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 ResearchMeSH KeywordsAdultChildDeath, Sudden, CardiacElectrocardiographyHeart RateHumansLong QT SyndromeRetrospective StudiesWilliams SyndromeConceptsSudden 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 details
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 ResearchMeSH KeywordsAdultElasticity Imaging TechniquesFontan ProcedureHemodynamicsHumansLiverLiver CirrhosisRetrospective StudiesConceptsAdult 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 controlsPatients
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 ResearchMeSH KeywordsAdolescentAdultAgedCalciumChildChild, PreschoolFemaleHumansHypercalcemiaInfantMaleMiddle AgedRetrospective StudiesWilliams SyndromeYoung AdultConceptsAcute 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 ResearchMeSH KeywordsAdultBlood Flow VelocityDiastoleEchocardiographyFontan ProcedureHumansMesenteric ArteriesProtein-Losing EnteropathiesRetrospective StudiesConceptsVelocity 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 obstructionCatheter‐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 defects
2014
Risk 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 ResearchMeSH KeywordsAdultFemaleFontan ProcedureHumansMalePalliative CarePostoperative ComplicationsRetrospective StudiesRisk FactorsTime FactorsConceptsMajor 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
Hemodynamic 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 cohortFeatures 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
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 dilatationPediatric Pulmonary Arterial Hypertension and Hyperthyroidism: A Potentially Fatal Combination
Trapp CM, Elder RW, Gerken AT, Sopher AB, Lerner S, Aranoff GS, Rosenzweig EB. Pediatric Pulmonary Arterial Hypertension and Hyperthyroidism: A Potentially Fatal Combination. The Journal Of Clinical Endocrinology & Metabolism 2012, 97: 2217-2222. PMID: 22622024, PMCID: PMC3387403, DOI: 10.1210/jc.2012-1109.Peer-Reviewed Original ResearchConceptsPulmonary arterial hypertensionAcute cardiopulmonary decompensationThyroid function testsCardiopulmonary decompensationAcute decompensationArterial hypertensionPAH patientsFunction testsPediatric pulmonary arterial hypertensionEmergency total thyroidectomySetting of hyperthyroidismStable PAH patientsTargeted PAH therapyRadioactive iodine ablationInstitutional review board approvalSymptoms of hyperthyroidismReview board approvalEmergency thyroidectomyPAH therapyIodine ablationTotal thyroidectomyΒ-blockadeAggressive treatmentPediatric patientsCardiopulmonary status