:01pericardial effusions using cardiac
02:53:03ultrasound, whereas story goes.
02:53:04He borrowed one of the ultrasound
02:53:06machines from a neurologist and
02:53:08just happened to have a patient
02:53:09with a known pericardial effusion.
02:53:13He then proceeded to advance that research
02:53:16with his fellow doctor Richard Pop.
02:53:19They traveled down to
02:53:21Alabama with Harold Dodge,
02:53:23who was doing Sidney angiography in
02:53:25the Cath lab and tried to correlate
02:53:29their mode measurements with with
02:53:32Doctor Dodges volumes and although
02:53:34like what seems like landmark at
02:53:36that time it was rejected by every
02:53:39single journal because people didn't
02:53:41really understand echo at that time.
02:53:44Though not to be deterred,
02:53:46Dr Feigenbaum kind of started a real
02:53:49grassroots campaign and really did that
02:53:51through the training of of trainees.
02:53:53So in 1968,
02:53:55he created the first dedicated
02:53:57cardiac ultrasound course.
02:53:59He helped adopt the name change
02:54:03to echocardiography.
02:54:04And published the first book of ECHO in 1972.
02:54:06He was also the first person to hire non
02:54:10physicians to work to acquire these images.
02:54:13So really started the field of stenography.
02:54:16And this is him receiving a lifetime
02:54:19achievement award at the ACC by and he
02:54:21was presented it by his fellow Dr Pop.
02:54:23And then he also established the American
02:54:26Society of Echocardiography in 1975.
02:54:27So it was really through that clinical.
02:54:31Push that we saw a lot of
02:54:34technical advancements.
02:54:35In 1971 Nicholas Bomb from the Netherlands
02:54:38used multi crystal array to produce the
02:54:40first real time images of the heart.
02:54:42In 73,
02:54:43Doctor Johnson combined 2D imaging
02:54:46with pulsed wave Doppler and in 79
02:54:48Holland and Hattle used the modified
02:54:50Bernoulli equation to estimate pressure
02:54:52gradients across stenotic valves.
02:54:54So really this was a time period where
02:54:56we were finally able to at least
02:54:58have a piece of the hemodynamic data
02:55:00that was being uncovered by calf.
02:55:03And in 1985,
02:55:04Doctor Amato from Japan with the
02:55:06Aloca company helped pioneer color
02:55:08Doppler kind of setting up that final
02:55:11foundational piece to the traditional
02:55:13echo that we all know about now.
02:55:17And so it was really all of these
02:55:19interventions that extremely helped
02:55:21the field of imaging of congenital
02:55:23heart disease where so much of what
02:55:25we're interested in is is far more
02:55:27than just what the size of the
02:55:28ventricles are in the function.
02:55:32But to take a step back the again,
02:55:34the results were slow.
02:55:36Initially in 1959 effort published the
02:55:39first ultrasound results in patients with
02:55:42a wide range of congenital heart disease.
02:55:44But in this paper he was unable to
02:55:46demonstrate consistent patterns or even
02:55:48differentiate it from normal hearts.
02:55:50So it took a whole another decade before
02:55:53another paper on congenital heart imaging
02:55:55came out that was in 1969 with Lundstrom,
02:55:57who had worked in Lund,
02:55:59Sweden with Edler,
02:56:00and he described the mode findings of RV
02:56:04and LV dilation in patients with ASD and VSD.
02:56:08Fast forwarding a little bit in 1974,
02:56:10Norm Silverman observed that left
02:56:12atrial enlargement occurred in premature
02:56:14infants with Peyton ductus arteriosus
02:56:17and started a conversation that will
02:56:19continue between cardiologists and
02:56:21neonatologists that to this day, thanks norm.
02:56:26I just came off console service,
02:56:27so I was like, man,
02:56:28if only I had a time machine,
02:56:30you know.
02:56:35And then there was a real shift in the
02:56:38transition from M mode to real time echo
02:56:40this is Doctor Davidson who is a resident
02:56:42and house officer here at Yale who then
02:56:45did Cardiology fellowship in San Diego.
02:56:48So while in San Diego both he and
02:56:52Richard Pop both got the multi Crystal
02:56:55Echo technology for real time Echo
02:56:57delivered to them but there was no
02:57:00manual so they were the story has it?
02:57:02They were on the phone with each other trying
02:57:04to figure out how to get the thing to work.
02:57:06And in 1972, he was the first person to use
02:57:10real time 2D echo in the United States.
02:57:13But he'll say the only reason that was true
02:57:15was because he found the on button sooner.
02:57:18These are life lessons.
02:57:22And these are some of the original
02:57:24pictures of that real time Echo,
02:57:25and I think we've advanced the
02:57:27fields quite a bit from there.
02:57:31In 1974, **** Mayer from Cincinnati
02:57:36assessed congenital heart disease
02:57:37using ECHO and chest X ray,
02:57:39and in his summary hope to avoid invasive
02:57:41human dynamic and angiographic assessment
02:57:43for all congenital heart disease patients.
02:57:45And I think the younger people in the
02:57:48room take this for granted because up
02:57:50until that point anyone would surgically
02:57:52needed heart repair was going to get capped.
02:57:55In 1978, Doctor Silverman
02:57:57proposed the apical imaging plan.
02:58:00This is when we started to try to catch
02:58:02up with Cav and angiographic information.
02:58:05We had to figure out our own plans outside
02:58:08of parasternal long axis and short axis.
02:58:11So in 78, Silverman proposed
02:58:13apical imaging plane in 1979,
02:58:15subcostal views were introduced by Roberta
02:58:18Williams to nicely outline the atrial septum,
02:58:21which which in her paper was
02:58:24particularly helpful for patients with.
02:58:27Before ballooney trials have passed me
02:58:29and she was also the first one to think
02:58:31about inverting the plane to create more
02:58:34of the anatomical appropriate image.
02:58:38And these are some of the
02:58:40pictures from her original paper.
02:58:42In 1981, Rebecca Snyder and Norm
02:58:45Silverman introduced the Super
02:58:47sternal views so that we could
02:58:49evaluate the complex arch anatomy
02:58:51issues that our patients encounter,
02:58:53and in 1989 a colleague of mine,
02:58:57Tom Kimball in Cincinnati,
02:58:58published the 1st Paper on the
02:59:00benefits of color Doppler to rule
02:59:02in or out total anomalous pulmonary
02:59:04venous return in those neonates who
02:59:06are in pulmonary hypertensive crisis.
02:59:11So it's easy to take all of
02:59:12that for granted, you know.
02:59:14But and then kind of
02:59:16looking at cardiac CT MRI,
02:59:18we're still in our infancy with this,
02:59:20but we're barring borrowing the same
02:59:23themes of innovation collaboration
02:59:25all with the game game plan to like,
02:59:27improve the care of our patients.
02:59:29So in the interest of time,
02:59:32I'm going to go through
02:59:33this a little bit quickly,
02:59:33but really it was up until
02:59:36the nine before the 1970s.
02:59:37MRI was nothing more than theoretical 1946
02:59:41block and Purcell won the Nobel Prize,
02:59:43reporting the phenomenon of being able
02:59:45to put a solid through a magnetic field
02:59:47and being able to emit radio waves.
02:59:50And in 66 Richard Ernst won the Nobel
02:59:52Prize by performing having a computer
02:59:55perform complex mathematical operations
02:59:57known as Fourier transformation.
02:59:59To improve the sensitivity
03:00:01of that received signal.
03:00:03In 1970s Lauterburg and Mansfield
03:00:05demonstrated the possibility of
03:00:08creating an MRI image signal.
03:00:10Excuse me using that MRI signal
03:00:12to create an image and in 1970
03:00:14Godfrey Hounsfield theorized
03:00:16by using multiple X ray beams,
03:00:19he could create computed tomography.
03:00:21So in 72 the 1st CT Scan was performed
03:00:25and in 1977 the first human MRI image
03:00:28was published which took five hours.
03:00:30That's a bad day for Jeremy
03:00:31and trying if he's trying to do
03:00:33that in today's day and age.
03:00:37In 1981,
03:00:38segmented data acquisition was introduced,
03:00:40which allowed us to take cardiac sinae
03:00:43imaging like that scene up here.
03:00:45This is actually an image of SSFP imaging,
03:00:48which was introduced in 1999,
03:00:50which is still become remains the
03:00:52backbone of cardiac MRI imaging.
03:00:55And I'm going to borrow this
03:00:57from a colleague of mine.
03:00:58Even the innovations haven't stopped
03:01:01in the 2010 CTS technology with
03:01:04multidetector scanners or dual source
03:01:07scanners have been able to improve
03:01:09their shutter speed to as much as
03:01:11like 60 milliseconds to able to take
03:01:14a snapshot of the heart and time.
03:01:16And this is for those who get a little dizzy.
03:01:18Just be careful.
03:01:23And so we take what we learned.
03:01:25And now where do we go?
03:01:27Where are we now and where do we go?
03:01:29This is a paper from Shay Anderson who
03:01:33had trained with us in Cincinnati.
03:01:36This is looking at all inpatient
03:01:38imaging modalities and all
03:01:40of them are on the rise,
03:01:42whether it be echo,
03:01:43MRI and we've seen a huge inflection
03:01:46point since 2014 of inpatient CT
03:01:49utilization for cardiac imaging.
03:01:54Where we in ECHO where we are,
03:01:57you know where.
03:01:58Once we are trying to prove our worth
03:02:01compared to Cath with regards to
03:02:03correlations of sizes and function,
03:02:06I think now with Z scores like
03:02:09this from the PHN we're now.
03:02:11Solely responsible for a majority
03:02:12of the clinical decision making,
03:02:14whether valves are adequate in
03:02:16size vessels or adequate in size,
03:02:17and whether people are going
03:02:19to be progressing to single
03:02:21or biventricular repair.
03:02:23Echoes also becoming smaller.
03:02:24Now we have handheld ECHO
03:02:26probes where we're hoping to.
03:02:30Decrease the socioeconomic gap and
03:02:33provide care for patients with limited
03:02:36resources here and around the world.
03:02:39And ECHO technology continues to improve,
03:02:41whether it be 3D echo.
03:02:43This is an image of a patient with a
03:02:46bioprosthetic mitral valve with severe
03:02:49stenosis and then postoperatively.
03:02:53And then newer ECHO technology,
03:02:56which this is a picture of
03:02:59ultrasound ultrafast ECHO,
03:03:00which has the capability of
03:03:02frame rates that are 100 times
03:03:04faster than conventional echo.
03:03:06So people are extremely excited about
03:03:08this technology and and the kind of
03:03:10cardiac interactions that maybe we've
03:03:12never been able to appreciate before.
03:03:16This is a paper from Jeremy Steele
03:03:19where we reviewed the kind of
03:03:22advanced imaging indications.
03:03:23The bottom line is from MRI and CT.
03:03:25It's continuing to grow and grow.
03:03:28And that's kind of indicative of
03:03:30I didn't have your guys volume,
03:03:32but in our own volume here in Cincinnati.
03:03:35Where we were doing,
03:03:37you know 50 and 2420,
03:03:392004 to now doing over 1000 in 2021.
03:03:42The volume keeps growing and
03:03:44I think around the world.
03:03:47The need for better imaging is vital.
03:03:51Given all the successes of all of our
03:03:54colleagues in the invasive and adult
03:03:56congenital world where our patients
03:03:57are not only living longer and longer,
03:04:00but our capabilities to perform
03:04:02some of these procedures that
03:04:05were only theoretical decades ago
03:04:07are now actually really reality.
03:04:12So with cardiac MRI,
03:04:13we're trying to kind of again.
03:04:16Prove we're as good as or better
03:04:19than cat that hemodynamics.
03:04:21And it wasn't this that was
03:04:24a slide from an SMR.
03:04:25Sorry where it's only imagers in the dark.
03:04:30This is a patient who has pulmonary
03:04:32hypertension with a pot shunt
03:04:33where we were calculating the QS.
03:04:37This is a patient who was a
03:04:40transposition status post atrial switch,
03:04:43where we were doing 40 flow to
03:04:45evaluate wall stress and you can
03:04:46see the narrowing of the SVC here.
03:04:51My primary interest is in cardiomyopathies,
03:04:54so we're using improved technology,
03:04:57whether it be late gadolinium
03:04:59enhancement or T1 mapping,
03:05:01and our cardiomyopathy.
03:05:02Patients hoping to kind of have better
03:05:05ideas of of when their function will
03:05:08rapidly decline and when interventions and
03:05:11newer drug modalities might be helpful.
03:05:14These are also strain imaging,
03:05:17you know, in some of our duchenne's
03:05:19muscular dystrophy patients.
03:05:23We're also trying to make MRI faster or
03:05:25more feasible for our younger population.
03:05:28Our group has been trying to use.
03:05:31Respiratory triggered sequences,
03:05:33but another idea is to do 4D
03:05:36flow or this is 3D CA Echo?
03:05:39I mean excuse me MRI.
03:05:40Where the patient sits in the scanner
03:05:43for 15 minutes and has a full data
03:05:45set acquired and then afterwards
03:05:47through post processing you can
03:05:50do unlimiting post processing to
03:05:52kind of manipulate and create
03:05:54all different planes and volume.
03:05:56This is my patient Jordan,
03:05:57who as a repaired tetralogy of fellow
03:06:00who has severe RV dilation prior
03:06:02to his pulmonary valve replacement.
03:06:07With CT now that the technology
03:06:09has improved more and more,
03:06:11we're able to visualize small
03:06:12and fast moving structures
03:06:13like the coronary arteries.
03:06:15This is a patient with an
03:06:17anomalous coronary artery origin,
03:06:18and we can look with endoscopy
03:06:20and look at the coronary orifice.
03:06:24Let's see if this will play.
03:06:27Sorry this this is an example of that.
03:06:31You won't see virtual dissection.
03:06:34One of my colleagues,
03:06:35my former colleague Justin Treder,
03:06:37who's now leading the group at the
03:06:40Cleveland Clinic, is using this.
03:06:42Our improved CT technology to help
03:06:45our surgeons better understand repairs
03:06:47or when repairs need to be fixed.
03:06:50And this is another joining
03:06:52him is my current training.
03:06:54Nicholas sugi.
03:06:55He and my colleague Ryan Moore.
03:06:57Are using kind of video game
03:06:59technology where they were taking
03:07:01these CT data sets with our surgeons
03:07:03and trying to plan out surgeries.
03:07:05This was a patient who had needed a
03:07:09complex baffling of their pulmonary
03:07:10and systemic venous return as well
03:07:12as the creation of an RV PA conduit.
03:07:15So this we're able to manipulate the
03:07:17data set here and have our surgeon
03:07:19kind of play around with the anatomy
03:07:21before they go into the operating room.
03:07:28So I didn't have like a lot of old pictures,
03:07:31but I found one of Jeremy Steele.
03:07:34This was I was just trying to get
03:07:36coffee and so I walked through
03:07:38this room and I saw these guys.
03:07:40This is Jeremy.
03:07:40This is my other colleague Ryan
03:07:42Moore and this is our surgeon David
03:07:44Morales all wearing sunglasses.
03:07:45It just was really unnerving to me.
03:07:47I didn't know whether we were
03:07:50like in a matrix like cosplay or.
03:07:53You know or this was some sort of cult?
03:07:55I mean, I should just backed away,
03:07:56but I mean I did take a picture,
03:07:58you know, just like you never know when
03:08:00those opportunities will come back.
03:08:02But I I've I've come to think
03:08:04later that this continues to prove
03:08:05that the future is bright.
03:08:06You know,
03:08:07I think if we borrow from the
03:08:08lessons of our predecessors and
03:08:10we continue to push the boulder
03:08:12up the hill a little bit better
03:08:14and continue to make the care of
03:08:16our patients all that much better.
03:08:18So with that, I'll end.
03:08:19And thank you guys so much for inviting me.
03:08:34That was also great talk.
03:08:37I feel like I keep saying
03:08:38that was a great talk.
03:08:40Thank you also for training.
03:08:41Jeremy Steele.
03:08:44Really like rely on him.
03:08:50I don't think he's still here.
03:08:52We can say whatever he wants.
03:08:54Oh, there he is.
03:08:57We were complimenting Sean
03:08:59on your training. Refund
03:09:04all right. So next I spoke about
03:09:06Tina earlier, so I'm going to
03:09:08cut this introduction short.
03:09:09Tina is a fantastic geneticist and
03:09:11researcher who's part of our section
03:09:13and she's going to talk to us about
03:09:16genetics of congenital heart disease.
03:09:30First of all, it's amazing to be
03:09:32here and see people I haven't seen
03:09:34in a long long time and be reminded
03:09:36of a lot of things that happened
03:09:37in the last 30 years that I had
03:09:40forgotten about by choice or not.
03:09:45So I'm going to talk about a Yale centric
03:09:47perspective on our understanding of the
03:09:49genetics of congenital heart disease and
03:09:51just starting with this cover slide.
03:09:53This is actually a drawing of a
03:09:56patient with heterotaxy from 1920
03:09:58something done by an amazing artist
03:10:00here at Yale Army and Hamburger.
03:10:02I found this in the archives,
03:10:04buried in the depths and this
03:10:05is how a lot of these patients
03:10:07were diagnosed in those days.
03:10:08This person had.
03:10:10Died of and complicated atrioventricular
03:10:13canal and endocarditis and the anatomy
03:10:16was very nicely outlined here.
03:10:18Here's the defect and it has some sort of
03:10:21cause that we don't completely understand.
03:10:23I have no conflicts other than whatever.
03:10:29So I know this slide has
03:10:30already been brought up by Rob,
03:10:32the prep congenital heart
03:10:33disease is super common.
03:10:34It is currently about 8 to 12 per.
03:10:381000 depending on who you ask and I
03:10:41always like to say 1% of people are born
03:10:43with a congenital heart malformation of 1.
03:10:45Sort of the other.
03:10:46I hate to call it a defect because
03:10:48that kind of has aspersions on
03:10:50our patients that I'd rather not.
03:10:52It is a congenital disease and
03:10:55is a lifelong disease.
03:10:56The increase in the incidence of
03:10:59congenital heart disease here is
03:11:00really over time due to the first the
03:11:03advent of congenital heart disease
03:11:05surgery that the patients survived,
03:11:07then echocardiography.
03:11:08That we actually diagnosed the patients
03:11:11with slightly less severe congenital heart.
03:11:14Disease and there is continuing a slow rise,
03:11:17which may be due to the fact that
03:11:20the patients of reproductive age are
03:11:22surviving and having kids who also are
03:11:24at risk for congenital heart disease.
03:11:27So when I started fellowship,
03:11:29what was our understanding of what
03:11:31really causes congenital heart disease?
03:11:34And this paper was published by
03:11:36a guy named James Nora in 1968,
03:11:38and that was really the prevailing model
03:11:41that persisted for probably 25 or 30 years.
03:11:46And when you read this paper,
03:11:48it was very interesting.
03:11:50Hypothesis one,
03:11:51there is no genetic basis to
03:11:53congenital heart disease,
03:11:54and he actually generously rejected that.
03:11:57Because there was a high incidence
03:11:59of affected relative and more
03:12:01congenital heart disease and siblings.
03:12:03However,
03:12:03now he starts getting into
03:12:05some deep water hypothesis too.
03:12:07Chromosomal aberrations cause
03:12:09congenital heart disease,
03:12:10and I think Dina and Josh and
03:12:12Charlie and many other people
03:12:14people here presented a lot of
03:12:16data to say that the answer is yes,
03:12:19but he went ahead and carry a
03:12:21typed 104 congenital heart disease.
03:12:23Patients showed no abnormalities and
03:12:25the conclusion was no chromosomal.
03:12:27Aberrations do not cause congenital
03:12:29heart disease.
03:12:30I think we start getting into some hot
03:12:32water due to inadequate measurement,
03:12:34measurement techniques and very
03:12:36small sample size.
03:12:38Hypothesis 3 single mutant genes
03:12:40cause congenital heart disease also
03:12:42rejected because he looked at a few
03:12:45pedigrees of different congenital heart
03:12:47lesions and found that Oh my God,
03:12:49some of them are dominant.
03:12:51Some of them are recessive, some
03:12:52of them don't show really clear penetrance.
03:12:54So wait a second.
03:12:55This can't be a single gene and.
03:12:57He rejected that model.
03:12:59Hypothesis 4 was that it was multifactorial,
03:13:03and he said no, this is not rejected.
03:13:05The environment plays a very important role,
03:13:08but depends on the balance of
03:13:09genes predisposing to the defect.
03:13:11So at least he sort of melded in his
03:13:13hypothesis one and four, and said, no.
03:13:15This is how it happens.
03:13:16But the major emphasis was on an
03:13:20important predominant environmental role,
03:13:22and I think this was the model.
03:13:26That was in all of the textbooks,
03:13:28at least when I started fellowship,
03:13:29and I think persisted for at
03:13:31least a decade after
03:13:32that that it was multifactorial.
03:13:34The environment was a major contributor.
03:13:36There was maybe a little chromosome,
03:13:38or maybe a little single mutant gene,
03:13:40and this little pie diagram was
03:13:42our understanding of the etiology
03:13:44of congenital heart disease,
03:13:45and from 1968 probably to the late 90s.
03:13:49Important thing to keep in mind is where
03:13:51did this leave our patients at left?
03:13:53Most of them, especially the mothers,
03:13:55because mothers tend to feel
03:13:57much more responsible here.
03:13:59Guilty that I did something that caused
03:14:02my child's congenital heart disease.
03:14:06And I think we had a lot to learn
03:14:08at the time and needed to take the
03:14:11blinders off so the person who actually
03:14:14began taking the blinders off was
03:14:16the progeny of the sort of original
03:14:19congenital heart disease people.
03:14:22So Blaylock housing and Thomas,
03:14:24who were already mentioned earlier,
03:14:26initiated the field of congenital
03:14:28heart disease care and one of their
03:14:31first fellows was Ruth Whittemore,
03:14:33who ended up establishing her career.
03:14:36Harry,
03:14:36Yale and actually Ruth retired
03:14:39the year I came in as a fellow,
03:14:42so I never really had a chance to
03:14:44interact with her and learn from her
03:14:46and I think doing this current project,
03:14:48which really terrified me when
03:14:50I started doing it,
03:14:51really made me step back and develop
03:14:54some humility and realize all
03:14:56the opportunities that I missed,
03:14:58some of which may have been due
03:15:00to the prevailing culture.
03:15:01When I started congenital
03:15:04heart disease training.
03:15:05So Ruth started out working with
03:15:07Bill Glenn and a lot of other
03:15:09people to develop better care for
03:15:11congenital heart disease patients.
03:15:13But she started questioning what
03:15:16causes congenital heart disease and
03:15:19looking at it in a very unbiased,
03:15:21rigorous scientific way by doing
03:15:23a prospective study of a large
03:15:26number of patients,
03:15:27and that was actually a super
03:15:29challenging thing to do in those days,
03:15:32she recruited eight 427 pro bands.
03:15:36With congenital heart defects,
03:15:38there are 837 children.
03:15:40In other words,
03:15:40she started with mothers that
03:15:42had congenital heart disease,
03:15:43then studied their offspring to see who
03:15:47had congenital heart disease and who didn't.
03:15:50This study took a long time.
03:15:52She started it in 1962 and
03:15:55the publication was in 1993.
03:15:58Now here's one of the reasons
03:16:01why this took so long,
03:16:03so Jeremy actually found this yesterday
03:16:05in the in the Fellows Office,
03:16:08hiding in the back corner of
03:16:10the mess back there.
03:16:12And you know the Fellows office here
03:16:14and I can tell you from my days
03:16:17starting out as a fellow was never
03:16:19an example of Good Housekeeping.
03:16:23And but what was back there
03:16:25were these boxes of like,
03:16:27the box was labeled jellied cranberry sauce,
03:16:32containing the records of her.
03:16:36430 congenital heart disease patients
03:16:37these were not on Excel spreadsheets.
03:16:40These had to be amalgamated by hand,
03:16:42probably with the help of a lot of
03:16:44medical students to come up with
03:16:46the data that she did come up with.
03:16:49But her findings ended up being
03:16:51remarkably prescient of what
03:16:53we found, using much more sophisticated
03:16:56technology going forward.
03:16:58The incidence of congenital heart disease
03:17:00in the offspring of parents with congenital
03:17:02heart disease was higher than expected.
03:17:0414% now we used to quote people and
03:17:07routine recurrence risk of three
03:17:09to 5% in families, and obviously,
03:17:11that turns out to be fantastically wrong.
03:17:15She also identified a subset of of
03:17:17her probands that were high risk.
03:17:19These were parents who had a quote
03:17:21UN quote diagnosed genetic syndrome,
03:17:23which is again something that we need to
03:17:25think about much more carefully these days.
03:17:27But she recognized that there was
03:17:29more going on than the heart lesion,
03:17:31and in those patients,
03:17:33the offspring risk was 53%,
03:17:35which turns out to be almost exactly right
03:17:38because a lot of these things are inherited.
03:17:40As autosomal dominance and in which
03:17:43case you will have 50% of your kids.
03:17:46Having some version of what you had
03:17:50and 41% in the ones with affected
03:17:52siblings and all the others,
03:17:54it was lower.
03:17:55It was around 10% but it was still a
03:17:57lot higher than the three to 5% we quoted.
03:18:01So her conclusions,
03:18:03unlike Nora's conclusions,
03:18:04were genetic liability is apparent
03:18:07in some families consistent with
03:18:09a single gene defect.
03:18:11Simple reliance on the three to
03:18:135% recurrence risk is inaccurate.
03:18:15There might be some molecular
03:18:17abnormalities such as chromosomal
03:18:19deletions and certain genes that in the
03:18:22early 90s we were just not able to detect.
03:18:24And all recognized that the role of
03:18:26genes will become better understood
03:18:28in the future with the rapid
03:18:30progress of molecular biology.
03:18:32So she retired when I started
03:18:35and when I started,
03:18:37I walked into the lab of an outstanding
03:18:40human geneticist aren't horwitch,
03:18:42who then became my husband after teaching me.
03:18:47How to do rigorous,
03:18:50thoughtful science that had a
03:18:53relationship to care of human patients,
03:18:56and he had hidden in the depths of the
03:18:59medical school a mouse, the IV mouse.
03:19:01And you can see this little mouse here.
03:19:04These are identical.
03:19:05These are genetically identical mice.
03:19:08One has the stomach on the right
03:19:10and one has the stomach on the left.
03:19:12A lot of their little mousy offspring died
03:19:14of pretty bad congenital heart disease.
03:19:17And in mice you die of congenital heart
03:19:19disease in utero usually and not postnatally.
03:19:22So he gave me this mouse and says
03:19:25you figure out what's going on
03:19:26with this mouse and that'll be a
03:19:29great ticket to understanding human
03:19:31congenital heart disease at that time.
03:19:33How to do mouse genetics was quite
03:19:35challenging, just like Human Genetics we ran,
03:19:38gels, gels were radioactive, they were nasty.
03:19:41I got really lucky.
03:19:42I got a result in my first year
03:19:44of fellowship,
03:19:44which is probably the only reason
03:19:46why I kept doing it because.
03:19:48If you don't get a result,
03:19:49it's super frustrating and you quit.
03:19:52And we map this gene to
03:19:54a chromosomal location.
03:19:55So at that point in time we knew
03:19:57that there was a gene that caused
03:19:59a type of congenital heart
03:20:01disease and mice actually turned out
03:20:03to be little furry humans when it
03:20:05comes to their hearts, they're small,
03:20:07they beat 500 times a minute,
03:20:09but they develop very much like ours do.
03:20:12So if it's there in the mouse,
03:20:13that's probably there in the human.
03:20:14Then we publish this and I remember
03:20:16Charlie Kleinman standing there.
03:20:17You know, we had to give these little
03:20:19fellow conferences where you talked to
03:20:21the other fellows about your project.
03:20:22And I was actually able to bring my
03:20:24mice to the conference, which nowadays
03:20:26you would get killed for doing.
03:20:29But Norman, Charlie and all they
03:20:30looked at the mice and said,
03:20:32Oh my gosh, you're going to identify
03:20:35the dextrocardia gene.
03:20:36They were much more optimistic than I was.
03:20:39So the problem with genetics
03:20:41of congenital heart disease.
03:20:42If Ruth was right.
03:20:45And.
03:20:46There is a major genetic contribution
03:20:48you had to find those genes and to be
03:20:51perfectly honest it wasn't going to
03:20:53turn out to be the dextrocardia gene.
03:20:55So how do you find the needle in
03:20:57the haystack to find the genetic
03:20:59abnormalities and congenital heart disease?
03:21:01There's 22 pairs of chromosomes.
03:21:04This is a karyotype which is
03:21:05what Nora thought was.
03:21:06Oh, if the carry type is normal,
03:21:08everything's cool.
03:21:09It's not,
03:21:10it's over 3,000,000 base pairs
03:21:13or three billion base pairs.
03:21:16And you have to find out one little
03:21:19mistake that screwed up your mouse
03:21:22or your patient 21,000 genes.
03:21:24And how do we do this?
03:21:26When we started with genomic analysis
03:21:28of congenital heart disease,
03:21:30we started with these very nasty
03:21:32things called sequencing gels.
03:21:34They were like this big.
03:21:35They were also profoundly radioactive,
03:21:38which means you got radioactive.
03:21:39Your lab got radioactive.
03:21:41We actually had a postdoc who's here?
03:21:43Urine got radioactive because she
03:21:45had her coffee cup on her bench.
03:21:48And you would get about 300 base pairs of.
03:21:52Sequence from this,
03:21:53and basically you sat there and
03:21:56you read off a CTG to the person
03:21:58standing next to you,
03:22:00who recorded it on a piece of paper
03:22:03until my first Mac computer arrived.
03:22:05This was incredibly laborious.
03:22:07You'd get about 300 to 500 base
03:22:09pairs per gel when everything worked,
03:22:11which it never did,
03:22:13and my first NIH grant came back
03:22:15as they're crazy.
03:22:16They're going to sequence a gene
03:22:18in 10 strains of mice.
03:22:20Are you kidding me rejected?
03:22:24But along came second generation
03:22:26DNA sequencing which really
03:22:29completely altered the landscape of
03:22:31how we analyze genetics in humans,
03:22:34mice or anything else.
03:22:36Basically,
03:22:37you change the reaction to
03:22:40something that can be adhered to
03:22:43modern substrate technology,
03:22:45and instead of having this huge
03:22:47radioactive gel that is read manually,
03:22:49everything is fed into a bunch of machines.
03:22:52Sequencing capabilities between
03:22:542000 and four 2010 doubled every
03:22:57five months and you could get
03:22:59giga bases of data per run.
03:23:01We also had computers and computing
03:23:04clusters and data specialists who
03:23:06can analyze all this stuff for
03:23:08you so that you no longer have
03:23:10to sit there and read gels.
03:23:12But you basically got reports back
03:23:14from the sequencing center that
03:23:16gave you what you're looking for.
03:23:18So in February of 2001, the first draft
03:23:21of the human genome was published,
03:23:23and what's really kind of cool about this?
03:23:25If you look at this nature publication,
03:23:27the Human genome nuclear fission
03:23:29actually took a second seat to
03:23:32publication of the human genome.
03:23:35Umm? So from there,
03:23:38Yale got very involved in this process
03:23:40and this is the next group of people
03:23:42on who's on whose shoulders all
03:23:44of this work really stand is Rick
03:23:47Lifton was brought in to Yale and
03:23:49ran genetics here from 1998 to 2016.
03:23:52He left here to become the President
03:23:55of Rockefeller University,
03:23:57but despite running an entire university,
03:23:59he is still intimately involved with
03:24:01this project and spends hours with us
03:24:04on the phone reviewing manuscripts that
03:24:06we have to try to get out the door.
03:24:08But Rick really first started taking the
03:24:11new sequencing technology and applying
03:24:14it to clinical medicine and clinical care,
03:24:17and he saw when.
03:24:20When this process started happening
03:24:22that this is something we could apply
03:24:24to congenital heart disease and set up
03:24:26a collaboration with me and Rick Kim
03:24:29here to sort of initiate one of the
03:24:31real strengths of Yale is that there is
03:24:35cross not only departmental collaboration,
03:24:37but cross institutional collaboration
03:24:39where the basic scientists and
03:24:41the clinicians all work together.
03:24:43The first grant that we put out
03:24:45on this congenital heart disease
03:24:47program was done with rickheim.
03:24:49We had to actually.
03:24:51Right, put everything on paper.
03:24:53Send 8 copies,
03:24:542 double sided to the NIH by like.
03:24:585:00 AM and I know Rick.
03:25:01Came over here, drove me out to the.
03:25:06One of the FedEx store in in North Haven.
03:25:09In my first trip ever in a Porsche Cayman.
03:25:15To get it there before the 7:00
03:25:17o'clock receipt deadline and we did,
03:25:19we got there at 658 and they took it.
03:25:22And Rick Lifton was on the
03:25:24phone with us and says, well,
03:25:25if you missed the deadline,
03:25:26don't worry about it.
03:25:27It only takes 5 hours to drive down
03:25:29to the NIH and you all are used
03:25:31to staying up all night anyway,
03:25:33so just go do it.
03:25:37But at the same time also they were building
03:25:39up the Yale Center for Genome Analysis,
03:25:41which is off of exit 42 on I-95.
03:25:46Which has really built the capacity to
03:25:49sequence huge numbers of human genetic
03:25:52data and mouse genetic data very rapidly.
03:25:55We can now spend $125 get
03:25:59every exon in a human genome,
03:26:01get it back to us within if you
03:26:04need it emergently clinically
03:26:05they can do it in five days.
03:26:08For research purposes.
03:26:09We do about 1000 to 2000.
03:26:12Congenital heart disease exomes
03:26:14over the course of four weeks.
03:26:16The data comes back as a whole ton of
03:26:19files and gets processed by informatics
03:26:21people and students and postdocs that
03:26:23are much better at that than I am.
03:26:26So what did we find out from that?
03:26:28So the first thing you've got
03:26:29to realize when you're looking
03:26:30through this needle in a haystack,
03:26:31we all have genetic variation because
03:26:33you and I don't look the same.
03:26:35So what we have to distinguish is
03:26:38what matters and causes congenital
03:26:40heart disease versus what is
03:26:42normal human genetic variation.
03:26:44So the first thing we looked at
03:26:45is looking for de Novo mutations.
03:26:47DENOVO mutations are mutations that
03:26:49are present in the affected patient
03:26:52but not in the patient's parents.
03:26:53In other words, they happened randomly.
03:26:55And when a gene is altered by
03:26:57a de Novo mutation,
03:26:58this is really one of Rick Lifton's
03:27:01greatest contributions to this field.
03:27:03It is much more likely to
03:27:05be clinically damaging,
03:27:06and we use that actually on the clinical
03:27:08service when we get a mutation back
03:27:10where we don't really know what it causes.
03:27:12If you look to see what the
03:27:14unaffected parents have,
03:27:15in other words that you're
03:27:17comparing the patient to parents,
03:27:18it's that you will be able to
03:27:20determine much more cleanly.
03:27:21Whether that mutation contributed
03:27:23to disease or not.
03:27:25So we found that de Novo mutations
03:27:28actually account for eight to 10%
03:27:29of all congenital heart disease,
03:27:323% of isolated congenital heart disease,
03:27:34but 28% of congenital heart disease who
03:27:37have also extracardiac anomalies or and
03:27:41or neurodevelopmental abnormalities.
03:27:42So in those multiply affected patients,
03:27:45these very damaging mutations of
03:27:48very large effects that happened
03:27:50spontaneously in the proband and are not
03:27:52present in the parents are a much greater.
03:27:55Contributor we also learned that it's not the
03:27:59dextrocardia gene congenital heart diseases,
03:28:02insanely complex and many,
03:28:04many,
03:28:04many genes contribute to
03:28:06congenital heart disease.
03:28:07At least 400 by the denovo mechanism alone.
03:28:12So where does this leave you?
03:28:14This leaves you with really having
03:28:16to use contemporary big data
03:28:18approaches to solve the problem.
03:28:20You can't go one patient at a time.
03:28:23So to do that,
03:28:25Yale has been a participant in the
03:28:27NIH pediatric Cardiac Genomics
03:28:29Consortium since its inception in 2009,
03:28:32and you'll recognize some of the
03:28:34people on this slide on George
03:28:36Porter sitting out there somewhere.
03:28:38Rick Kim,
03:28:39right there.
03:28:40And there's Rick Lifton and Peter
03:28:43Gruber if he's here.
03:28:44And this is a multi center
03:28:47program that has encompassed
03:28:48quite a few institutions across the US.
03:28:51We're now trying to establish an
03:28:53international collaboration. Also.
03:28:55To discover all genes involved
03:28:58in congenital heart disease.
03:29:00To identify the mutations to
03:29:02correlate to the phenotype.
03:29:03And most importantly,
03:29:04now to start developing the
03:29:07connections between the genetic
03:29:08variation and the clinical outcomes.
03:29:11So so far we've got 15,000
03:29:13congenital heart disease patients
03:29:15and about 14,000 relatives,
03:29:17and this will allow congenital heart
03:29:20disease research to start becoming
03:29:22more statistically rigorous and not
03:29:25rely on very small sample sizes.
03:29:28We've done very detailed echocardiographic
03:29:30phenotyping of all of the patients
03:29:33and are currently there is work in
03:29:35progress to link the genomics and
03:29:38electronic medical records data
03:29:39which gives me hope that all those
03:29:42hours we spend entering things in
03:29:44God darned Epic will eventually come
03:29:49to some useful clinical outcome.
03:29:53So we've done a lot of whole
03:29:55exome sequencing.
03:29:55We've done whole genome sequencing,
03:29:58targeted sequencing,
03:29:59and there's actually surgical tissue
03:30:01being banked on an increasing number
03:30:03of probands when this tissue that's
03:30:06being discarded as part of the
03:30:07procedure is flash frozen in the
03:30:09OR stored and then available for
03:30:11analysis at a later date. So so far.
03:30:15It's not the dextrocardia gene.
03:30:18There's 400 or more genes that
03:30:21contribute to congenital heart disease.
03:30:24There are some subclasses of genes
03:30:26that contribute more and give more
03:30:29insight into associated phenotypes.
03:30:31The most notable ones are
03:30:33chromatin remodeling genes,
03:30:34which globally regulate
03:30:36transcription when you don't.
03:30:37When you have a mutation
03:30:39affecting one of those,
03:30:40it's not just your heart,
03:30:41it affects your brain.
03:30:42It can affect your kidneys,
03:30:43and it probably has some really.
03:30:46Major impacts on the lifelong
03:30:48disease of congenital heart disease
03:30:51because it's not something that
03:30:53just happens for one moment,
03:30:55and then it's surgically fixed,
03:30:57but is something that patients
03:30:58live with and that we have to
03:31:00manage throughout their life span.
03:31:02A core set of 60 genes explains 8% of
03:31:05congenital heart disease recessive mutations.
03:31:08This is a manuscript that's
03:31:09sort of out there right now,
03:31:10not yet published.
03:31:12About 2.2% of congenital heart disease.
03:31:14And we're also finding an overlap between.
03:31:17Cardiomyopathy,
03:31:17genes,
03:31:18and structural congenital heart
03:31:20disease genes that may give some
03:31:23insight into why some patients
03:31:24end up developing myocardial
03:31:26dysfunction later in life.
03:31:30So I'd like to say it's a lot of
03:31:33genes that cause a broken heart.
03:31:35But an example of 1 gene.
03:31:40OK. I think I screwed something up here.
03:31:46One gene can cause a lot of different
03:31:49congenital heart defects. And just.
03:31:53OK, so there's I'm not touching it.
03:31:58So there's a lot going
03:31:59on that I don't know,
03:32:00but it magically reappeared,
03:32:03so this is. That was then 1968.
03:32:07Now just keep in mind,
03:32:08I just want to put in one more
03:32:10little advertisement here.
03:32:14Ruth Whittemore was one was one
03:32:17of six out of 72 students in her
03:32:20medical school class who were women.
03:32:23I was one of 21 out of 140 who
03:32:27are women and we now have 50%
03:32:30women starting medical school.
03:32:34We're going to keep pushing this field
03:32:36ahead and not let some prejudices
03:32:39cloud how we view what's been done
03:32:41by the people that preceded us.
03:32:44But what we have now is an
03:32:46expansion of what we know.
03:32:48There's still a lot left.
03:32:50Notice that the Gray zone 56%.
03:32:52We don't know our current
03:32:54efforts here with the congenital
03:32:57heart disease Genetics Clinic,
03:32:59which is one of the first congenital heart
03:33:01disease genetics clinics in the country,
03:33:03coupled with very aggressive genetic
03:33:05testing of patients that present
03:33:07with congenital heart disease,
03:33:09we can probably wear up to
03:33:11making diagnosis in 30 to 40%.
03:33:14But we have a long ways to go,
03:33:16and here's some of the things we can do.
03:33:18Multigenic contribution,
03:33:19and then most importantly evaluating
03:33:22the contribution of genetic data
03:33:24to patient outcome eventually
03:33:26hopefully being able to modulate
03:33:28some of the lifelong phenotypes,
03:33:30pharmacologically or otherwise,
03:33:32and really have this impact.
03:33:35We're going to we're collaborating with
03:33:37the Europeans and increasing our data set,
03:33:39hopefully to over 20,000 patients,
03:33:41so we can really draw some
03:33:44statistically significant.
03:33:45Conclusions and I'd like to thank
03:33:47all the people that help do this
03:33:50work from my husband to Rick
03:33:52Lifton to Charlie Kleinman,
03:33:53who's and Bill Helen Brand and all the
03:33:57people here who trained us and who
03:34:00probably scared some of us more than.
03:34:03Was always necessary.
03:34:09And I really am honored to be
03:34:12able to speak to this whole group
03:34:14and see all the people back.
03:34:17This is my lab and some of the
03:34:19people involved in the project.
03:34:20Most importantly the patients
03:34:22and families who contributed to
03:34:24this effort to begin to develop a
03:34:27contemporary scientific understanding
03:34:28of congenital heart disease.
03:34:30Thank you.
03:34:40Tina, thank you so much.
03:34:42I think Tina has a gift for taking
03:34:44stuff that most people don't understand
03:34:47and making it understandable,
03:34:50and I think all of her talks that
03:34:52I've seen over the last 20 years
03:34:55or so have have demonstrated that.
03:34:58So thank you all right.
03:35:00We're going to move on.
03:35:01Our next talk is going to be by Madonna Lee.
03:35:06Madonna joined us last summer
03:35:09as our cardiothoracic surgeon.
03:35:11She's trained in Seattle and
03:35:13now is here with us,
03:35:14so please take it away with dot.
03:35:31Hi, I've been here since August
03:35:33but I actually this is the first
03:35:36time seeing people's faces so
03:35:37please come and say hi to me.
03:35:39After this my name is Madonna Lee
03:35:41and I've been here less than a year.
03:35:44I don't have any stories
03:35:46from the 1950s or 19.
03:35:4816 So I'll be talking mostly
03:35:50about what we do today,
03:35:53and some of the new things on the
03:35:55horizon for congenital valve surgery.
03:35:57I don't have any financial disclosures
03:35:59or conflicts of interest as this is a
03:36:01photo of me in the operating room with
03:36:04Doctor Peter Gruber who's my senior partner.
03:36:06We will be talking very specifically today
03:36:09about Valve repair replacement options,
03:36:12and we'll be looking specifically at
03:36:14the Ozaukee procedure and also the core
03:36:17matrix trial for tricuspid valve replacement.
03:36:20So currently for Valve replacement
03:36:21options we have a mechanical prosthesis
03:36:24or a tissue valve prosthesis and
03:36:26the pediatric cardiology fellows
03:36:28have already seen this slide.
03:36:30But you know when we think about what's
03:36:32the ideal valve replacement option.
03:36:34Unfortunately it doesn't doesn't exist.
03:36:36So for adults we want it
03:36:38to be readily available.
03:36:39We want it to be inexpensive.
03:36:42Great hemodynamics,
03:36:43durable and a minimal thromboembolic risk.
03:36:47When we think about our pediatric patients,
03:36:49we need smaller sizes.
03:36:50Hopefully the valve will grow overtime
03:36:53have a non immunogenic response be even
03:36:56more durable than in the adults and still
03:36:59carry a minimal thrombotic thrombotic.
03:37:02Thromboembolic risk,
03:37:02which again would this.
03:37:04Currently we we don't have something
03:37:06that fits all of these criteria.
03:37:09One of the limitations we have specifically
03:37:11in Pediatrics is that the valve
03:37:13sizes that we create are are limited,
03:37:15so this is an example I I think I
03:37:18showed the pediatric cardiology
03:37:19fellows a shoe size chart,
03:37:21but for each product that we have,
03:37:24so each specific valve there is a range
03:37:27of sizes that exist and what we have
03:37:29to look at is the size of the patient.
03:37:31So how much they weigh and there's the
03:37:34calculation and called the body surface
03:37:35area that really gives us a range for
03:37:38whether the opening and the valve.
03:37:39Would be adequate to provide enough
03:37:41blood flow to the patient and you can
03:37:44see in the green that this is good,
03:37:46and then as you increase the
03:37:48size of the patient,
03:37:50the size is going to be
03:37:53inadequate for the patient.
03:37:55We then also for a congenital heart disease.
03:37:57Sometimes it's not just the valve that's
03:37:59dysplastic or that needs to be replaced,
03:38:01but that other structures around the heart
03:38:03may also limit the size that we can put in.
03:38:06And unfortunately currently
03:38:07any valve that we put in it,
03:38:09it's not the same as having your
03:38:12native valve tissue or a normally
03:38:15functioning native valve tissue.
03:38:16So we don't have time to get
03:38:18into all the details today,
03:38:19but one of the options is someone
03:38:21had mentioned the Ross procedure,
03:38:23so taking a pulmonary valve and putting
03:38:25it into the aortic valve position.
03:38:28Gives us at least an option
03:38:29for the aortic valve,
03:38:30but then we have to replace the
03:38:32pulmonary valve with another
03:38:34conduit or another prosthesis so
03:38:35it's still an imperfect option.
03:38:37We also don't have time to talk today,
03:38:39but Doctor Arner gearson he's
03:38:42done over 200 mitral valve robotic
03:38:45repair replacements here at Yale,
03:38:47and so this is something that I think
03:38:49that as a surgical program we're
03:38:51excited about because it offers us the
03:38:54ability to avoid a median sternotomy,
03:38:56especially for our patients
03:38:57who later on May need more.
03:38:59Surgical intervention and
03:39:00really you can see in this view.
03:39:02I mean you can see the mitral
03:39:04valve better than even through
03:39:06an open median sternotomy,
03:39:07but it doesn't necessarily change
03:39:09the valve replacement or repair
03:39:11options that we have for patients.
03:39:13And so I think the other concept
03:39:15that we really need to start thinking
03:39:17about specifically for our patients is
03:39:19looking at the valve leaflets themselves,
03:39:21and how can we recreate the
03:39:24function of native valve tissue?
03:39:26So Doctor Marcus crane.
03:39:28He actually I have to mention it
03:39:30comes to us from Munich, Germany.
03:39:32We're excited because he will be
03:39:34staying on his faculty here at Yale.
03:39:36He has been someone who goes around.
03:39:38Actually the entire world and country
03:39:41teaching surgeons about this technique.
03:39:43So in terms of the aortic
03:39:45valve replacement in general,
03:39:46we had mentioned have two options,
03:39:49but the limitations to these two
03:39:51options we really have to think about
03:39:53full anticoagulation for mechanical
03:39:54valve in exchange for durability and
03:39:57then having structural degeneration
03:39:58over time with the tissue valve.
03:40:00So we see ourselves going back and forth
03:40:03between these two imperfect options.
03:40:05We also don't have time to talk about it,
03:40:07but there are some aortic valve repair
03:40:09techniques that really have limitations,
03:40:11mostly with durability and also
03:40:14with anatomic limitations.
03:40:15So the Ozaukee procedure,
03:40:18I think in the ICU they said
03:40:19that this was a made up word,
03:40:21which it's not made up.
03:40:22It's actually named after the Japanese
03:40:25surgeon who created this procedure.
03:40:28And and I told them I said when
03:40:30you create a surgical procedure,
03:40:31they'll name something after you.
03:40:34So this is looking at the Ozaukee template.
03:40:37Looking at how,
03:40:38how do we create the ortic valve leaflets?
03:40:41So this is also now called the
03:40:44AB NEO technique,
03:40:44and NEO really means NEO customization.
03:40:47So we're able to take the template and
03:40:49we size each of the aortic valve cusps.
03:40:52So that's the left right and non
03:40:54coronary cusp and we're able to use
03:40:56autologous pericardium and treat it.
03:40:57So this is the patients.
03:40:58Bone tissue, then for each cusp.
03:41:01This gets future to the aortic
03:41:03valve annulus in the position that
03:41:06it would anatomically be.
03:41:07And looking at it once it's
03:41:10created through the aortic root,
03:41:12this is how it looks.
03:41:14This video is from Doctor Crane and from
03:41:16his they've done over 160 patients in Munich,
03:41:19Germany,
03:41:20and through the aortic root.
03:41:21Once you're on cardio pulmonary bypass,
03:41:23you excise the aortic valve leaflets.
03:41:25Then, each sizer is put in each of
03:41:27the cusps and the template is used to
03:41:30be able to create the pericardial.
03:41:32Neo cusp and it's a little
03:41:35bit like connect the dots.
03:41:38You put your sutures in place and you
03:41:41suture each separate cusp into the aortic
03:41:44valve annulus in that configuration,
03:41:47and then so the costs to each other
03:41:50and secure it to the aortic root.
03:41:52What this allows is actually
03:41:54preservation of the ortic valve annulus.
03:41:56It also allows the creation
03:41:59of the cups for coaptation.
03:42:02And also when you suture
03:42:04the two cups together,
03:42:05the valves are able to have a very
03:42:08large and long coaptation zone, so the.
03:42:14Learning curve I think takes a little bit.
03:42:17You can see that it is more
03:42:19challenging I think,
03:42:20and more difficult than putting in
03:42:23a mechanical or prosthetic valve.
03:42:25But as Doctor Crane has gone around
03:42:28and and even using this technique,
03:42:30for example in a truncus arteriosus
03:42:32or in other congenital heart
03:42:34disease that really needs another
03:42:36valve replacement repair technique,
03:42:38this is actually a short access
03:42:41image of after implantation,
03:42:43and you can see that there is
03:42:44no regurgitation.
03:42:45You know leakage and the competency
03:42:47is is very good.
03:42:50So one of the questions because
03:42:52this technique is relatively new is
03:42:54what is the long term durability,
03:42:55and unfortunately we will have to
03:42:57wait another five or ten years before
03:42:59I'll be able to report on that.
03:43:01But currently we have about midterm
03:43:04follow-up which is looking at really
03:43:06patients who've had this procedure
03:43:07done over three to five years,
03:43:09and this is all in adult patients
03:43:11here at Yale, we've been able to
03:43:14offer this procedure to 6 adults
03:43:16and one pediatric patient,
03:43:17and the freedom from REOPERATION is.
03:43:20Low we know this so far and the durability
03:43:23we think is also very good and very high,
03:43:26but this is limited limited time frame
03:43:30for follow-up because of how how long
03:43:33it's taken for us to adopt this technique.
03:43:36You talked about the advantages
03:43:38which I think you can see in the
03:43:41in the video and what I like about
03:43:44thinking about this type of procedure,
03:43:46especially for our adult congenital
03:43:48patients is that it?
03:43:49Even if the durability for this
03:43:51type of procedure is 10 years when
03:43:54we look at all the other.
03:43:56Options that we have now,
03:43:57so that includes transcatheter aortic
03:43:59valve replacement and that continues
03:44:01into a valve and valve even giving our
03:44:04patients another 10 or 15 years before that,
03:44:06and not excluding them from these
03:44:08other procedures that can offer
03:44:10us again some some time,
03:44:12which is usually what we need
03:44:14in our patients.
03:44:15Doctor Crane also gave me this video
03:44:19and what he really wanted to emphasize
03:44:21and this is one of the advantages is
03:44:23that throughout the cardiac cycle,
03:44:25the aortic valve annulus itself
03:44:27actually expands and contracts,
03:44:29and you can see that in his
03:44:31post repair video.
03:44:33He actually then states that because
03:44:36because this is naturally what
03:44:38occurs when we whenever we put in
03:44:41a tissue valve or mechanical valve,
03:44:44we're actually limiting and restricting.
03:44:46The motion to the aortic valve annulus,
03:44:48and so we think, and we have shown,
03:44:51that there's actually better
03:44:53hemodynamics with this type of repair.
03:44:55Also, we can use the patient's own tissue.
03:44:58Patients do not require full anticoagulation.
03:45:01They're on aspirin,
03:45:01and there is a low rate of
03:45:04permanent pacemaker implantation,
03:45:06and right now it is a good option
03:45:09in relation to other aortic
03:45:11valve repair techniques.
03:45:22So I think that when we look
03:45:24at Valve repair techniques,
03:45:26the Ozaki procedure makes sense to us because
03:45:29we're recreating the the valve leaflets.
03:45:31The core matrix trial is a little
03:45:33bit different and is looking at the
03:45:35tricuspid valve and also really we
03:45:37we're not recreating the leaflets or
03:45:39recreating the mechanism of the valve.
03:45:41Yale is currently a Center for
03:45:43this study that's ongoing.
03:45:45In pediatric patients looking at
03:45:48core matrix for the tricuspid valve
03:45:50and we also call this an ECM,
03:45:52an extracellular matrix.
03:45:54And So what is that?
03:45:56This is a material that's in
03:45:58a rectangular shape and it's a
03:46:00harvested from pig and Tustin,
03:46:01but then the pig cells are actually
03:46:03removed and so it's a scaffolding
03:46:05for native cells.
03:46:06This is used in other types of surgery.
03:46:08Other types of repair,
03:46:10not just in the heart but in our mind.
03:46:13We think of it as a matrix, a scaffolding.
03:46:16And so one of the potential benefits
03:46:18is that the patients native tissue can
03:46:21start to grow into the into the valve.
03:46:24This is an example of a case that
03:46:27this valve replacement was used,
03:46:29and this is a 2 year old female who
03:46:31had a history of a transitional AB
03:46:33canal and a dysplastic right sided 80 valve.
03:46:35How to repair an infancy?
03:46:38Take it off for one second.
03:46:39I have to.
03:47:30I just don't know where you left off.
03:47:32OK, we'll keep going.
03:47:35Thank you. So this is a case
03:47:38example for the core matrix,
03:47:39tricuspid valve and this was a
03:47:41patient who is now 2 years old with
03:47:43severe right sided regurgitation.
03:47:45After two attempts at repair so
03:47:47especially in a patient with this size,
03:47:49we're limited by the size of prostheses that
03:47:52are available and so this is an example
03:47:55of case that we can use the ECM matrix.
03:47:59So because it comes in a rectangular shape,
03:48:01we can customize this to the size based
03:48:04off of the annulus and we take the.
03:48:07A sheet and we roll it into a tubular
03:48:11structure and we suture the edge and
03:48:14then you have to turn the triangle in
03:48:17your head and you fix the two points
03:48:21of fixation into the right ventricle.
03:48:24This line shows you at the top where it opens
03:48:27up and then that is sutured to the annulus.
03:48:30So how does this work?
03:48:32In the thoracic world,
03:48:33there's something called the
03:48:35Heimlich Heimlich valve,
03:48:36and this is actually connected
03:48:37to a chest tube.
03:48:38This is for patients who have an
03:48:40air leak or persistent air leak,
03:48:42and when the pressure builds up in the
03:48:44chest and the air escapes out but then
03:48:46is unable to come back in because of
03:48:49this long tube that then collapses in.
03:48:50So if we think about this and we
03:48:52turn it and we put it into the heart,
03:48:54then this is how the core matrix valve works.
03:48:57The top is the right atrium,
03:48:59the bottom would be the right ventricle
03:49:01and the blood is allowed to come in.
03:49:02Through through the valve,
03:49:03but then it's unable to go to go back in.
03:49:06Given that long area of Coaptation
03:49:08and so we can see this,
03:49:10this is a core matrix valve that's
03:49:12been placed in the same patient
03:49:14and you can see the competency
03:49:15for this type of valve without
03:49:18actually creating the leaflets.
03:49:19This is just showing you the mechanism
03:49:22of the valve that we're able to achieve.
03:49:25So the advantages are that we
03:49:27can produce a competent valve.
03:49:28We can customize this and place it
03:49:30into a small size for our patients.
03:49:32There's also no requirement
03:49:34for full anticoagulation,
03:49:36and it's not considered a
03:49:37synthetic material because they've
03:49:39taken out all the other cells.
03:49:41Supposedly there's a potential for
03:49:43growth and also for remodeling
03:49:45because the patient's own cells
03:49:47can grow into this matrix as the
03:49:49patient grows and you can see that
03:49:52from our perspective in surgery we
03:49:54are still starting to think about.
03:49:56Out of best,
03:49:57come up with different tools that
03:49:59are creative for our patients that
03:50:01we can extend the years that we can
03:50:04offer them a good quality of life.
03:50:07I do want to mention that we do
03:50:09really all kinds of surgery here
03:50:11and I think as Rob showed,
03:50:13the adult congenital patients really are
03:50:16contributing to this increase in the
03:50:19amount of procedures that we're doing.
03:50:21Also, we what we do takes a lot
03:50:24of people to do that, and so I do
03:50:27want to thank Doctor Peter Gruber.
03:50:28He gave me the core matrix material.
03:50:31Also Doctor Marcus Crane,
03:50:32who is now going to join our faculty
03:50:34and lend his expertise with the Ozaki.
03:50:36Procedure and doctor Erner Gearson,
03:50:39who performs all the robotic cardiac surgery.
03:50:42There are more people than I can
03:50:45list here for us to be able to offer
03:50:48our excellent care to our pediatric
03:50:50surgical patients and everyone
03:50:52has been very supportive of me,
03:50:54so I'll steal from ALS talk to say that
03:50:56I feel like I'm in the right place at
03:50:58the right time and so I wanted to thank
03:51:00everyone for their help and support
03:51:02as I start my career here at Yale.
03:51:05So thank you very.
03:51:16Thank you Madonna. It's great to have
03:51:17you on board and you're definitely in
03:51:18the right place at the right time.
03:51:22Next, I'm pleased to introduce,
03:51:26although most of you all know him,
03:51:27Doctor Britton Keeshan who is
03:51:30my partner in the Cath lab.
03:51:32Written, trained with Tom Jones out in
03:51:35Seattle and then came to us and he's
03:51:38been a fantastic addition to the lab
03:51:40and he's going to speak to us about
03:51:42innovations in the cast space and
03:51:44where the future of Cath is going.
03:51:46And then I'm happy to say we'll
03:51:47be able to have lunch.
03:51:57I realize I'm standing between everybody
03:51:59and lunch and so for that I apologize.
03:52:02Let you know that I tried to trim my
03:52:05talk down from 122 slides to 114 slides.
03:52:11Guessing I just click.
03:52:19Maybe someone will click for me.
03:52:26Maybe I don't have to give the talk.
03:52:32Ah, there we go.
03:52:33Uh, so I'm going to speak to you guys
03:52:36about innovations in transcatheter
03:52:37therapies for congenital heart disease.
03:52:39I don't have any financial
03:52:41disclosures except to say that
03:52:42really there aren't many FDA approved
03:52:45devices in the congenital space.
03:52:47There are some which I will be talking about,
03:52:50but I will talk about some
03:52:51off label use of some devices.
03:52:54I think Jeremy laid the groundwork for the
03:52:57historical context of where we've come from,
03:52:59which helps us understand where we're going.
03:53:01But rather than talk about all the crazy.
03:53:04Innovations that we have in
03:53:06the past cat space.
03:53:07I think I'll talk about how innovation
03:53:09comes in multiple different forms,
03:53:11including pushing boundaries,
03:53:12looking at the management of the premium PDA,
03:53:15changing our paradigms for how we deal
03:53:18with RV outflow tract dysfunction,
03:53:20and then where the new frontiers
03:53:23are and how we're exploring those.
03:53:25So from a historical perspective.
03:53:28The congenital Cath space is in infancy.
03:53:32We only have a short 70 year history
03:53:35from the initial use of a ureteral
03:53:38catheter catheter to perform a
03:53:40pulmonary valvotomy in the 1950s,
03:53:43and it's only been 70 short years and
03:53:45now we have transcatheter valves devices
03:53:47to close ASD devices to close PDA,
03:53:50and I'm really excited about where the
03:53:52future is going to go from here so.
03:53:56I think I'd like to start by talking
03:53:59about how we're using kind of pushing
03:54:02boundaries with old technology and
03:54:05really pushing the envelope of taking
03:54:08care of our premature infants.
03:54:10I think everybody in the room knows
03:54:13about PDA's and how they're left
03:54:14to right shunt that results in
03:54:15pulmonary overcirculation,
03:54:16systemic hypoperfusion,
03:54:17and the PDA is poses a real problem
03:54:20in our neonatal population,
03:54:22with increased incidences of neck
03:54:25acute kidney injury.
03:54:26Worsening of bronchopulmonary dysplasia
03:54:28and so eliminating the PDA in this very
03:54:32vulnerable population is important,
03:54:34and the technique is really only
03:54:36been around since the 1990s,
03:54:37mainly initially through a surgical
03:54:41management which involves thoracoscopic
03:54:43technique, lateral thoracotomy,
03:54:45and complication rate.
03:54:47Even in the current era,
03:54:49remains high as high as 5%,
03:54:51so can we do better?
03:54:54And how we manage this?
03:54:55And I think a lot of the impetus
03:54:58for innovation has come from the
03:55:01fact that the complication rate is
03:55:03high and as a result our neonatology
03:55:05colleagues have turned away from
03:55:08performing a surgical ligation.
03:55:10So the end argument for Transcatheter
03:55:13PDA closure we've had tremendous
03:55:15success and evolution in the procedure
03:55:17initially described in the 1960s,
03:55:19but the initial technique,
03:55:23which involved large arterial and
03:55:24venous sheaths would preclude
03:55:26its use in smaller children.
03:55:28But there's been an evolution
03:55:30of devices over time.
03:55:31Doctor Hellenbrand participated
03:55:32in many of the initial trials,
03:55:34getting these devices up and
03:55:36writing running here at Yale,
03:55:38and it's become the standard
03:55:39of care for children.
03:55:40Greater than 6 kilos,
03:55:41but we like to push the boundaries
03:55:44of possibility and beginning
03:55:45in the early 2000 and 10s,
03:55:47many of our colleagues around
03:55:49the world started to try
03:55:50and use this technology in smaller
03:55:52and smaller infants, demonstrating
03:55:53that an infants as small as 700 grams.
03:55:56You could get good transcatheter closure
03:55:59with minimal complications and what
03:56:02this looks like is now a procedural
03:56:05success rate that approaches 9596%,
03:56:08so at least not inferior to.
03:56:11Surgery. And perhaps even better.
03:56:14In understanding how to treat this,
03:56:17we have to understand that the premature
03:56:20ductus is different than the ductus
03:56:22that exists in our one year olds.
03:56:25Most duct dye are this type,
03:56:28a conical ductus.
03:56:29It has somewhat of a tornado shape,
03:56:32but the premature and the devices
03:56:34were designed and made to fit
03:56:36that shape and that geometry.
03:56:38Unfortunately,
03:56:39the fetal ductus and the
03:56:41premature ductus is different.
03:56:42We call it now an F type ductus.
03:56:44It's tubular,
03:56:45it looks like a hockey stick and so
03:56:48our traditional devices that have
03:56:50that tornado like conical shape,
03:56:52really don't fit the bill here.
03:56:56And I think this is a a great story
03:56:59about how our field has collaborated
03:57:02with industry to take vascular plugs
03:57:05and really talk to the companies
03:57:08and develop a device that is
03:57:11specifically for this population.
03:57:12So I'm happy to report that through
03:57:17partnering with Amplatzer and Abbott,
03:57:19we've been engineered the Piccolo device,
03:57:22which really came into fruition
03:57:24in the later twenty 10s.
03:57:26And this is the first trial that
03:57:28looked at a a premature duck specific
03:57:31device and showed tremendous success
03:57:35with a 95.5% success rate in terms
03:57:39of implant with a relatively low
03:57:41complication rate, less than 2%,
03:57:46and so for the first time.
03:57:49We now have an FDA approved device for
03:57:53premature PDA closures here at Yale.
03:57:56We've also relied on using adult
03:57:59technology in the premium population,
03:58:02so the Medtronic microvascular plug
03:58:04is a device that we tend to use
03:58:06because of its soft nature and its
03:58:08ability to go through small sheaths.
03:58:10You can see that,
03:58:12similar to the Piccolo in relation to a dime,
03:58:14these devices are incredibly small
03:58:16and flexible and as a result we
03:58:18can get them into the premature
03:58:20infant with relative ease and
03:58:23not significant difficulty.
03:58:24So this is a catheter.
03:58:26A premature infant,
03:58:27less than a kilogram in size that
03:58:30we've been able to put a forefront
03:58:33sheath in direct A4 French
03:58:35catheter easily across the ductus,
03:58:37with the help of a wire.
03:58:39We do use fluoroscopy and echo
03:58:41guidance to help guide the procedure.
03:58:43Here's a picture of the ductus you
03:58:45can see that hockey stick shape
03:58:47on the angiogram on the left and
03:58:50we're able to place a device.
03:58:52Nicely within the ductus,
03:58:54it doesn't obstruct flow to either of
03:58:57the pulmonary arteries and on echocardiogram.
03:58:59Once the device is released,
03:59:01we see that we've relieved the.
03:59:05Have to write shunt and this is
03:59:07all we're left with no longer do
03:59:09we have a big lateral thoracotomy,
03:59:12but really,
03:59:12just a tiny little pinhole that
03:59:14doesn't even require a band aid.
03:59:16So just because we can doesn't
03:59:19mean that we should.
03:59:20I think there's an evolving body
03:59:22of literature that shows on some
03:59:24real promise in this technology.
03:59:26This paper looks at group of
03:59:29premature infants who's ducked.
03:59:31I were closed before 4 weeks versus
03:59:35later in life after eight weeks of age,
03:59:39they show that there's really
03:59:41decreased incidence of neck
03:59:44decrease incidence of sepsis.
03:59:46Uh. PVR or pulmonary vascular
03:59:49resistance is lower.
03:59:51The sooner that these ductus are closed and
03:59:56we improve the respiratory severity score.
03:59:58They're shorter times to
04:00:00extubation in these infants,
04:00:02and the amount of support that they need
04:00:06decreases more frequently or more rapidly.
04:00:09Similarly pictured here
04:00:11on a Kaplan Meier curve,
04:00:14and I think one of the exciting
04:00:17pieces of information that comes
04:00:19out of this paper is growth.
04:00:21These duck dye increase the
04:00:23caloric needs of these infants,
04:00:25particularly at critical times of growth.
04:00:28And when you close these duck
04:00:30die early earlier,
04:00:31you have a quicker weight
04:00:33gain that is more normal.
04:00:35At 25 grams per day compared to 14
04:00:37grams per day pre and I think that.
04:00:40Our NICU colleagues would corroborate
04:00:42that growth earlier on in development
04:00:44and in the NICU results in better
04:00:47outcomes overall and so I think
04:00:49that there's an evolving body of
04:00:52literature that supports us doing this.
04:00:54And here at Yale,
04:00:56I think we've managed to collaborate
04:00:58with our NICU colleagues and I think
04:01:01that before I arrived here in 2018
04:01:04there have been 4 surgical closures.
04:01:07Since that time we've done none.
04:01:09I'm happy to report.
04:01:11That in terms of our
04:01:13transcatheter PDA closure volume,
04:01:15it continues to increase,
04:01:17and now we're doing somewhere
04:01:19between one to three per month.
04:01:21I did 2 this week and doing one on Monday,
04:01:24so that volume continues
04:01:26to potentially increase,
04:01:28and I think that people are beginning to
04:01:31buy into doing these closures even earlier,
04:01:34so the referral that came in
04:01:36today is for a two week old,
04:01:38and I think that that is really
04:01:39going to make a big difference.
04:01:41All of these infants we've done
04:01:43infants as small as 670 grams,
04:01:44and I think that when you
04:01:46look around the country,
04:01:48I've talked to colleagues anecdotally who
04:01:50have done this down to about 400 grams,
04:01:52which you know,
04:01:53gives me some pause.
04:01:55But as people are doing it,
04:01:57I guess we'll become more
04:01:59and more comfortable with it,
04:02:00and I think that this is an area of
04:02:04innovation because we do transcatheter
04:02:06PDA closure at every age group here,
04:02:09and it's only recently that we're
04:02:11finally beginning to recommend it.
04:02:13Here at this premature population,
04:02:15it makes sense physiologically,
04:02:17and I think anatomically.
04:02:19So I think it's time to rethink that
04:02:22paradigm of not closing the PDA.
04:02:25Speaking of paradigms,
04:02:26I think R VOT disrupt dysfunction in
04:02:29congenital heart disease is a great story,
04:02:33especially in the Cath lab.
04:02:35So out of all congenital heart disease,
04:02:4022% of it includes dysfunction of
04:02:42the RBOT diagnosis like tetralogy of
04:02:45fellow critical PS, pulmonary atresia,
04:02:47attachment circular septum.
04:02:49And when you look at the.
04:02:50Lifespan of these patients you
04:02:52typically have to have initiation of PG
04:02:55E to stabilize pulmonary blood flow,
04:02:57then you have to surgically manage
04:02:59that with a BT shunt followed by
04:03:02either a complete repair or a conduit
04:03:04placement around six months of age,
04:03:07and then there are set up for
04:03:09needing multiple surgical revisions
04:03:10throughout the course of life.
04:03:11At least three.
04:03:12If they're conduits,
04:03:13you may even need to have
04:03:15further surgeries there,
04:03:16so you're looking at somewhere
04:03:18between 5:00 and 8:00.
04:03:20Economies over the course of
04:03:22a patient's life and so how do
04:03:24we innovate and eliminate that
04:03:26lifetime burden of surgical
04:03:28intervention and sternotomy?
04:03:29We've covered the history of the BT
04:03:31shunt a little bit here already,
04:03:33but obviously it has its drawbacks.
04:03:35With concern for Shannon collusion
04:03:38and the perioperative risk
04:03:40of morbidity and mortality,
04:03:43it's not a mortality free procedure,
04:03:48as people will obviously cite
04:03:50with as much as.
04:03:5216% mortality to discharge and
04:03:55the pulmonary atresia intact
04:03:57ventricular septum population.
04:03:59When you look at patients who need a
04:04:01BT shunt with chromosomal abnormalities
04:04:03for stabilization of pulmonary blood flow,
04:04:05that mortality risk increases to
04:04:08as much as 50%.
04:04:09So how can we do better?
04:04:11Well,
04:04:12early in the 1990s with the advent
04:04:14of more lower profile stents
04:04:16and people pushing the envelope
04:04:18in terms of the Cath lab,
04:04:20it was proposed that you could stent
04:04:22the neonatal ductus as opposed to
04:04:24included in the last set of patients
04:04:26that we were talking about and
04:04:28stabilize pulmonary blood flow that way.
04:04:31As this procedure has become
04:04:33more and more common,
04:04:35we have more and more data on this
04:04:38coming out of a registry from the
04:04:41UK that shows significant survival
04:04:44benefit when you look at stenting
04:04:46of the ductus versus BTT shunt.
04:04:50With significantly significantly
04:04:52improved survival,
04:04:54decreased risk for the need for
04:04:56ECMO following procedure, which is,
04:04:58I think, always good for patients.
04:04:59Despite how good you are, Madonna.
04:05:02Hadn't decreased length of stay
04:05:04further when you look at the
04:05:06potential complications of a surgical
04:05:08shunt versus that of a PDA stent.
04:05:10When you look at this list of
04:05:12complications and the surgical category
04:05:14it extends to really scary stuff,
04:05:15arrhythmias, cardiac arrest, ECMO,
04:05:18thrombosis, wound infection, et cetera.
04:05:20And when you look at the ductal stent,
04:05:22you're mostly looking at vascular
04:05:24access injury as the predominant
04:05:26form of complication,
04:05:27which I would argue is certainly less
04:05:31severe and something I would tolerate.
04:05:34This is a similar data from
04:05:36the US from the CIC,
04:05:38which is a collaboration of several
04:05:41hospitals looking at freedom from
04:05:43death or reintervention for cyanosis.
04:05:45Again,
04:05:46the PDA stent appears to be superior
04:05:49at least on univariate analysis,
04:05:51and when you look at propensity
04:05:54score adjusted analysis,
04:05:55the risk of procedural complications is
04:05:58less need for diuretic discharge is lower.
04:06:01ICU stay is shorter.
04:06:04And growth of the PA's as a result of
04:06:07the PDA stent appeared to be better,
04:06:09so I think.
04:06:10In the current era,
04:06:11PDA stenting is a reasonable opportunity
04:06:14to palliative therapy in these patients
04:06:16that need ductal dependent or that have
04:06:19ductal dependent pulmonary blood flow.
04:06:21And so the question then becomes,
04:06:22should it become the new standard of care?
04:06:24And while we don't know
04:06:26the answer to that yet,
04:06:27and at least appears to be non inferior
04:06:30and might ultimately prove to be superior,
04:06:32I think one of the great things about
04:06:35this community is that we're really
04:06:37trying to figure this out and I'm happy
04:06:39that the community has received NIH
04:06:41funding to launch the compass trial,
04:06:43which is launching this quarter this year,
04:06:46which is actually going to randomize patients
04:06:48to a surgical shunt versus PDA stent.
04:06:51Across the country and so single,
04:06:53similar to the single
04:06:54ventricle reconstruction trial,
04:06:55I think in three years we're going to have
04:06:57a definitive answer to this question,
04:06:59but it's just an example of how we in
04:07:01the Community continue to innovate.
04:07:03So we come back to our lifespan here
04:07:07and if we can eliminate the initial
04:07:10need for a surgical PDS BT shunt,
04:07:14we've eliminated at least least one
04:07:17sternotomy. But what about later in life?
04:07:19And I think that this is another example
04:07:21of how we're partnering with industry,
04:07:23the Natural History,
04:07:25I think of tetralogy flow repair is
04:07:27not something that people in this room
04:07:30are unfamiliar with, but essentially,
04:07:32when you do a transiently.
04:07:33Patch and you're left with chronic
04:07:36pulmonary regurgitation that leads
04:07:37to RV overload, dilation,
04:07:38and impaired systolic function.
04:07:40So ultimately we have to restore
04:07:43competence to that pulmonary valve,
04:07:44or at least where it would be.
04:07:47So traditionally this has been
04:07:49done in the operating room,
04:07:51and because of the widely variable and
04:07:54dynamic Physiology of these native ROTJ,
04:07:56it's been hard to come up
04:07:58with a percutaneous option.
04:07:59These outflow tracks come in
04:08:01various shapes and sizes.
04:08:03And this is where both Medtronic and Edwards
04:08:08have kind of listened to our community,
04:08:12and they've developed the latest generation
04:08:15of transcatheter pulmonary valves,
04:08:17which are harmony and Alterra,
04:08:20and these valves can now accommodate
04:08:24outflow tracks that were previously
04:08:26untenable with previous technology up
04:08:29to 28 to 38 millimeters in diameter.
04:08:33And I think partnering with our
04:08:34Advanced Imaging colleagues,
04:08:35we've really been able to understand
04:08:37how to best apply this technology in
04:08:40the real world and to real patients
04:08:42and bring them to the clinical need.
04:08:44We can use these dynamic systolic
04:08:47and diastolic CT scans to understand
04:08:49how the valve is going to interact
04:08:52with the RV OT during systole and
04:08:55diastole and identify the landing zone.
04:08:57We can use virtual reality software to
04:09:00actually see how the outflow track moves.
04:09:03During systole and diastole,
04:09:05and we can create artificial and
04:09:07virtual renderings of what our
04:09:09angiograms are going to look like.
04:09:11These are not actual angiograms of patients,
04:09:13they're simulated angiograms that have
04:09:16been created from the CT datasets,
04:09:20and we can see what these valves
04:09:21are now going to look like in the
04:09:23outflow tracks themselves.
04:09:24To understand how they're going to fit.
04:09:28The initial results following Harmony.
04:09:32The early feasibility trial showed
04:09:35really excellent results here,
04:09:37with 83% of patients having none to trace
04:09:43valve regurgitation over five years,
04:09:45but no more than mild.
04:09:48And that gave laid the groundwork
04:09:51for the pivotal trial,
04:09:52which we were a part of here at Yale,
04:09:54bringing the current
04:09:56generation of technology,
04:09:57TB 22 and TV 25 to market again.
04:10:02We only have one year follow up because
04:10:05the trial just concluded not too long ago.
04:10:08But at one year these valves are
04:10:12functioning incredibly well with
04:10:14very few complications or concerns.
04:10:16So I think in the current era we've
04:10:21expanded the treatment through
04:10:23innovation for patients with our
04:10:26patients with RBOT dysfunction,
04:10:28and we've taken what used to be 5 to 6.
04:10:31They're nominees.
04:10:32We've replaced them now in the 20s,
04:10:34and now we can do Valve and
04:10:36Valve within these valves,
04:10:37and hopefully we've taken the number
04:10:39of surgeries 5 to 6 open heart down
04:10:42to one to two over the patient's life,
04:10:44and I think that that's real innovation.
04:10:47I think the last thing I want
04:10:49to touch on is the new frontier.
04:10:51What's coming down the pipeline?
04:10:53What are we really excited about
04:10:55and people have always been excited
04:10:58about Bioresorbable devices?
04:10:59Metal stents pose problems for us.
04:11:02They're static.
04:11:02Once they're implanted,
04:11:03they need to be re dilated.
04:11:05With balloons,
04:11:06there are subject to complications such
04:11:09as thrombosis and instant stenosis.
04:11:11And when you implant these stents
04:11:13and little pediatric patients,
04:11:14they can't always be expanded
04:11:16up to adult sizes,
04:11:17leaving the Holy Grail being
04:11:19a biodegradable sent,
04:11:20that will disappear slowly over time,
04:11:22achieve its initial desire,
04:11:24opening up any narrowing,
04:11:26but then just kind of melt away,
04:11:29leaving natural tissue and natural vessel.
04:11:33This is a slide.
04:11:34I got a text from a buddy of mine or
04:11:36a peer over in Minnesota who's been
04:11:39working with the Chinese company.
04:11:40This is a iron based,
04:11:42bioresorbable stent that.
04:11:46Resorbs after approximately 2
04:11:49years in an RV outflow track.
04:11:52And I'm excited that we now have some
04:11:55bioresorbable technology here at Yale,
04:11:57so our traditional way of closing the
04:11:59ASD in patients was the amplatzer ASD
04:12:01occluder or the Gore septal occluder.
04:12:04You can see that it's a lot of metal,
04:12:05some Dacron mesh.
04:12:06It poses problems,
04:12:08particularly if this is implanted
04:12:10early in life and later in life.
04:12:13The patient develops A-fib or a
04:12:15flutter and an electrophysiologist
04:12:17needs to go access the left atrium.
04:12:19It's hard to get a catheter
04:12:20there when you put this big.
04:12:21Until the disk there.
04:12:24Enter the recept ASD occluder
04:12:28which is the latest buy resorbable
04:12:31ASD device on the market.
04:12:33It's the first and only on device
04:12:38that's metal free with these
04:12:42polyglycol RPGLE filaments.
04:12:44I'm not a scientist or an engineer,
04:12:48but all you've left with after two
04:12:52years following implantation are the Dacron.
04:12:55Patch which makes our
04:12:58electrophysiologists job a lot easier,
04:13:00so I'm happy to report that
04:13:04we were were part of the trial
04:13:06for this device and we did our
04:13:09first implant back in April,
04:13:11so a little less than two months ago.
04:13:13So I think bioresorbable
04:13:15technology is evolving.
04:13:17But it's here and we're excited
04:13:19to be part of that innovation.
04:13:21So with that,
04:13:22I think that we continue to evolve.
04:13:26Innovate, and we're going to use
04:13:28all the technology that we have
04:13:30at our finger necks fingertips to
04:13:32reduce morbidity and mortality.
04:13:34And I think they're more
04:13:36exciting things to come.
04:13:37Thank you.
04:13:48Thanks Britain.
04:13:52So we left time at the end for a
04:13:56question answer session, or you know,
04:13:59sort of time for people to.
04:14:01Reminisce a bit.
04:14:02There's also a lot of which is.
04:14:05Outside so I I don't know if there if
04:14:07people feel like they have questions
04:14:09that they'd like to pitch to anybody.
04:14:11Any of our speakers.
04:14:13We can do that for a couple of minutes and
04:14:15if not we can just mingle outside and.
04:14:18Eat. OK, let's mingle outside and eat.