Komajda Michel, Charron Philippe
Département de cardiologie Centre hospitalier universitaire La Pitié-La Salpêtrière 75651 Paris.
Rev Prat. 2002 Oct 1;52(15):1664-70.
These heart muscle diseases, associated with systolic or diastolic dysfunction, are associated with a high prevalence and morbidity, because of the frequent evolution towards congestive heart failure or sudden death. Dilated cardiomyopathy is the first cause of heart transplantation and Hypertrophic cardiomyopathy is the first cause of sudden death in athletes. Familial and monogenic forms of these diseases are frequent and this should lead to a systematic cardiological familial inquest in first degree relatives of a patient. Although they were previously called as "idiopathic", the pathophysiology of the disease is actually under investigation thanks to molecular genetics. This completely new knowledge should lead to the development of new therapeutic strategies.
这些与收缩或舒张功能障碍相关的心肌疾病,由于常发展为充血性心力衰竭或猝死,因而具有很高的患病率和发病率。扩张型心肌病是心脏移植的首要病因,肥厚型心肌病是运动员猝死的首要病因。这些疾病的家族性和单基因形式很常见,这就要求对患者的一级亲属进行系统的心脏病学家族调查。尽管这些疾病以前被称为“特发性”,但由于分子遗传学的发展,目前该病的病理生理学实际上正在研究中。这一全新的知识应能推动新治疗策略的开发。