Zarco P, Alfonso F
Catedrático de Cardiología, Hospital Universitario San Carlos, Madrid.
Rev Esp Cardiol. 1995 Sep;48(9):616-27.
This report provides an overview of current concepts on the cardiomyopathies. They constitute a heterogeneous group of diseases with complex interrelations between clinical manifestations, pathophysiology and therapy. Special emphasis was made to review new advances on the diagnosis and current management of patients with hypertrophic cardiomyopathy. The link between the classical knowledge and recent--still evolving--ideas is addressed to provide the--state of the art--concerning the implications on the clinical decision making process. Controversial issues such as diagnostic criteria, prognostic features and implications of electrocardiographic and echocardiographic data are revisited in brief. Data suggesting the value of dual chamber pacing as a means to relieve outflow tract obstruction and to improve symptoms in patients with hypertrophic cardiomyopathy are discussed. The molecular basis of this entity together with the characterization of the responsible genetic mutations are reviewed in detail.
本报告概述了目前关于心肌病的概念。它们构成了一组异质性疾病，临床表现、病理生理学和治疗之间存在复杂的相互关系。特别强调回顾肥厚型心肌病患者诊断和当前管理方面的新进展。阐述了经典知识与最新的（仍在不断发展的）观点之间的联系，以提供有关临床决策过程影响的最新技术水平。简要回顾了诸如诊断标准、预后特征以及心电图和超声心动图数据的意义等有争议的问题。讨论了提示双腔起搏作为缓解肥厚型心肌病患者流出道梗阻和改善症状手段的价值的数据。详细回顾了该疾病实体的分子基础以及相关基因突变的特征。
