2024
Effect of Aficamten on Cardiac Structure and Function in Obstructive Hypertrophic Cardiomyopathy SEQUOIA-HCM CMR Substudy
Masri A, Cardoso R, Abraham T, Claggett B, Coats C, Hegde S, Kulac I, Lee M, Maron M, Merkely B, Michels M, Olivotto I, Oreziak A, Jacoby D, Heitner S, Kupfer S, Malik F, Meng L, Solomon S, Wohltman A, Kwong R, Kramer C, Investigators S. Effect of Aficamten on Cardiac Structure and Function in Obstructive Hypertrophic Cardiomyopathy SEQUOIA-HCM CMR Substudy. Journal Of The American College Of Cardiology 2024, 84: 1806-1817. PMID: 39217563, DOI: 10.1016/j.jacc.2024.08.015.Peer-Reviewed Original ResearchCardiovascular magnetic resonanceObstructive hypertrophic cardiomyopathyOutflow tract obstructionReduce cardiovascular eventsCardiac remodelingAtrial fibrillationHeart failureCardiovascular eventsAssociated with progressive heart failureLeft ventricular (LV) hypertrophyPhase 3 double-blindSymptomatic obstructive hypertrophic cardiomyopathyLV outflow tract obstructionMaximal LV wall thicknessLeft atrial volume indexAtrial volume indexLV mass indexLV) hypertrophyPlacebo-controlled trialProgressive heart failureFavorable cardiac remodelingLeft atrial dilatationCardiac myosin inhibitorLeft atrial sizeLV wall thickness
2021
Predictors of Major Atrial Fibrillation Endpoints in the National Heart, Lung, and Blood Institute HCMR
Kramer CM, DiMarco JP, Kolm P, Ho CY, Desai MY, Kwong RY, Dolman SF, Desvigne-Nickens P, Geller N, Kim DY, Maron MS, Appelbaum E, Jerosch-Herold M, Friedrich MG, Schulz-Menger J, Piechnik SK, Mahmod M, Jacoby D, White J, Chiribiri A, Helms A, Choudhury L, Michels M, Bradlow W, Salerno M, Dawson DK, Weinsaft JW, Berry C, Nagueh SF, Buccarelli-Ducci C, Owens A, Casadei B, Watkins H, Weintraub WS, Neubauer S, Investigators H. Predictors of Major Atrial Fibrillation Endpoints in the National Heart, Lung, and Blood Institute HCMR. JACC Clinical Electrophysiology 2021, 7: 1376-1386. PMID: 34217663, PMCID: PMC8605982, DOI: 10.1016/j.jacep.2021.04.004.Peer-Reviewed Original ResearchConceptsBody mass indexSevere mitral regurgitationHistory of arrhythmiaMitral regurgitationHypertrophic cardiomyopathyAtrial fibrillationLA volumeProspective natural history studyVolume indexHigher body mass indexAtrial volume indexLA volume indexPermanent atrial fibrillationOlder adult patientsStrong risk factorNatural history studiesCommon morbidityAdult patientsElectrical cardioversionYounger patientsCatheter ablationEndpoint eventsMass indexNational HeartRisk factorsClinical characteristics and outcomes in childhood-onset hypertrophic cardiomyopathy
Marston NA, Han L, Olivotto I, Day SM, Ashley EA, Michels M, Pereira AC, Ingles J, Semsarian C, Jacoby D, Colan SD, Rossano JW, Wittekind SG, Ware JS, Saberi S, Helms AS, Ho CY. Clinical characteristics and outcomes in childhood-onset hypertrophic cardiomyopathy. European Heart Journal 2021, 42: 1988-1996. PMID: 33769460, PMCID: PMC8139852, DOI: 10.1093/eurheartj/ehab148.Peer-Reviewed Original ResearchConceptsChildhood-onset hypertrophic cardiomyopathyLife-threatening ventricular arrhythmiasHeart failureHypertrophic cardiomyopathyAtrial fibrillationVentricular arrhythmiasComposite endpointHCM patientsRisk of HFSarcomeric Human Cardiomyopathy RegistryNatural historyAdvanced HF therapiesOverall composite outcomeObservational cohort studyClinical risk stratificationHypertrophic cardiomyopathy patientsAge of diagnosisVentricular assist deviceAdult-onset diseaseHF therapyClinical characteristicsCohort studyComposite outcomeBaseline visitRisk stratification
2020
Hypertrophic Cardiomyopathy with Left Ventricular Systolic Dysfunction: Insights from the SHaRe Registry
Marstrand P, Han L, Day SM, Olivotto I, Ashley EA, Michels M, Pereira AC, Wittekind SG, Helms A, Saberi S, Jacoby D, Ware JS, Colan SD, Semsarian C, Ingles J, Lakdawala NK, Ho CY. Hypertrophic Cardiomyopathy with Left Ventricular Systolic Dysfunction: Insights from the SHaRe Registry. Circulation 2020, 141: 1371-1383. PMID: 32228044, PMCID: PMC7182243, DOI: 10.1161/circulationaha.119.044366.Peer-Reviewed Original ResearchConceptsLeft ventricular systolic dysfunctionVentricular ejection fractionComposite outcomeHypertrophic cardiomyopathyNatural historyEjection fractionLeft ventricular cavity sizeVentricular systolic dysfunctionPredictor of prognosisVentricular cavity sizeProportional hazards modelSignificant predictorsSystolic dysfunctionMedian timeAtrial fibrillationSpecialty centersSarcomeric variantsHazards modelIncident developmentPatientsEnd stageRelative rarityPrognosisOutcomesPredictorsAssociation of Race With Disease Expression and Clinical Outcomes Among Patients With Hypertrophic Cardiomyopathy
Eberly LA, Day SM, Ashley EA, Jacoby DL, Jefferies JL, Colan SD, Rossano JW, Semsarian C, Pereira AC, Olivotto I, Ingles J, Seidman CE, Channaoui N, Cirino AL, Han L, Ho CY, Lakdawala NK. Association of Race With Disease Expression and Clinical Outcomes Among Patients With Hypertrophic Cardiomyopathy. JAMA Cardiology 2020, 5: 83-91. PMID: 31799990, PMCID: PMC6902181, DOI: 10.1001/jamacardio.2019.4638.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedAtrial FibrillationBlack or African AmericanCardiomyopathy, HypertrophicCohort StudiesDeath, Sudden, CardiacDefibrillators, ImplantableFemaleGenetic TestingHealth Services AccessibilityHealthcare DisparitiesHeart FailureHeart SeptumHeart TransplantationHeart-Assist DevicesHumansMaleMiddle AgedMortalityPhenotypeQuality of Health CareStrokeUnited StatesWhite PeopleConceptsNYHA class IIIAssociation of raceVentricular assist device implantationOverall composite outcomeSeptal reduction therapyAssist device implantationHeart failureBlack patientsHypertrophic cardiomyopathyCause mortalityWhite patientsAtrial fibrillationClass IIICardiac transplantationGenetic testingComposite outcomeClinical outcomesDisease expressionDevice implantationReduction therapyNew York Heart Association functional class IIIMultivariable Cox proportional hazards regressionInvasive septal reduction therapySarcomeric Human Cardiomyopathy RegistryImplantable cardioverter-defibrillator therapy