Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli," Naples, Italy (E.M., M.R., F.V., G.D., G.D.M., I.A., M.L., A.P., L.F., G.P., G.L.).
Institute of Cardiovascular Sciences, University College London, United Kingdom (E.M., A.B., P.M.E., G.L.).
Circ Heart Fail. 2023 Aug;16(8):e010687. doi: 10.1161/CIRCHEARTFAILURE.123.010687. Epub 2023 Jul 21.
Hypertrophic cardiomyopathy is a myocardial disease defined by an increased left ventricular wall thickness not solely explained by abnormal loading conditions. It is often genetically determined, with sarcomeric gene mutations accounting for around 50% of cases. Several conditions, including syndromic, metabolic, infiltrative, and neuromuscular diseases, may present with left ventricular hypertrophy, mimicking the hypertrophic cardiomyopathy phenotype but showing a different pathophysiology, clinical course, and outcome. Despite being rare, they are collectively responsible for a large proportion of patients presenting with hypertrophic heart disease, and their timely diagnosis can significantly impact patients' management. The understanding of disease pathophysiology has advanced over the last few years, and several therapeutic targets have been identified, leading to a new era of tailored treatments applying to different etiologies associated with left ventricular hypertrophy. This review aims to provide an overview of the existing and emerging therapies for the principal causes of hypertrophic heart disease, discussing the potential impact on patients' management and clinical outcome.
肥厚型心肌病是一种心肌疾病,其特征为左心室壁厚度增加,不能仅用异常负荷条件来解释。它通常由基因突变引起,约 50%的病例与肌节基因突变有关。一些疾病,包括综合征、代谢、浸润性和神经肌肉疾病,可能会出现左心室肥厚,模仿肥厚型心肌病表型,但表现出不同的病理生理学、临床过程和结局。尽管它们很少见,但它们共同导致了很大一部分出现肥厚性心脏病的患者,及时诊断可以显著影响患者的管理。近年来,对疾病病理生理学的认识已经取得了进展,已经确定了几个治疗靶点,为不同病因引起的左心室肥厚的针对性治疗带来了一个新时代。本综述旨在概述肥厚性心脏病的主要病因的现有和新兴治疗方法,讨论其对患者管理和临床结局的潜在影响。