Wu Y L, Chen M R, Chiu I S, Chen B F
Department of Surgery, National Taiwan University Hospital, Taipei.
Thorac Cardiovasc Surg. 1994 Oct;42(5):315-7. doi: 10.1055/s-2007-1016513.
A full-term baby girl was diagnosed at birth using echocardiography to have a single ventricle with common atrioventricular valve and subaortic ridge. On the 22nd day after birth she was admitted to hospital for intensive care after sudden onset of cyanosis. Three days later she died after repeated attacks of bradycardia. At necropsy the heart was examined by sequential segmental approach. Situs solitus with double-inlet right ventricle and ventriculoarterial discordance was revealed. The left atrial floor was separated from the ventricle by a small hole leading to a translucent blind pouch, which bulged toward the subaortic outflow tract with its hypoplastic tensor apparatus connected to the subaortic infundibulum. The aortic valve was normal. The pulmonary trunk with a competent bicuspid valve arose from the left-sided rudimentary left ventricle. The pulmonary blood flow was limited by a slit-like interventricular communication. Obstruction of the pulmonary venous return due to the natural closing of the patent foramen ovale and the mitral atresia led to her premature death.
一名足月女婴出生时通过超声心动图诊断为单心室，伴有共同房室瓣和主动脉下嵴。出生后第22天，她因突然出现青紫而入院接受重症监护。三天后，她在反复出现心动过缓后死亡。尸检时采用顺序分段法检查心脏。结果显示为正位心，右心室双入口，心室动脉不一致。左心房底部通过一个小孔与心室分开，该小孔通向一个半透明的盲袋，盲袋向主动脉下流出道凸出，其发育不全的张肌装置与主动脉下漏斗相连。主动脉瓣正常。肺动脉干和一个功能正常的二尖瓣起源于左侧发育不全的左心室。肺血流受一个狭缝状室间隔交通的限制。卵圆孔自然闭合和二尖瓣闭锁导致肺静脉回流受阻，致使她过早死亡。
