Hypertrophic Cardiomyopathy: From Medical Treatment to Advanced Heart Failure Therapies

Curr Cardiol Rep. 2024 Sep;26(9):985-994. doi: 10.1007/s11886-024-02095-6. Epub 2024 Jul 11.

Abstract

Purpose of review: There has been much debate surrounding novel medical therapies and heart transplantation listing challenges in patients with hypertrophic cardiomyopathy (HCM).

Recent findings: Recent clinical trials led to FDA approval of mavacamten (a cardiac myosin inhibitor), offering symptom relief and potentially delaying/avoiding invasive septal reduction therapies for some patients with HCM and left ventricular outflow obstruction (LVOTO). For those with refractory symptoms and end-stage heart failure, heart transplantation remains the gold standard. However, the concern for the organ allocation system failing to prioritize those individuals persists. HCM is a heterogeneous genetic condition with variable penetration and clinical presentation. Even though a large portion of patients remain asymptomatic, an important minority develops debilitating symptoms refractory to medical therapy. Post-HT short- and long-term outcomes are favorable. However, HT waitlist mortality remains high. For highly selected patients with HCM, a left ventricular assist device is a viable option.

Keywords: Cardiac myosin inhibitors; Heart failure; Heart transplant; Hypertrophic cardiomyopathy; Left ventricular assist device.

Publication types

  • Review

MeSH terms

  • Benzylamines
  • Cardiomyopathy, Hypertrophic* / drug therapy
  • Cardiomyopathy, Hypertrophic* / therapy
  • Heart Failure* / drug therapy
  • Heart Failure* / therapy
  • Heart Transplantation*
  • Heart-Assist Devices*
  • Humans
  • Uracil / analogs & derivatives
  • Waiting Lists

Substances

  • MYK-461
  • Benzylamines
  • Uracil