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 causesHair 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
How Good Are Cardiologists at Predicting Major Adverse Events in Fontan Patients?
Elder R, Valente A, Davey B, Wu F, Drucker N, Lombardi K, Lee S, McCollum S, Shabanova V, St. Clair N, Azcue N, Toro-Salazar O, Rathod R. How Good Are Cardiologists at Predicting Major Adverse Events in Fontan Patients? JACC Advances 2023, 3: 100736. PMID: 38939804, PMCID: PMC11198720, DOI: 10.1016/j.jacadv.2023.100736.Peer-Reviewed Original ResearchMajor adverse eventsBeta-blocker useFontan patientsSimple kappa coefficientAdverse eventsAtrioventricular valve regurgitationBlocker useSystolic dysfunctionCardiac admissionsValve regurgitationRisk cohortMulticenter studyPatientsResults OverallLateral tunnelLinear mixed effects modelsCardiologistsFontanKappa coefficientMixed effects modelsEffects modelRiskMonthsYearsRegurgitationFontan-related morbidity and paced QRS duration: A case series
Keaney J, Simmons M, Elder R, Beach C. Fontan-related morbidity and paced QRS duration: A case series. Progress In Pediatric Cardiology 2023, 69: 101641. DOI: 10.1016/j.ppedcard.2023.101641.Peer-Reviewed Original Research
2021
Diagnosis, surgical management, and long-term outcomes of double chambered right ventricle: a rare and challenging disease in the adult population
Otero-Luna A, Ghiroli S, Hsia T, Fahey J, Elder R. Diagnosis, surgical management, and long-term outcomes of double chambered right ventricle: a rare and challenging disease in the adult population. Progress In Pediatric Cardiology 2021, 62: 101417. DOI: 10.1016/j.ppedcard.2021.101417.Peer-Reviewed Original ResearchDouble chambered right ventricleHistory of ventricular septal defectVentricular septal defectRight ventricleSeptal defectSurgical repairRare diseaseRight ventricular inflowPost-operative resultsPre-operative assessmentLong-term outcomesYears of ageSurgical outcomesEchocardiographic studiesSurgical managementVentricular inflowPostoperative periodInstitutional experienceClinical statusResidual gradientClinical dataPatient populationIncidental presentationPatientsVentricle
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 studiesModern Day Care of Patients With Single Ventricle Heart Disease: Late Complications of Fontan Palliation
Simmons M, Elder R. Modern Day Care of Patients With Single Ventricle Heart Disease: Late Complications of Fontan Palliation. Current Pediatrics Reports 2019, 7: 53-61. DOI: 10.1007/s40124-019-00192-7.Peer-Reviewed Original ResearchFontan palliationSingle ventricle heart diseaseLate hepatic complicationsHepatic complicationsPulmonary vasodilatorsReviewThe majorityFontan failureVascular complicationsFontan operationLate complicationsHeart failureHeart diseaseLymphatic complicationsNovel therapiesLiver cancerHigh riskComplicationsRecent FindingsRecentScoring systemPatientsDay carePalliationTherapyStructural complicationsSeparate entities
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 ResearchClinical 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 majorityExtracardiacPresentingPopulationCardiologists
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 defects
2013
Features 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
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 exposureCarcinomaDiseasePatientsSyndromeCancerCerebellumPulmonary Venous Obstruction in the Atrial Switch Operation: A Forgotten Complication
Elder RW, Hellenbrand WE. Pulmonary Venous Obstruction in the Atrial Switch Operation: A Forgotten Complication. Pediatric Cardiology 2012, 33: 1183-1186. PMID: 22354225, DOI: 10.1007/s00246-012-0241-8.Peer-Reviewed Original ResearchConceptsPulmonary venous obstructionVenous obstructionObstructive heart diseaseSymptoms of dyspneaAtrial switch operationChest X-rayContext of patientsForgotten ComplicationMustard surgeryRare complicationCardiac catheterizationSurgical managementGreat arteriesHeart diseaseBaffle obstructionSitus inversusComplicationsObstructionSwitch operationSymptomsCurrent literatureDyspneaCcTGACatheterizationPatients