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A deep-intronic MYBPC3 variant magnifies natural mRNA missplicing events, causing hypertrophic cardiomyopathy and explaining previously genotype-negative cases through an ancient founder effect and novel splice regulation.
A novel left atrial (LA) staging system, integrating hemodynamic load and contractile function, predicts new-onset atrial fibrillation risk in hypertrophic cardiomyopathy and may guide tailored AF surveillance.
An international multicenter study shows ICDs effectively prevent sudden death in high-risk hypertrophic cardiomyopathy patients, demonstrating low mortality rates and significant lifesaving interventions over long-term follow-up.
Rigosertib demonstrates significant reversal of hypertrophic cardiomyopathy and other syndromic features in Noonan syndrome models, highlighting its potential as a targeted therapy for RASopathy-associated cardiac and developmental abnormal
This study explores the potential of an AI-powered ECG tool to monitor left ventricular systolic dysfunction in hypertrophic cardiomyopathy patients treated with mavacamten, offering a less burdensome alternative to frequent echocardiograms
This study reveals that 21% of asymptomatic patients with mild HCM develop major adverse cardiovascular events over 7 years, highlighting key predictors such as age, symptom progression, and cardiac remodeling markers that inform management
The MAPLE-HCM trial demonstrates aficamten monotherapy significantly outperforms metoprolol in improving exercise capacity, hemodynamics, and patient-reported outcomes in obstructive hypertrophic cardiomyopathy (oHCM), supporting aficamten
This study demonstrates that septal myectomy combined with cutting of secondary mitral valve chordae delivers excellent long-term survival and symptom relief in obstructive hypertrophic cardiomyopathy patients unresponsive to medical therap
A multiregistry analysis reveals that massive LVH in pediatric HCM is linked to early diagnosis, sarcomeric mutations, and heightened risks of arrhythmias, heart failure, and mortality. Nearly 25% of patients show significant LVH regression
A comparative pressure-volume analysis reveals that mavacamten and alcohol septal ablation produce equivalent hemodynamic improvements in obstructive HCM, primarily through reversal of the chronic Anrep response that drives elevated myocard
The REVEAL-HCM study demonstrates that the prognostic impact of mitral regurgitation varies significantly across HCM subtypes, with notable risk in nonobstructive and apical forms, but not in obstructive or end-stage HCM.
This article explores a groundbreaking study identifying CRLF1, a protein secreted by cardiac fibroblasts, as a key driver of Hypertrophic Cardiomyopathy (HCM). This paracrine signaling mechanism offers a potential universal therapeutic tar
This review analyzes the SHaRe registry data, revealing that sarcomeric HCM represents a more aggressive clinical phenotype with earlier onset and higher HCM-related mortality compared to nonsarcomeric cases, which are more heavily influenc
A pivotal triple-crossover RCT reveals that while bisoprolol reduces exercise capacity and worsens diastolic markers in nonobstructive HCM, verapamil maintains exercise performance and improves myocardial strain.
The REVEAL-HCM study demonstrates that while mitral regurgitation is a frequent comorbidity in hypertrophic cardiomyopathy, its prognostic impact is highly subtype-specific, significantly affecting outcomes in non-obstructive subtypes but s
The 128-week follow-up of the VALOR-HCM trial demonstrates that mavacamten provides sustained relief from LVOT obstruction, promotes favorable cardiac remodeling, and significantly reduces the need for invasive septal reduction therapy in p
This exploratory analysis of the ODYSSEY-HCM trial demonstrates that mavacamten induces modest regression of left ventricular hypertrophy and improves diastolic and left atrial function in nonobstructive HCM, despite a 21.5% incidence of re
The MAPLE-HCM trial demonstrates that aficamten monotherapy provides superior, rapid, and comprehensive clinical benefits compared to the traditional first-line treatment, metoprolol, across multiple disease domains in patients with symptom
A substudy of the SEQUOIA-HCM trial identifies VO2T12.5% as a novel, clinically relevant CPET metric that correlates with cardiac hemodynamics, predicts heart failure outcomes, and improves significantly with aficamten treatment in patients
While the ODYSSEY-HCM trial missed its primary functional endpoints, a new exploratory analysis reveals that mavacamten significantly reduces NT-proBNP and hs-cTnI levels in patients with nonobstructive hypertrophic cardiomyopathy, suggesti