Hereditary hemorrhagic teleangiectasia (HHT) is a hereditary disorder where blood vessels in certain parts of a person’s body lack capillaries. Capillaries are tiny blood vessels that allow blood to flow through tissues from the higher pressure arteries into lower pressure veins. Without capillaries, blood flows more forcefully from arteries into veins and in areas where this occurs, patients are prone to bleeding. Nosebleeds are often a sign that a patient may have HHT. Larger arteriovenous malformations can occur in the gastrointestinal tract, the liver, lungs or brain. While 40% of patients with HHT have AVMs in their lungs and brain, screening and treatment can prevent life threatening events.
In 1991, Robert I. White Jr., MD, Yale New Haven Hospital and the Yale School of Medicine, developed the concept of an HHT center. Dr. White and his associates have set up over 20 HHT centers worldwide. On the 20th anniversary of the Yale HHT Center (ww.hhtavm.org), Dr. White, Dr. Jeffrey Pollak and Dr. John Fahey performed live cases for the Pediatric and Adult Interventional Cardiac Symposium (http://www.picsymposium.com) which was held in Boston.
Two patients with HHT and pulmonary AVMs were treated in an HVC interventional radiology lab. Using a variety of catheters threaded through the patients’ femoral veins, the AVMs were embolized or had their blood supply shut off by means of platinum coils or plugs. Embolization of pulmonary malformations reduces the risk of stroke and serious infection. These therapeutic fluoroscopic procedures were part of a teleconference for the four-day Cardiac Symposium.
The audience in Boston – 800 pediatric and adult interventional cardiologists and radiologists – was able to view live case demonstrations from Saudi Arabia, Brazil, Germany, Montreal, Canada, Miami, Florida, St. Louis, Missouri, Boston, Massachusetts and Yale. The Yale physicians answered questions from the audience and Drs. White, Pollak and Fahey emphasized the importance of being familiar with a number of techniques for treating pulmonary malformations as well as delivering coordinated multidisciplinary care to patients with HHT.
Dr. White started the HHT Foundation, Inc. (www.hht.org) in 1991 in New Haven with 15 patients. Today, the Yale HHT Center follows three thousand patients with HHT. Dr. White is retiring from doing procedures this year and the HVC is proud to have this world-renowned physician on its medical staff.