Abstract：
Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiac diseases, defined as a left ventricular wall thickness of ≥15 mm, in the absence of other causes of abnormal ventricular loading. A major hallmark of this disease is the presence of left ventricular outflow tract obstruction, which develops in up to three quarters of patients, referred to as obstructive hypertrophic cardiomyopathy. Current treatment is offered to symptomatic patients, based on the presence of documented left ventricular obstruction, aimed at reducing symptoms and disease progression. This is achieved through pharmacological empirical therapy, surgery, alcohol ablation and/or pacing. Mavacamten is a first-in-class allosteric inhibitor of cardiac myosin that promises to provide clinicians with targeted therapy for these patients. The aim of this review is to provide a general overview of the modern approach to the diagnosis and management of HCM, as well as to integrate all the current knowledge on mavacamten, in anticipation of a future change in the treatment algorithm of patients with HCM.
摘要：
肥厚型心肌病(HCM)是最常见的遗传性心脏病之一。定义为左心室壁厚≥15mm，在没有其他原因的异常心室负荷。这种疾病的主要标志是存在左心室流出道阻塞，在多达四分之三的患者中发展，被称为阻塞性肥厚型心肌病。目前的治疗是针对有症状的患者，根据左心室梗阻的记录，旨在减少症状和疾病进展。这是通过药理学经验疗法实现的，手术,酒精消融和/或起搏。Mavacamten是一流的心脏肌球蛋白变构抑制剂，有望为临床医生提供针对这些患者的靶向治疗。这篇综述的目的是对HCM的诊断和管理的现代方法进行概述，以及整合mavacampen上的所有现有知识，预计未来HCM患者的治疗算法会发生变化。
