2024
Long‐Term Safety and Efficacy of Mavacamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Interim Results of the PIONEER‐OLE Study
Masri A, Lester S, Stendahl J, Hegde S, Sehnert A, Balaratnam G, Shah A, Fox S, Wang A. Long‐Term Safety and Efficacy of Mavacamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Interim Results of the PIONEER‐OLE Study. Journal Of The American Heart Association 2024, 13: e030607. PMID: 38591260, PMCID: PMC11262496, DOI: 10.1161/jaha.123.030607.Peer-Reviewed Original ResearchMeSH KeywordsBenzylaminesCardiomyopathy, HypertrophicHumansPilot ProjectsStroke VolumeUracilVentricular Function, LeftConceptsObstructive hypertrophic cardiomyopathyKansas City Cardiomyopathy Questionnaire–Overall Summary scoreSymptomatic obstructive hypertrophic cardiomyopathyOutflow tract gradientHypertrophic cardiomyopathyNew York Heart Association class improvementLeft ventricular outflow tract gradientTreatment-emergent adverse eventsSerum NT-proBNP levelsLeft ventricular ejection fractionEffect of mavacamtenIndividual dose titrationNT-proBNP levelsVentricular ejection fractionSerum NT-proBNPSummary scoreLong-term safetyLonger-term treatmentDose titrationEjection fractionNT-proBNPB-blockersReduced doseAdverse eventsInterim results
2023
Left Ventricular Systolic Dysfunction in Patients Diagnosed With Hypertrophic Cardiomyopathy During Childhood: Insights From the SHaRe Registry
Alaiwi S, Roston T, Marstrand P, Claggett B, Parikh V, Helms A, Ingles J, Lampert R, Lakdawala N, Michels M, Owens A, Rossano J, Saberi S, Abrams D, Ashley E, Semsarian C, Stendahl J, Ware J, Miller E, Ryan T, Russell M, Day S, Olivotto I, Vissing C, Ho C. Left Ventricular Systolic Dysfunction in Patients Diagnosed With Hypertrophic Cardiomyopathy During Childhood: Insights From the SHaRe Registry. Circulation 2023, 148: 394-404. PMID: 37226762, PMCID: PMC10373850, DOI: 10.1161/circulationaha.122.062517.Peer-Reviewed Original ResearchConceptsLeft ventricular systolic dysfunctionHypertrophic cardiomyopathyMedian ageHCM diagnosisVentricular assist device implantationCox proportional hazards modelComposite of deathVentricular systolic dysfunctionAssist device implantationVentricular ejection fractionProportional hazards modelCardiac transplantationSystolic dysfunctionEjection fractionHCM cohortPediatric cohortDevice implantationPoor outcomeSubsequent prognosisEchocardiographic reportsHazards modelPatientsAdult cohortPrognosisCohort