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 causesT cell response to influenza vaccination remains intact in adults with congenital heart disease who underwent early thymectomy
Leone DM, Park HJ, Unlu S, Gurvitz M, Kang I, Elder RW. T cell response to influenza vaccination remains intact in adults with congenital heart disease who underwent early thymectomy. International Journal Of Cardiology Congenital Heart Disease 2022, 8: 100359. PMID: 35600131, PMCID: PMC9122016, DOI: 10.1016/j.ijcchd.2022.100359.Peer-Reviewed Original ResearchT cell responsesCongenital heart diseaseT cellsInfluenza vaccinationEarly thymectomyMemory CD4Heart diseaseAdequate T-cell responsesCell responsesT cell distributionT cell subsetsNaïve T cellsCongenital heart surgeryYears of ageHemagglutination inhibition assaysRoutine vaccinationVaccine immunityCell subsetsMean ageHeart surgeryNaïve CD4Healthy controlsThymectomyBlood samplesDiminished responseNoonan 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
2024
Life and Death: A Multicenter Study Evaluating Cardiologists’ Approach to Difficult Conversations with Fontan Patients and Families
Lee S, Rathod R, Valente A, Davey B, Wu F, Drucker N, Lombardi K, St. Clair N, Azcue N, Toro-Salazar O, Elder R. Life and Death: A Multicenter Study Evaluating Cardiologists’ Approach to Difficult Conversations with Fontan Patients and Families. Pediatric Cardiology 2024, 1-8. PMID: 39164409, DOI: 10.1007/s00246-024-03631-5.Peer-Reviewed Original ResearchPatient-related barriersGoals of carePhysician demographic dataProvider-relatedEnrollment of individualsPerceived barriersRisk counselingLongitudinal careMental healthThematic approachYears post fellowshipFontan patientsLack of familiarityAdverse event riskDifficult conversationsOutpatient cardiologistPsychosocial stressorsNarrative commentsSignificant barriersAdolescent/young adultPatient-relatedSocial barriersDemographic dataCardiologists' attitudesCare
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 modelRiskMonthsYearsRegurgitationUnique 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 interviewingFontan-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
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
Robotic mitral valve repair in a patient with cardiac dextroversion
Bourdillon A, Elder R, Lalonde M, Steele J, Gruber PJ, Geirsson A. Robotic mitral valve repair in a patient with cardiac dextroversion. JTCVS Techniques 2021, 11: 12-16. PMID: 35169722, PMCID: PMC8828793, DOI: 10.1016/j.xjtc.2021.10.031.Peer-Reviewed Case Reports and Technical NotesDiagnosis, 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 presentationPatientsVentricleImpact 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 measuresHospitalDiseaseModern 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 entitiesHIGH PREVALENCE OF PROLONGED QTC IN ADULTS AND CHILDREN WITH WILLIAMS SYNDROME
Elder R, Brink B, Feinn R, Sindhar S, Kozel B, Yu E, Lampert R, He B, Rouse C, Pober B. HIGH PREVALENCE OF PROLONGED QTC IN ADULTS AND CHILDREN WITH WILLIAMS SYNDROME. Journal Of The American College Of Cardiology 2019, 73: 640. DOI: 10.1016/s0735-1097(19)31248-3.Peer-Reviewed Original Research
2018
Postpartum anticoagulation in women with mechanical heart valves
Irani RA, Santa-Ines A, Elder RW, Lipkind HS, Paidas MJ, Campbell KH. Postpartum anticoagulation in women with mechanical heart valves. International Journal Of Women's Health 2018, 10: 663-670. PMID: 30498374, PMCID: PMC6207249, DOI: 10.2147/ijwh.s177547.Peer-Reviewed Original ResearchMechanical heart valvesPregnant womenPostpartum anticoagulationHemorrhagic complicationsHeart valvesNeonatal intensive care unitAC managementYale-New Haven HospitalImmediate neonatal outcomeIntra-abdominal bleedingMedian gestational ageSignificant maternal morbidityIntra-abdominal hematomaIntensive care unitEvidence-based guidelinesEvidence-based recommendationsAC therapyDelivery hospitalizationsMaternal complicationsMaternal strokeMaternal morbidityMaternal outcomesNeonatal outcomesChart reviewRetrospective cohort