左心发育不良综合征9例临床分析

CLINICAL ANALYSIS OF 9 CASES OF NEONATAL HYPOPLASTIC LEFT HEART SYNDROME

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．

During 1960-1992, there were 14 cases of Hypoplastic left heart syndrome diagnosed on autopsy,the clinical features of 9 cases from the 14 cases were analyzed. Male 7, female 2,full term newborns considered normal at birth, onset of distress presented within 11 days after birth (6 cases<3 days). On admission, all cases had dyspnea and/or cyanosis, 6 cases had circulating collapese, 8 cases had soft systolic murmurs, and 8 cases had rales and hepatomegaly. Chest X ray showed severe cardiomegaly with pulmonary vascular markings increased. EKG showed right atrial and ventricle enlargement, and T waves changed. 8 cases died of circulating collapse. 2 cases diagnosied by 2D and Doppler Echocardio-graphy. The anatomy of 9 cases had hypoplastic left heart with aortic atresia and/or mitral atresia or stenosis. The diagnosis and heart transplantation were discussed, and for the eugenic purpose, that' s better to stop the pregnancy when hypoplastic left heart syndrome is diagnosed in fetus.