Baseline and Longitudinal Imaging of Hypertrophic Cardiomyopathy in the Era of Emerging Therapeutics

Abstract

Purpose of review: In this review, we will overview the baseline and longitudinal imaging modalities utilized in the care of patients with hypertrophic cardiomyopathy (HCM) with a focus on echocardiography and cardiac magnetic resonance (CMR) imaging, especially in the new era of cardiac myosin inhibitors (CMIs).

Recent findings: Traditional therapies for hypertrophic cardiomyopathy (HCM) have been well established for decades. Attempts to investigate new drug therapy in HCM resulted in neutral clinical trials, until the discovery of cardiac myosin inhibitors (CMIs). The introduction of this new class of small oral molecules which target the hypercontractility resulting from excessive actin-myosin cross-bridging at the sarcomere level is the first therapeutic option which directly addresses the underlying pathophysiology of HCM. While imaging has always played a central role in HCM diagnosis and management, CMIs introduced a new paradigm in the use of imaging to evaluate and monitor patients with HCM. Echocardiography and cardiac magnetic resonance imaging (CMR) are the central modalities in the care of patients with HCM, but their roles and our understanding of their strengths and limitations are evolving as newer therapeutics are being investigated in clinical trials and in daily practice. In this review, we will focus the recent CMI trials and discuss the role of baseline and longitudinal imaging with echocardiography and CMR in the care of patients with HCM in the era of CMIs.

Keywords: Aficamten; Cardiac magnetic resonance (CMR); Cardiac myosin inhibitors (CMIs); Echocardiogram; Hypertrophic cardiomyopathy (HCM); Mavacamten.