Favorable cardiac remodeling in response to treatment in obstructive hypertrophic cardiomyopathy: a current appraisal

Abstract

Hypertrophic cardiomyopathy is a heterogenous genetic condition caused by myocyte disarray with varying degrees of interstitial fibrosis. Traditional management strategies focused on symptom mitigation and sudden cardiac death prevention among high-risk patients. The only disease-modifying treatments have historically been septal reduction therapy or heart transplantation. However, cardiac myosin inhibitors have emerged as promising novel pharmacotherapies. Emerging evidence indicates that cardiac myosin inhibitors as well as surgical myectomy result in not only symptomatic benefit and reduction in left ventricular outflow tract obstruction, but also positive cardiac remodeling including reduction in left ventricular mass and maximum wall thickness, improved left ventricular diastolic parameters, reduced left atrial volumes, and improved left atrial strain. The long-term durability and clinical significance of these findings require further study. In this article, we will review the existing evidence of favorable reverse remodeling conferred by cardiac myosin inhibitors and surgical myectomy.

Keywords: Hypertrophic cardiomyopathy; cardiac myosin inhibitors; cardiac remodeling; global longitudinal strain; late gadolinium enhancement; mavacamten; septal myectomy.