Yoshida S, Yamagishi M, Syuntou K, Okano T, Yamada Y, Fujiwara K, Kitamura N
Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural Medical University, Kyoto, Japan.
Kyobu Geka. 2001 Aug;54(9):794-6.
A male infant with right isomerism, single ventricle, severe pulmonary stenosis (PS), and total anomalous pulmonary venous return (TAPVR) underwent successful staged Fontan operation. Pulmonary vein drained into right superior vena cava without a stenotic lesion. Common pulmonary venous chamber was also connected with the atrium by a thin bridging vein. Blalock-Taussig shunt was performed at 2-month-old. Because of progressive pulmonary congestion, an anastomosis between common pulmonary venous chamber and the atrium followed 4 days after the first shunt. Bidirectional cavopulmonary shunt was performed 7 months after the second operation. Because of progressive atrioventricular valve regurgitation, repeated bandings of the shunt were required to regulate the pulmonary flow. Total cavopulmonary connection was completed 9 months after the BCPS. Staged operation is a useful strategy to acquire an appropriate pulmonary blood flow for the isomeric heart with TAPVR and severe PS.
一名患有右位异构、单心室、严重肺动脉狭窄（PS）和完全性肺静脉异位引流（TAPVR）的男婴接受了成功的分期Fontan手术。肺静脉引流至上腔静脉右侧，无狭窄病变。共同肺静脉腔也通过一条细的桥静脉与心房相连。在2个月大时进行了Blalock-Taussig分流术。由于进行性肺充血，在第一次分流术后4天进行了共同肺静脉腔与心房之间的吻合术。在第二次手术后7个月进行了双向腔肺分流术。由于进行性房室瓣反流，需要反复绑扎分流以调节肺血流量。在双向腔肺分流术后9个月完成了全腔肺连接。分期手术是为患有TAPVR和严重PS的异构心脏获得适当肺血流量的有用策略。
