2024
Impact of Aficamten on Echocardiographic Cardiac Structure and Function in Symptomatic Obstructive Hypertrophic Cardiomyopathy
Hegde S, Claggett B, Wang X, Jering K, Prasad N, Roshanali F, Masri A, Nassif M, Barriales-Villa R, Abraham T, Cardim N, Coats C, Kramer C, Maron M, Michels M, Olivotto I, Saberi S, Jacoby D, Heitner S, Kupfer S, Meng L, Wohltman A, Malik F, Solomon S, Investigators S. Impact of Aficamten on Echocardiographic Cardiac Structure and Function in Symptomatic Obstructive Hypertrophic Cardiomyopathy. Journal Of The American College Of Cardiology 2024, 84: 1789-1802. PMID: 39217556, DOI: 10.1016/j.jacc.2024.08.002.Peer-Reviewed Original ResearchLeft ventricular ejection fractionObstructive hypertrophic cardiomyopathySymptomatic obstructive hypertrophic cardiomyopathyLVOT gradientKCCQ-CSSNT-proBNPAssociated with improvementsHypertrophic cardiomyopathyKansas City Cardiomyopathy Questionnaire Clinical Summary ScoreCardiac structureMeasures of LV diastolic functionLV global circumferential strainN-terminal pro-B-type natriuretic peptideLV global longitudinal strainLV end-systolic volumePro-B-type natriuretic peptideEchocardiographic measures of cardiac structureMeasures of cardiac structureLateral e' velocityLV diastolic functionAtrial volume indexGlobal longitudinal strainLV systolic functionPlacebo-controlled studyGlobal circumferential strain
2020
Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy
Smith ED, Lakdawala NK, Papoutsidakis N, Aubert G, Mazzanti A, McCanta AC, Agarwal PP, Arscott P, Dellefave-Castillo LM, Vorovich EE, Nutakki K, Wilsbacher LD, Priori SG, Jacoby DL, McNally EM, Helms AS. Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy. Circulation 2020, 141: 1872-1884. PMID: 32372669, PMCID: PMC7286080, DOI: 10.1161/circulationaha.119.044934.Peer-Reviewed Original ResearchConceptsLV late gadolinium enhancementLate gadolinium enhancementSevere ventricular arrhythmiasAcute myocardial injuryMyocardial injuryRight ventricular cardiomyopathyVentricular arrhythmiasGadolinium enhancementVentricular cardiomyopathyEjection fractionSevere arrhythmiasFrequent premature ventricular contractionsPathogenic plakophilin-2 mutationNormal LV systolic functionLeft ventricle ejection fractionRight ventricular ejection fractionPositron emission tomography scanArrhythmogenic right ventricular cardiomyopathyNormal ventricular functionLV systolic functionVentricle ejection fractionVentricular ejection fractionEmission tomography scanSmall case seriesPlakophilin-2 mutations