Advanced Heart Failure Therapies for Hypertrophic Cardiomyopathy: State-of-the-Art Review and an Updated Analysis From UNOS.

Hypertrophic cardiomyopathy (HCM) is most commonly associated with obstructive symptoms and sudden cardiac death; however, predominantly nonobstructive advanced heart failure in HCM, marked by medically refractory disease with severe functional impairment, occurs in 5% to 7% of patients with HCM. The diagnosis relies on the integration of imaging (echocardiography/cardiac magnetic resonance), hemodynamic data, and cardiopulmonary exercise testing to identify the patients who will benefit from advanced heart failure therapies. Most advanced heart failure therapies focus on systolic dysfunction and are not always applicable to this patient population. Left ventricular assist devices may be an option in a highly selected population with left ventricular dilation. Heart transplantation is often the best option for patients with advanced heart failure in HCM with excellent post-transplantation survival.
Competing Interests: Funding Support and Author Disclosures Dr Shimada was supported by the National Institutes of Health (R01 HL157216). Dr Maurer was supported by funding from the National Institutes of Health (HL139671-01, AG R21AG058348, and AG K24AG036778). Dr Clerkin was supported by the National Institutes of Health (K23 HL148528). Dr Shimada has received research support from the American Heart Association National Clinical and Population Research Awards, an American Heart Association Career Development Award, the Korea Institute of Oriental Medicine, a Columbia University Irving Medical Center Irving Institute for Clinical and Translational Research Precision Medicine Pilot Award, and Bristol Myers Squibb; and consulting income from Bristol Myers Squibb; and his institution has also received funding for clinical trials for Bristol Myers Squibb. Dr Maurer has received consulting income from Akcea, Alnylam, Eidos Therapeutics, Pfizer, Prothena, Novo Nordisk, and Intellia; and his institution has also received funding for clinical trials for Alnylam, Eidos Therapeutics, Pfizer, and Prothena. Dr Sayer has received consulting fees from Abbott. Dr Uriel has received grants from Abbott, Abiomed, and Fire 1; and served on the medical advisory board for Livemetric, Leviticus, and Revamp outside the submitted work. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
(Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)