Crochelet A S, Crochelet L, Piérard L A
Université de Liège.
Rev Med Liege. 2009 Nov;64(11):576-80.
Hypertrophic cardiomyopathy is of genetic origin, characterized by asymmetric left ventricular hypertrophy and variable clinical presentation. The physiopathology includes diastolic dysfunction and, in one third of the patients, dynamic left ventricular outflow tract obstruction. Patients are at increased risk of sudden death. Risk stratification in the individual patient is an essential component of management. This article describes the clinical presentation of a patient and his mother and summarizes essential features of the disease and the current recommendations for the prevention of sudden cardiac death.
肥厚型心肌病起源于遗传,其特征为不对称性左心室肥厚以及临床表现多样。病理生理学包括舒张功能障碍,并且在三分之一的患者中存在动态左心室流出道梗阻。患者猝死风险增加。对个体患者进行风险分层是管理的重要组成部分。本文描述了一名患者及其母亲的临床表现,并总结了该疾病的基本特征以及目前预防心源性猝死的建议。