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 ResearchConceptsPrimary 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 aggregationFrequency of QTc Interval Prolongation in Children and Adults with Williams Syndrome
Brink BD, Feinn R, Kozel BA, Billington CJ, Liu D, Yu E, Sindhar S, He J, Rouse C, Lampert R, Pober BR, Elder RW. Frequency of QTc Interval Prolongation in Children and Adults with Williams Syndrome. Pediatric Cardiology 2022, 43: 1559-1567. PMID: 35366065, DOI: 10.1007/s00246-022-02883-3.Peer-Reviewed Original ResearchConceptsSudden cardiac deathHeart rateBazett's formulaCardiac deathWilliams syndromeRetrospective chart reviewQTc interval prolongationBundle branch blockMean heart rateHigher heart rateChart reviewQTc prolongationInterval prolongationSinus rhythmBranch blockGeneral populationNormal rangePatientsQTcWS patientsQTcBEKGProlongationSyndromePotential causesNoonan 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 detailsHair and skin predict cardiomyopathies: Carvajal and erythrokeratodermia cardiomyopathy syndromes
Sun Q, Lee L, Hall EK, Choate KA, Elder RW. Hair and skin predict cardiomyopathies: Carvajal and erythrokeratodermia cardiomyopathy syndromes. Pediatric Dermatology 2020, 38: 31-38. PMID: 33275305, DOI: 10.1111/pde.14478.Peer-Reviewed Original ResearchConceptsCongestive heart failureCardiac evaluationHeart failureHeart diseaseSudden deathNail abnormalitiesCardiac pathologyNovel therapeuticsCardiomyopathy syndromeFatal consequencesYoung childrenPatientsWarning signsSyndromeFamily membersCardiocutaneous disorderSkinCharacteristic hairCardiomyopathyAbnormalitiesDiseasePathology
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
2022
Short-Term Outcomes After Myopericarditis Related to COVID-19 Vaccination
Pareek M, Steele J, Asnes J, Baldassarre L, Casale L, Desai N, Elder R, Faherty E, Ferguson I, Fishman R, Ghazizadeh Z, Glick L, Hall E, Khera R, Kokkinidis D, Kwan J, O'Marr J, Schussheim A, Tuohy E, Wang Y, Spatz E, Jacoby D, Miller E. Short-Term Outcomes After Myopericarditis Related to COVID-19 Vaccination. JACC Cardiovascular Imaging 2022, 15: 2002-2005. PMID: 36357140, PMCID: PMC9094612, DOI: 10.1016/j.jcmg.2022.03.026.Peer-Reviewed Case Reports and Technical Notes
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
A window into portal hemodynamics in adult fontan patients?
Simmons MA, Revzin M, To U, Liapakis A, Fahey J, Elder RW. A window into portal hemodynamics in adult fontan patients? International Journal Of Cardiology 2020, 323: 61-64. PMID: 32979426, DOI: 10.1016/j.ijcard.2020.09.047.Peer-Reviewed Original ResearchConceptsAdult Fontan patientsFontan patientsSpleen stiffnessCongenital heart diseaseLiver stiffnessShear wave elastographyControl patientsPortal hemodynamicsLiver diseaseHeart diseaseFontan-associated liver diseaseLimited subgroup analysisPortal venous hemodynamicsAssociated liver diseasePassive hepatic congestionUltrasound shear wave elastographyFontan circulationStudy cohortHealthy patientsHepatic congestionVenous hemodynamicsSubgroup analysisLiver fibrosisHealthy controlsPatientsCardio-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
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 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
“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 ResearchHypercalcemia 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 intervalsSuperior Mesenteric Arterial Flow Pattern is Associated with Major Adverse Events in Adults with Fontan Circulation
Mori M, Shioda K, Elder RW, Pernetz MA, Rodriguez FH, Rangosch A, Kogon BE, Book WM. Superior Mesenteric Arterial Flow Pattern is Associated with Major Adverse Events in Adults with Fontan Circulation. Pediatric Cardiology 2016, 37: 1013-1021. PMID: 27033246, DOI: 10.1007/s00246-016-1382-y.Peer-Reviewed Original ResearchConceptsVelocity time integralAdult Fontan patientsEnd-diastolic velocitySystolic/diastolic ratioMajor adverse eventsAdverse eventsMesenteric arterial flowFontan patientsFontan circulationResistive indexDiastolic ratioArterial flowSuperior mesenteric arterial flowDiastolic velocity-time integralAdult Fontan populationSystolic velocity time integralVenous outflow obstructionCox regression analysisPediatric Fontan patientsKaplan-Meyer analysisNon-event groupArterial flow patternsFontan populationAdult patientsOutflow obstructionClinical 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 ResearchConceptsClinical 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 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 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 disappearanceMortality