2024
Efficacy and Safety of aficamten in Symptomatic Non-Obstructive Hypertrophic Cardiomyopathy: Results From the REDWOOD-HCM Trial, Cohort 4
Masri A, Sherrid M, Abraham T, Choudhury L, Garcia-Pavia P, Kramer C, Barriales-Villa R, Owens A, Rader F, Nagueh S, Olivotto I, Saberi S, Tower-Rader A, Wong T, Coats C, Watkins H, Fifer M, Solomon S, Heitner S, Jacoby D, Kupfer S, Malik F, Meng L, Sohn R, Wohltman A, Maron M, Investigators R. Efficacy and Safety of aficamten in Symptomatic Non-Obstructive Hypertrophic Cardiomyopathy: Results From the REDWOOD-HCM Trial, Cohort 4. Journal Of Cardiac Failure 2024 PMID: 38493832, DOI: 10.1016/j.cardfail.2024.02.020.Peer-Reviewed Original ResearchNonobstructive hypertrophic cardiomyopathyHypertrophic cardiomyopathyKansas City Cardiomyopathy Questionnaire Clinical Summary ScoreOpen-label phase 2 trialNew York Heart Association classHistory of aborted sudden cardiac deathNon-obstructive hypertrophic cardiomyopathyHigh-sensitivity cardiac troponin IAborted sudden cardiac deathBlood levels of biomarkersNT-proBNP levelsPhase 2 trialPlacebo-controlled studyHeart failure symptomsCardiac myosin inhibitorClinical summary scoreSudden cardiac deathClinically relevant improvementWeeks of washoutLevels of biomarkersCardiac troponin IHeart muscle cellsAsymptomatic reductionNT-proBNPAssociated with improvementsBaseline Characteristics of Patients in SEQUOIA-HCM: A Phase 3 Trial of Aficamten in Obstructive Hypertrophic Cardiomyopathy
Maron M, Abraham T, Coats C, Olivotto I, Lee M, Arad M, Cardim N, Ma C, Choudhury L, Düngen H, Garcia-Pavia P, Hagege A, Lewis G, Michels M, Oreziak A, Owens A, Tfelt-Hansen J, Veselka J, Watkins H, Heitner S, Jacoby D, Kupfer S, Malik F, Meng L, Wohltman A, Masri A. Baseline Characteristics of Patients in SEQUOIA-HCM: A Phase 3 Trial of Aficamten in Obstructive Hypertrophic Cardiomyopathy. Journal Of Cardiac Failure 2024, 30: s3. DOI: 10.1016/j.cardfail.2023.11.006.Peer-Reviewed Original ResearchLeft ventricular outflow tract obstructionObstructive hypertrophic cardiomyopathySymptomatic obstructive hypertrophic cardiomyopathyBaseline characteristics of patientsCardiac myosin inhibitorPhase 3 trialCharacteristics of patientsBaseline characteristicsHypertrophic cardiomyopathyNew York Heart Association functional classVentricular outflow tract obstructionN-terminal pro-B-type natriuretic peptidePro-B-type natriuretic peptideTreated with beta-blockersHigh-sensitivity cardiac troponin IBaseline New York Heart Association functional classOutflow tract obstructionPlacebo-controlled trialBaseline to weekHeart failure symptomsIndividual daily doseReduced exercise capacityCardiac troponin IMyocardial hypercontractilityOral placebo
2020
Associations Between Female Sex, Sarcomere Variants, and Clinical Outcomes in Hypertrophic Cardiomyopathy
Lakdawala NK, Olivotto I, Day SM, Han L, Ashley EA, Michels M, Ingles J, Semsarian C, Jacoby D, Jefferies JL, Colan SD, Pereira AC, Rossano JW, Wittekind S, Ware JS, Saberi S, Helms AS, Cirino AL, Leinwand LA, Seidman CE, Ho CY. Associations Between Female Sex, Sarcomere Variants, and Clinical Outcomes in Hypertrophic Cardiomyopathy. Circulation Genomic And Precision Medicine 2020, 14: e003062. PMID: 33284039, DOI: 10.1161/circgen.120.003062.Peer-Reviewed Original ResearchConceptsHypertrophic cardiomyopathyClinical outcomesNew York Heart Association IIIImplantable cardioverter-defibrillator utilizationSevere heart failure symptomsHeart failure symptomsReduced ejection fractionRetrospective cohort studyImpact of sexSex-based differencesHCM centersCause mortalityTract obstructionBaseline characteristicsCohort studyEjection fractionHeart failureVentricular arrhythmiasFemale sexHCM patientsMyocardial performanceFailure symptomsInternational registrySarcomere variantsHigh burdenPsychological stress in heart failure: a potentially actionable disease modifier
Harris KM, Jacoby DL, Lampert R, Soucier RJ, Burg MM. Psychological stress in heart failure: a potentially actionable disease modifier. Heart Failure Reviews 2020, 26: 561-575. PMID: 33215323, PMCID: PMC8026548, DOI: 10.1007/s10741-020-10056-8.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsHeart failurePsychological stressHeart failure symptomsHeart failure patientsLife stressorsDisease-related consequencesQuality of lifePatterns of exposureAdverse eventsFailure patientsDisease courseEjection fractionPatients' qualityModifiable factorsPatient subgroupsSymptom exacerbationDaily life stressorsFailure symptomsPathophysiological pathwaysEffective diseaseFunctional limitationsDiminished qualityPatientsActionable pathwaysStress exposureTemporal Trend of Age at Diagnosis in Hypertrophic Cardiomyopathy
Canepa M, Fumagalli C, Tini G, Vincent-Tompkins J, Day SM, Ashley EA, Mazzarotto F, Ware JS, Michels M, Jacoby D, Ho CY, Olivotto I, Investigators T. Temporal Trend of Age at Diagnosis in Hypertrophic Cardiomyopathy. Circulation Heart Failure 2020, 13: e007230-e007230. PMID: 32894986, PMCID: PMC7497482, DOI: 10.1161/circheartfailure.120.007230.Peer-Reviewed Original ResearchConceptsHypertrophic cardiomyopathyHCM diagnosisSarcomeric Human Cardiomyopathy RegistryGenetic testingHeart failure symptomsObstructive hypertrophic cardiomyopathyNon-US sitesEra of diagnosisLikely pathogenic variantsClinical characteristicsOlder patientsFamilial hypertrophic cardiomyopathyHCM populationVentricular hypertrophyFemale ratioFailure symptomsSporadic diseasePathogenic variantsAdvanced diagnostic toolsDiagnosisTemporal trendsStable maleMild phenotypeAgePatientsAssociation of Obesity With Adverse Long-term Outcomes in Hypertrophic Cardiomyopathy
Fumagalli C, Maurizi N, Day SM, Ashley EA, Michels M, Colan SD, Jacoby D, Marchionni N, Vincent-Tompkins J, Ho CY, Olivotto I. Association of Obesity With Adverse Long-term Outcomes in Hypertrophic Cardiomyopathy. JAMA Cardiology 2020, 5: 65-72. PMID: 31693057, PMCID: PMC6865784, DOI: 10.1001/jamacardio.2019.4268.Peer-Reviewed Original ResearchMeSH KeywordsAdultArrhythmias, CardiacAtrial FibrillationBody Mass IndexCardiomyopathy, HypertrophicCohort StudiesDeath, Sudden, CardiacDefibrillators, ImplantableDisease ProgressionElectric CountershockFemaleHeart ArrestHeart FailureHeart TransplantationHeart-Assist DevicesHumansMaleMiddle AgedMortalityObesityRetrospective StudiesStrokeStroke VolumeVentricular Outflow ObstructionConceptsOverall composite outcomeLong-term outcomesHypertrophic cardiomyopathyComposite outcomeObstructive physiologyAdverse outcomesSarcomeric Human Cardiomyopathy RegistryAdverse long-term outcomesLong-term adverse outcomesLife-threatening ventricular arrhythmiasHeart failure outcomesHeart failure symptomsAssociation of obesityDisease-related complicationsNormal weight groupBody mass indexOverall disease progressionBody weight increaseHCM centersAtrium diameterBaseline BMIObesity groupCardiovascular mortalityCohort studyMass index