The Heart Muscle Disease Clinic, London Chest Hospital, Barts and The London NHS Trust, London, UK.
Cardiovasc Ther. 2012 Jun;30(3):e107-14. doi: 10.1111/j.1755-5922.2010.00229.x. Epub 2011 Jul 31.
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder. This autosomal dominant condition is defined by left ventricular hypertrophy and associated with functional limitation and premature death. In fact, many individuals are asymptomatic and the annual mortality in most modern series is 1% or less. However, severe symptoms may develop at any age, and the risk of premature death from arrhythmia, stroke, and progressive systolic impairment may complicate asymptomatic disease. The clinical management of patients with HCM therefore encompasses (1) genetic counseling including discussion of indications for genetic testing and cascade family screening, (2) assessment of prognostic risk from ventricular arrhythmia, stroke, and heart failure, and (3) symptom management. This article describes the interventional treatments in the management of severe symptoms associated with left ventricular outflow tract obstruction (LVOTO).
肥厚型心肌病(HCM)是最常见的遗传性心脏病。这种常染色体显性疾病的特征是左心室肥厚,并伴有功能受限和过早死亡。事实上,许多人无症状,在大多数现代系列中,每年的死亡率为 1%或更低。然而,严重的症状可能在任何年龄发展,心律失常、中风和进行性收缩功能障碍的过早死亡风险可能使无症状疾病复杂化。因此,HCM 患者的临床管理包括(1)遗传咨询,包括讨论基因检测和级联家族筛查的适应证,(2)评估室性心律失常、中风和心力衰竭的预后风险,以及(3)症状管理。本文描述了在管理与左心室流出道梗阻(LVOTO)相关的严重症状时的介入治疗。