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1960~1992年北京儿童医院病理诊断左心发育不良综合征14例,其中9例作临床分析。男7例,女2例,均为足月儿,发病日龄在11天以内(<3天者6例),以哭后或吃奶后突然气急,面色发青而入院。入院时有重度循环衰竭者6例,有心脏杂音8例,肺有罗音及肝大者8例。X线胸片示心脏明显扩大伴肺血增多,心电图示右房室肥大及T波改变。入院3天内死亡8例,10天1例,均死于心源性休克。心脏B超诊断2例,9例病理均有左心室发育不良伴有主动脉瓣和(或)二尖瓣闭锁或狭窄。本文讨论了诊断及心脏移植可获成功,为优生目的,若宫内诊断者建议终止妊娠.
Beijing Children’s Hospital from 1960 to 1992 pathological diagnosis of hypoplastic left heart syndrome in 14 cases, of which 9 cases for clinical analysis. There were 7 males and 2 females, all of whom were full-term infants. The age of onset was within 11 days (6 in 3 days). There were 6 cases of severe circulatory failure on admission, 8 cases of cardiac murmur, 8 cases of pulmonary rales and liver enlargement. X-ray showed significantly enlarged heart with pulmonary blood increased, ECG showed right atrial hypertrophy and T wave changes. 8 cases died within 3 days of admission, 10 cases in 1 day, all died of cardiogenic shock. Cardiac B-ultrasound diagnosis in 2 cases, 9 cases of pathology with left ventricular dysplasia associated with aortic valve and (or) mitral valve atresia or stenosis. This article discusses the diagnostic and cardiac transplantation can be successful for the purpose of eugenics, if the intrauterine diagnosis suggested termination of pregnancy.