Featured Publications
Familial Recurrence Patterns in Congenitally Corrected Transposition of the Great Arteries: An International Study
Tortigue M, Nield LE, Karakachoff M, McLeod CJ, Belli E, Babu-Narayan SV, Prigent S, Boet A, Conway M, Elder RW, Ladouceur M, Khairy P, Kowalik E, Kalfa DM, Barron DJ, Mussa S, Hiippala A, Temple J, Abadir S, Le Gloan L, Lachaud M, Sanatani S, Thambo JB, Gronier CG, Amedro P, Vaksmann G, Charbonneau A, Koutbi L, Ovaert C, Houeijeh A, Combes N, Maury P, Duthoit G, Hiel B, Erickson CC, Bonnet C, Van Hare GF, Dina C, Karsenty C, Fournier E, Le Bloa M, Pass RH, Liberman L, Happonen JM, Perry JC, Romefort B, Benbrik N, Hauet Q, Fraisse A, Gatzoulis MA, Abrams DJ, Dubin AM, Ho SY, Redon R, Bacha EA, Schott JJ, Baruteau AE. Familial Recurrence Patterns in Congenitally Corrected Transposition of the Great Arteries: An International Study. Circulation Genomic And Precision Medicine 2022, 15: e003464. PMID: 35549293, DOI: 10.1161/circgen.121.003464.Peer-Reviewed Original ResearchMeSH KeywordsArteriesCiliary Motility DisordersCongenitally Corrected Transposition of the Great ArteriesHeart Defects, CongenitalHumansRetrospective StudiesTransposition of Great VesselsConceptsPrimary ciliary dyskinesiaGreat arteriesCongenital heart defectsCiliary dyskinesiaRecurrence patternsFamilial clustersHeart defectsRetrospective cohort studyCommon pathogenetic pathwayCongenitally Corrected TranspositionFirst-degree relativesLaterality defectsCohort studyAtrioventricular blockTertiary hospitalHeterotaxy syndromeCorrected TranspositionPathogenetic pathwaysUnknown causeCcTGARare diseaseArteryParental consanguinityDyskinesiaFamilial aggregationNoonan 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 ResearchMeSH KeywordsCesarean SectionFemaleHeart Defects, CongenitalHumansInfantInfant, NewbornNoonan SyndromePregnancyPregnancy OutcomePulmonary Valve StenosisRetrospective StudiesConceptsNoonan 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 ResearchMeSH KeywordsAdolescentAdultCommunicationContraceptionExerciseFemaleHeartHeart Defects, CongenitalHumansPregnancyConceptsCongenital 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
2019
Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation
Davey B, Toro‐Salazar O, Gauthier N, Valente A, Elder R, Wu F, Berman N, Pollack P, Lee J, Rathod R. Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation. Congenital Heart Disease 2019, 14: 1013-1023. PMID: 31642600, DOI: 10.1111/chd.12854.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAdultCardiologistsChildChild, PreschoolFontan ProcedureHealth Care SurveysHealth Services AccessibilityHealthcare DisparitiesHeart Defects, CongenitalHeart Function TestsHumansNew EnglandPractice Patterns, Physicians'Predictive Value of TestsReferral and ConsultationRisk FactorsTreatment OutcomeYoung AdultConceptsHigh-risk Fontan patientsFontan patientsCongenital cardiologistsLow riskMagnetic resonance imaging availabilityQuality of life testHigh-risk populationEvidence-based guidelinesFontan operationFontan procedureCardiac catheterizationScreening low-riskAnticoagulation regimensPractice of screeningFontanAspirin monotherapyCardiology associationsPatientsCardiac physiciansComplex patientsScreening practicesOutcome measuresCardiologistsYears of practiceDesigning future research studiesT-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 ResearchMeSH KeywordsCase-Control StudiesConnecticutFemaleHeart Defects, CongenitalHospitalizationHumansInfant, NewbornMaleNeonatal ScreeningReceptors, Antigen, T-CellSensitivity and SpecificityConceptsCongenital 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
Evaluation 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 ResearchMeSH KeywordsEchocardiographyFemaleHeart Defects, CongenitalHumansInfant, NewbornMaleNeonatal ScreeningOximetryPregnancyPrenatal DiagnosisProgram EvaluationConceptsCritical 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
“Frontiers in Fontan failure: A summary of conference proceedings”
Hebson C, Book W, Elder RW, Ford R, Jokhadar M, Kanter K, Kogon B, Kovacs AH, Levit RD, Lloyd M, Maher K, Reshamwala P, Rodriguez F, Romero R, Tejada T, Valente A, Veldtman G, McConnell M. “Frontiers in Fontan failure: A summary of conference proceedings”. Congenital Heart Disease 2016, 12: 6-16. PMID: 27597140, DOI: 10.1111/chd.12407.Peer-Reviewed Original ResearchMeSH KeywordsFontan ProcedureHeart Defects, CongenitalHumansLiverLiver DiseasesPalliative CareQuality of LifeRisk FactorsTime FactorsTreatment FailureClinical Approaches to the Patient with a Failing Fontan Procedure
Elder RW, Wu FM. Clinical Approaches to the Patient with a Failing Fontan Procedure. Current Cardiology Reports 2016, 18: 44. PMID: 27002620, DOI: 10.1007/s11886-016-0716-y.Peer-Reviewed Original ResearchMeSH KeywordsFontan ProcedureHeart Defects, CongenitalHeart VentriclesHumansOutcome Assessment, Health CarePractice Guidelines as TopicSocieties, MedicalVentricular DysfunctionConceptsClinical approachPotential therapeutic optionGeneral clinical approachLong-term healthFontan failureFontan populationFontan operationFontan procedureFontan survivorsFontan circulationUniventricular heartPatient presentingTherapeutic optionsOrgan systemsAltered physiologyVaried manifestationsVulnerable populationsMulti-institutional collaborationPatientsModern managementVast majorityExtracardiacPresentingPopulationCardiologistsCatheter‐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 ResearchMeSH KeywordsChildFemaleFollow-Up StudiesFontan ProcedureForecastingHeart Defects, CongenitalHeart VentriclesHumansMalePalliative CarePulmonary ArteryRetrospective StudiesTreatment OutcomeConceptsGlenn 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 studyThe Changing Epidemiology of Pediatric Endocarditis
Elder RW, Baltimore RS. The Changing Epidemiology of Pediatric Endocarditis. Infectious Disease Clinics Of North America 2015, 29: 513-524. PMID: 26311357, DOI: 10.1016/j.idc.2015.05.004.Peer-Reviewed Original ResearchConceptsCongenital heart diseaseInfective endocarditisHeart diseaseRheumatic heart diseaseManagement of childrenIntroduction of antibioticsPediatric EndocarditisSurgical interventionMicrobiologic changesCardiac interventionsViridans streptococciEarly detectionEndocarditisEarly predominanceEpidemiologyDiseaseInterventionVirtual disappearanceMortalityImmunologic 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
Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan
Mori M, Aguirre AJ, Elder RW, Kashkouli A, Farris AB, Ford RM, Book WM. Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan. Pediatric Cardiology 2014, 35: 569-579. PMID: 24531876, DOI: 10.1007/s00246-014-0881-y.Peer-Reviewed Original ResearchMeSH KeywordsAdultFontan ProcedureGlobal HealthHeart Defects, CongenitalHeart VentriclesHemodynamicsHumansHypertension, PortalIncidenceSurvival RateTreatment FailureConceptsAdult Fontan patientsFontan patientsCirculatory dysfunctionPortal hypertensionPostsinusoidal portal hypertensionMultiple organ involvementPulmonary blood flowFailing FontanClinical deteriorationFontan palliationHeart transplantationHepatic dysfunctionLate morbidityVentricular dysfunctionOrgan involvementUniventricular heartVentricular functionLiver diseaseHepatic perfusionPulmonary vasculatureCardiac functionLiver histopathologyBlood flowCirrhotic diseaseClinicopathologic states
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 ResearchMore 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 ResearchCarcinoma, HepatocellularFemaleFontan ProcedureHeart Defects, CongenitalHumansLiver NeoplasmsMalePostoperative Complications
2012
Cerebellar metastatic papillary thyroid carcinoma in a pediatric patient with complex congenital heart disease
Remy KE, Elder RW, Tsankova NM, Conroy RM. Cerebellar metastatic papillary thyroid carcinoma in a pediatric patient with complex congenital heart disease. Journal Of Pediatric Endocrinology And Metabolism 2012, 25: 1195-1199. PMID: 23329771, DOI: 10.1515/jpem-2012-0162.Peer-Reviewed Original ResearchConceptsPapillary thyroid carcinomaMetastatic papillary thyroid carcinomaComplex congenital heart diseaseCongenital heart diseaseHeart diseaseThyroid carcinomaFirst pediatric caseStrong risk factorDiagnostic radiation exposurePediatric patientsPresenting signPediatric casesRisk factorsCHARGE syndromeRadiation exposureCarcinomaDiseasePatientsSyndromeCancerCerebellum