Featured Publications
Noonan syndrome and pregnancy outcomes
Chow CA, Campbell KH, Chou JC, Elder RW. Noonan syndrome and pregnancy outcomes. Cardiology In The Young 2022, 32: 1925-1929. PMID: 35034678, DOI: 10.1017/s104795112100514x.Peer-Reviewed Original ResearchConceptsNoonan syndromeCaesarean sectionHeart defectsLow-risk cardiac lesionsNeonatal intensive care unitIntra-uterine fetal demiseYale-New Haven HospitalMaternal cardiac diseaseMaternal cardiac dysfunctionSafety of pregnancyPulmonary valve stenosisLong-term morbidityRetrospective chart reviewTrial of laborIntensive care unitInterventricular septal thicknessPre-term birthTime of pregnancyAtrial septal defectStructural heart defectsMajority of mothersCongenital heart defectsWeeks of ageMaternal complicationsPregnancy details
2023
Unique clinical challenges for the teenager with congenital heart disease
Pastor T, Elder R. Unique clinical challenges for the teenager with congenital heart disease. Current Opinion In Pediatrics 2023, 35: 553-560. PMID: 37700620, DOI: 10.1097/mop.0000000000001281.Peer-Reviewed Original ResearchConceptsCongenital heart diseaseHeart diseaseHeart-healthy behaviorsUnderlying medical conditionsAdult-oriented careCareful history takingImportance of exerciseUnique clinical challengesImportant long-term benefitsMental health concernsOutpatient cardiologistCHD riskCHD patientsLong-term benefitsPotential complicationsWeight managementAppropriate screeningClinical challengeMedical conditionsHistory takingClinical practiceSexual healthCHD adolescentsHealth concernMotivational interviewing
2021
Impact of the new heart allocation policy on patients with restrictive, hypertrophic, or congenital cardiomyopathies
Chouairi F, Mullan CW, Sen S, Mori M, Fuery M, Elder RW, Lesse J, Norton K, Clark KA, Miller PE, Mulligan D, Formica R, Rogers JG, Jacoby D, Maulion C, Anwer M, Geirsson A, Desai NR, Ahmad T. Impact of the new heart allocation policy on patients with restrictive, hypertrophic, or congenital cardiomyopathies. PLOS ONE 2021, 16: e0247789. PMID: 33651802, PMCID: PMC7924739, DOI: 10.1371/journal.pone.0247789.Peer-Reviewed Original ResearchConceptsCongenital heart diseaseAllocation system changeHeart allocation systemNew heart allocation policyNew heart allocation systemTransplantation of patientsOrgan Sharing databasePost-transplantation outcomesPost-transplant survivalTime of transplantationMechanical circulatory supportRate of transplantationNumber of patientsHeart allocation policyNew allocation systemStatus 1ACardiac transplantAdult patientsSharing databaseWaitlist survivalCirculatory supportClinical benefitCongenital cardiomyopathyUnited NetworkCHD patients
2020
Cardio-obstetrics: Recognizing and managing cardiovascular complications of pregnancy
Shapero KS, Desai NR, Elder RW, Lipkind HS, Chou JC, Spatz ES. Cardio-obstetrics: Recognizing and managing cardiovascular complications of pregnancy. Cleveland Clinic Journal Of Medicine 2020, 87: 43-52. PMID: 31990654, DOI: 10.3949/ccjm.87a.18137.Peer-Reviewed Original ResearchConceptsPrimary pulmonary hypertensionCongenital heart disordersCardiovascular complicationsCollaborative interdisciplinary teamHypertensive disordersPeripartum cardiomyopathyPulmonary hypertensionValvular diseaseCardiovascular disordersPregnancyHeart disordersDisordersUnrecognized problemSpecialty trainingInterdisciplinary teamHypertensionComplicationsPatientsCardiomyopathyObstetricsCardiologistsDisease
2019
T-Cell Receptor Excision Circles in Newborns with Congenital Heart Disease
Davey B, Elder R, Cloutier M, Bennett N, Lee J, Wang Z, Manning A, Doan T, Griffiths M, Perez M, Ahluwalia N, Toro-Salazar O. T-Cell Receptor Excision Circles in Newborns with Congenital Heart Disease. The Journal Of Pediatrics 2019, 213: 96-102.e2. PMID: 31277900, DOI: 10.1016/j.jpeds.2019.05.061.Peer-Reviewed Original ResearchConceptsCongenital heart diseaseLower TREC levelsTREC levelsGeneral populationT-cell receptor excision circlesCongenital heart disease severityAssociated with clinical complicationsHeart diseaseCombination of prematurityRetrospective chart reviewNewborn screening programsPreterm childrenAssociated with hospitalizationExcision circlesT cellsChart reviewClinical complicationsNewbornsScreening programHigh riskChildren's hospitalInfectionOutcome measuresHospitalDisease
2017
Ventricular arrhythmias immediately following transcatheter pulmonary valve implantation: A cause for concern?
Simmons MA, Elder RW, Shabanova V, Hellenbrand W, Asnes J. Ventricular arrhythmias immediately following transcatheter pulmonary valve implantation: A cause for concern? Catheterization And Cardiovascular Interventions 2017, 91: 920-926. PMID: 29280541, DOI: 10.1002/ccd.27454.Peer-Reviewed Original ResearchConceptsTranscatheter pulmonary valve implantationRight ventricular outflow tractNative outflow tractsSignificant ventricular arrhythmiasPulmonary valve implantationVentricular arrhythmiasOutflow tractValve implantationAnti-arrhythmic medicationsBeta-blocker therapySingle-center studyVentricular outflow tractLong-term therapySudden cardiac deathCare of patientsChange of doseHemodynamic instabilityHemodynamic compromiseCardiac deathMedian ageMedical therapyPediatric patientsSignificant arrhythmiasCenter studyTerm therapyEvaluation of a Screening Program to Detect Critical Congenital Heart Defects in Newborns
Klausner R, Shapiro ED, Elder RW, Colson E, Loyal J. Evaluation of a Screening Program to Detect Critical Congenital Heart Defects in Newborns. Hospital Pediatrics 2017, 7: 214-218. PMID: 28250095, PMCID: PMC5924700, DOI: 10.1542/hpeds.2016-0176.Peer-Reviewed Original ResearchConceptsCritical congenital heart defectsPulse oximetryScreening programFalse-negative screening resultsMajority of infantsHospital health systemHealth System hospitalsCritical congenital heartYears of ageCongenital heart defectsCardiac lesionsPrenatal ultrasoundClinical concernHeart defectsCongenital heartEarly identificationSystem hospitalsInfantsNewbornsMost childrenEchocardiogramHealth systemLesionsScreening resultsAge intervals
2016
Hypercalcemia in Patients with Williams-Beuren Syndrome
Sindhar S, Lugo M, Levin MD, Danback JR, Brink BD, Yu E, Dietzen DJ, Clark AL, Purgert CA, Waxler JL, Elder RW, Pober BR, Kozel BA. Hypercalcemia in Patients with Williams-Beuren Syndrome. The Journal Of Pediatrics 2016, 178: 254-260.e4. PMID: 27574996, PMCID: PMC5085847, DOI: 10.1016/j.jpeds.2016.08.027.Peer-Reviewed Original ResearchConceptsAcute life-threatening eventsLife-threatening eventsPlasma calcium levelsWilliams-Beuren syndromeCalcium levelsHigh plasma calcium levelsOccurrence of hypercalcemiaMulticenter retrospective studyPediatric reference intervalsGastrointestinal complaintsRelevant comorbiditiesRetrospective studyRenal anomaliesSubgroup analysisCardiovascular diseaseQT intervalHypercalcemiaDisease processEvidence of dehydrationAvailable normative dataOlder individualsSmall childrenCalcium concentrationInverse correlationReference intervalsCatheter‐measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End‐organ Dysfunctions
Mori M, Hebson C, Shioda K, Elder RW, Kogon BE, Rodriguez FH, Jokhadar M, Book WM. Catheter‐measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End‐organ Dysfunctions. Congenital Heart Disease 2016, 11: 589-597. PMID: 26932151, DOI: 10.1111/chd.12345.Peer-Reviewed Original ResearchMeSH KeywordsAdultCardiac CatheterizationChi-Square DistributionFemaleFontan ProcedureGeorgiaHeart Defects, CongenitalHeart FailureHeart TransplantationHemodynamicsHumansKidney DiseasesLinear ModelsLiver DiseasesMaleMultivariate AnalysisPredictive Value of TestsProportional Hazards ModelsRetrospective StudiesRisk FactorsTime FactorsTreatment OutcomeYoung AdultConceptsPulmonary capillary wedge pressureHigh central venous pressureCentral venous pressureEnd-organ dysfunctionSystemic vascular resistance indexCox proportional hazards analysisAdult Fontan patientsProportional hazards analysisCardiac indexFontan patientsAdverse eventsFontan circulationHemodynamic measurementsHigh systemic vascular resistance indexMultivariate Cox proportional hazards analysisRight heart catheterization dataSignificant associationLow cardiac indexVascular resistance indexCapillary wedge pressureChild-Pugh scoreSerious adverse eventsVenous outflow obstructionHigher cardiac indexPost-Fontan patients
2015
Half a Century’s Experience With the Superior Cavopulmonary (Classic Glenn) Shunt
Zahr RA, Kirshbom PM, Kopf GS, Sainathan S, Steele MM, Elder RW, Karimi M. Half a Century’s Experience With the Superior Cavopulmonary (Classic Glenn) Shunt. The Annals Of Thoracic Surgery 2015, 101: 177-182. PMID: 26603022, DOI: 10.1016/j.athoracsur.2015.08.018.Peer-Reviewed Original ResearchConceptsGlenn shuntPulmonary arteriovenous fistulaClassic Glenn shuntArteriovenous fistulaOriginal cohortSuperior cavopulmonary shuntMedian overall survivalEarly postoperative periodPermanent pacemaker placementSingle ventricle patientsSingle-ventricle palliationSinus node dysfunctionTwo-ventricle repairImportant outcome measureElectronic medical recordsCavopulmonary shuntLate deathsMedian survivalAtresia groupOverall survivalPostoperative periodBidirectional GlennPacemaker placementAtrial tachyarrhythmiasRetrospective studyModern Incidence of Complete Heart Block in Patients with L‐looped Ventricles: Does Univentricular Status Matter?
Simmons MA, Rollinson N, Fishberger S, Qin L, Fahey J, Elder RW. Modern Incidence of Complete Heart Block in Patients with L‐looped Ventricles: Does Univentricular Status Matter? Congenital Heart Disease 2015, 10: e237-e242. PMID: 26077963, DOI: 10.1111/chd.12279.Peer-Reviewed Original ResearchConceptsComplete heart blockSpontaneous complete heart blockSingle ventricle anatomyHeart blockL-transpositionGreat arteriesVentricle anatomyYale-New Haven HospitalGroup III patientsTwo-ventricle anatomyRetrospective cohort studyMajor cardiac defectsAge of onsetSized ventriclesVentricle patientsCohort studyMedian ageModern cohortPerson yearsIII patientsIncidence rateSimilar riskPatientsArteryCardiac defectsImmunologic Aging in Adults with Congenital Heart Disease: Does Infant Sternotomy Matter?
Elder RW, George RP, McCabe NM, Rodriguez III FH, Book WM, Mahle WT, Kirk AD. Immunologic Aging in Adults with Congenital Heart Disease: Does Infant Sternotomy Matter? Pediatric Cardiology 2015, 36: 1411-1416. PMID: 25916315, PMCID: PMC5319831, DOI: 10.1007/s00246-015-1174-9.Peer-Reviewed Original ResearchConceptsAdult congenital heart diseaseCongenital heart diseaseACHD patientsHeart diseaseExact testMean percentageImmune phenotypic changesT lymphocyte compositionCD8 T cellsFisher's exact testPolychromatic flow cytometryT cell maturationMann-Whitney ULower mean percentageImmune exhaustionImmunologic exhaustionPrior sternotomyCD8 lymphocytesCD4 cellsCD8 cellsLymphocyte compositionMedian ageClinical impactT cellsT lymphocytes
2014
Adult Fontan failure — Distinct disease entity
Mori M, Elder RW, Book WM. Adult Fontan failure — Distinct disease entity. International Journal Of Cardiology 2014, 177: 650. PMID: 25449472, DOI: 10.1016/j.ijcard.2014.09.177.Peer-Reviewed Original ResearchRisk Factors for Major Adverse Events Late after Fontan Palliation
Elder RW, McCabe NM, Veledar E, Kogon BE, Jokhadar M, Rodriguez FH, McConnell ME, Book WM. Risk Factors for Major Adverse Events Late after Fontan Palliation. Congenital Heart Disease 2014, 10: 159-168. PMID: 25130602, PMCID: PMC4512755, DOI: 10.1111/chd.12212.Peer-Reviewed Original ResearchConceptsMajor adverse eventsAdult Fontan patientsAdverse eventsRisk factorsFontan patientsFontan palliationOxygen desaturationPortal hypertensionVentricular functionAdverse outcomesTransplant-free survival rateLate-term outcomesSystemic oxygen desaturationSystolic ventricular functionAdverse event profileCongestive heart failureKaplan-Meier methodTertiary academic centerAdditional risk factorsImportant risk factorMode of deathFontan failureFontan typeSystemic ventricleThromboembolic events
2013
Learning From Improbable Longevity
Elder RW. Learning From Improbable Longevity. JAMA 2013, 310: 2041-2042. PMID: 24240928, DOI: 10.1001/jama.2013.276685.Peer-Reviewed Original ResearchHemodynamic Phenotype of the Failing Fontan in an Adult Population
Hebson CL, McCabe NM, Elder RW, Mahle WT, McConnell M, Kogon BE, Veledar E, Jokhadar M, Vincent RN, Sahu A, Book WM. Hemodynamic Phenotype of the Failing Fontan in an Adult Population. The American Journal Of Cardiology 2013, 112: 1943-1947. PMID: 24075283, PMCID: PMC4505550, DOI: 10.1016/j.amjcard.2013.08.023.Peer-Reviewed Original ResearchConceptsFontan failureCardiac indexFontan patientsLiver diseaseSVR indexLower systemic vascular resistance indexNormal systolic ventricular functionSystemic vascular resistance indexAdult Fontan patientsSimilar cardiac indexSystolic ventricular functionVascular resistance indexSignificant liver diseasePediatric Fontan patientsCase-cohort analysisFailing FontanPF cohortsPortal hypertensionAdult patientsCatheterization findingsFontan circulationHeart failureVentricular functionCirculatory derangementsPediatric cohortMore on Hepatocellular Carcinoma after the Fontan Procedure
Elder RW, Parekh S, Book WM. More on Hepatocellular Carcinoma after the Fontan Procedure. New England Journal Of Medicine 2013, 369: 490. PMID: 23902510, DOI: 10.1056/nejmc1306854.Peer-Reviewed Original ResearchFeatures of portal hypertension are associated with major adverse events in Fontan patients: The VAST study
Elder RW, McCabe NM, Hebson C, Veledar E, Romero R, Ford RM, Mahle WT, Kogon BE, Sahu A, Jokhadar M, McConnell ME, Book WM. Features of portal hypertension are associated with major adverse events in Fontan patients: The VAST study. International Journal Of Cardiology 2013, 168: 3764-3769. PMID: 23849105, PMCID: PMC3805740, DOI: 10.1016/j.ijcard.2013.06.008.Peer-Reviewed Original ResearchConceptsMajor adverse eventsPortal hypertensionAdverse eventsPost-Fontan patientsFontan patientsVAST scoresSingle-ventricle palliationFuture prospective studiesCongestive hepatopathyEarly referralHeart transplantRetrospective reviewClinical manifestationsProspective studyHepatic fibrosisClinical significanceHypertensionLiver evaluationPatientsLogistic regressionTransplantScoresSignificant relationshipPalliationFontan“Double whammy”: anomalous pulmonary and systemic venous drainage in a patient with scimitar syndrome
Elder RW, Kogon BE, Sahu A. “Double whammy”: anomalous pulmonary and systemic venous drainage in a patient with scimitar syndrome. Cardiology In The Young 2013, 23: 738-739. PMID: 23987117, DOI: 10.1017/s1047951113000553.Peer-Reviewed Original Research
2012
Outcomes of the infant Ross procedure for congenital aortic stenosis followed into adolescence
Elder RW, Quaegebeur JM, Bacha EA, Chen JM, Bourlon F, Williams IA. Outcomes of the infant Ross procedure for congenital aortic stenosis followed into adolescence. Journal Of Thoracic And Cardiovascular Surgery 2012, 145: 1504-1511. PMID: 23062969, PMCID: PMC3657341, DOI: 10.1016/j.jtcvs.2012.09.004.Peer-Reviewed Original ResearchConceptsCongenital aortic stenosisRoss procedureLong-term outcomesAortic stenosisRight ventricular outflow tract reinterventionVentricular outflow tract reinterventionRight ventricular outflow tractSignificant aortic stenosisVentricular outflow tractAortic valve diseaseNew York-PresbyterianAvoidance of anticoagulationCatheter-based interventionsMonths of ageAutograft annulusAutograft growthAutograft reinterventionAortic insufficiencyLatest followMedian ageRetrospective reviewRoss operationCardiothoracic centreRecent followModerate dilatation