儿童扩张型心肌病的治疗进展

摘要

儿童扩张型心肌病(pediatric dilated cardiomyopathy,PDCM)不仅与感染、代谢等因素相关,也是一种先天性心肌病.PDCM的超声心动图(ultrasonic cardiogram)显示左心室或双心室严重扩大,伴左心室射血收缩能力减弱.临床上起病隐匿,常表现为顽固性心力衰竭、心律失常及猝死(心功能往往已达III～IV级).该病预后差,病死率及病残率较高,是儿童心脏移植的主要病因.在过去的三十年里,对于左心室功能障碍的治疗一直是成人心肌病研究的重心,儿童扩张型心肌病的治疗大多从成人扩张性心肌病的治疗中延伸过来.该文将借助成人扩张性心肌病治疗指南,结合近年国内外研究来阐述最新PDCM治疗的研究与经验,探讨近年来PDCM治疗的进展情况.%Pediatric dilated cardiomyopathy( PDCM) is not only associated with infection,metabolism and other factors, but also a congenital cardiomyopathy. PDCM echocardiography ( ultrasonic cardiogram ) showed a severe enlargement of the left ventricle or double ventricle,with decreased left ventricle ejection con-traction. Clinical onset of disease concealment,often manifested as refractory heart failure,arrhythmia and sudden death( heart function often reached III～IV level) . The prognosis of the disease is poor,and mortality and mor-bidity are high,which may be the main cause of heart transplantation in children. Over the past three decades, treatment of left ventricular dysfunction has been the focus of research on adult cardiomyopathy,and treatment of PDCM has mostly extended from the treatment of dilated cardiomyopathy in adults. This article will provide the guidance of adult patients with cardiac dilation therapy,and is combined with recent research at home and abroad to illustrate the latest research and experience of PDCM treatment,and to explore the progress of PDCM treat-ment in recent years.