Sivasankaran Sivasubramonian, Sharland Gurleen K, Simpson John M
Department of Congenital Heart Disease, Guy's Hospital, Fetal Cardiology Unit, London, United Kingdom.
Cardiol Young. 2005 Aug;15(4):409-16. doi: 10.1017/S1047951105000855.
To describe the echocardiographic features, underlying causes, and outcome of fetuses with dilated cardiomyopathy.
A retrospective observational study between 1983 and 2003 at a tertiary centre for fetal cardiology.
Affected fetuses were identified using a computerised database. We included fetuses with dilation and reduced systolic function of either the right ventricle, left ventricle, or both. We excluded fetuses with abnormal cardiac connections, arrhythmias, or stenosis of the aortic or pulmonary valves. In all, we identified 50 fetuses, born to 46 mothers. Of the fetuses, 24 had biventricular cardiomyopathy, 17 had isolated right ventricular cardiomyopathy, and 9 had isolated left ventricular cardiomyopathy. Two-thirds of the fetuses (32) were hydropic at some point during gestation.
A cause of cardiomyopathy was identified in 37 cases (74 per cent). This was genetic or metabolic in 11 fetuses; infective in 11; fetal anaemia, without proven parvovirus infection, in 5; of cardiac origin in 5; and an association with renal disease in 5. In 10 cases (20 per cent), the pregnancy was terminated. Based on an intention to treat, the survival to delivery was 25 of 40 (62.5 per cent, 95 per cent confidence intervals from 46 to 77 per cent), at 28 days was 17 of 40 (42.5 per cent, 95 per cent confidence intervals from 27 to 59 per cent), and at 1 year was 15 of 40 (37.5 per cent, 95 per cent confidence intervals from 23 to 54 per cent). The overall survival of non-hydropic fetuses was 9 of 18 (50 per cent), compared to 6 of 32 (18 per cent) hydropic fetuses.
Genetic, metabolic, infective, and cardiac diseases may present with dilated cardiomyopathy during fetal life. There is a high rate of spontaneous intra-uterine and early neonatal death. The prognosis is particularly poor for hydropic fetuses.
描述扩张型心肌病胎儿的超声心动图特征、潜在病因及预后。
1983年至2003年在一家三级胎儿心脏病中心进行的一项回顾性观察研究。
使用计算机数据库识别受影响的胎儿。我们纳入了右心室、左心室或两者均有扩张且收缩功能降低的胎儿。我们排除了心脏连接异常、心律失常或主动脉或肺动脉瓣狭窄的胎儿。总共，我们识别出50例胎儿，其母亲为46位。其中，24例患有双心室心肌病，17例患有孤立性右心室心肌病，9例患有孤立性左心室心肌病。三分之二的胎儿（32例）在妊娠期间的某个时间出现水肿。
37例（74%）确定了心肌病的病因。其中11例胎儿病因是遗传或代谢性的；11例是感染性的；5例是胎儿贫血（未证实细小病毒感染）；5例是心脏源性的；5例与肾脏疾病有关。10例（20%）妊娠终止。基于意向性治疗，至分娩存活的有40例中的25例（62.5%，95%置信区间为46%至77%），28天时存活的有40例中的17例（42.5%，95%置信区间为27%至59%），1岁时存活的有40例中的15例（37.5%，95%置信区间为23%至54%）。非水肿胎儿的总体存活率为18例中的9例（50%），而水肿胎儿为32例中的6例（18%）。
遗传、代谢、感染和心脏疾病在胎儿期可能表现为扩张型心肌病。宫内和早期新生儿自发死亡率很高。水肿胎儿的预后尤其差。
