Card Fail Rev. 2026 Mar 2;12:e05. doi: 10.15420/cfr.2025.25. eCollection 2026.
ABSTRACT
End-stage hypertrophic cardiomyopathy (ES-HCM) presents on a spectrum between the classic left ventricular systolic dysfunction and a less-recognised, restrictive phenotype with small-to-normal-sized left ventricular cavity and preserved ejection fraction. Relatively poor prognoses follow those on this morpho-functional spectrum, marked by low cardiac output and high arrhythmic risk. Contemporary guideline-directed therapies, such as ICDs, CRT and cardiac transplantation, have significantly improved outcomes, reshaping the natural history of ES-HCM. Meanwhile, the roles of neurohormonal modulation and left ventricular assist devices remain less well defined. Still, timely recognition and intervention remain essential in patients with ES-HCM, given its considerable morbidity and mortality. As cardiac myosin inhibitors and anti-fibrotic strategies advance, early targeted treatment may modify the natural history of hypertrophic cardiomyopathy, leading to a reduction in the incidence of ES-HCM. Moving forward, broader adoption of phenotype-specific approaches and equitable application of advanced therapies are critical to address the rare but clinically significant burden of ES-HCM.
PMID:41837112 | PMC:PMC12983141 | DOI:10.15420/cfr.2025.25