Featured Publications
Frequency 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 causes
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
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 controlsPatients
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 studies
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 therapy
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 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 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
Modern 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
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 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 eventsBeyond 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 ResearchConceptsAdult 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 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 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“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