永存左上腔静脉畸形的临床意义与手术处理

Persistent Left Superior Vena Cava:Clinical Significance and Surgical Treatment

目的：永存左上腔静脉畸形（ＰＬＳＶＣ）常给心血管疾病的诊治带来困扰。随着心脏病学的发展，其临床意义愈来愈受到重视。本文试图确定在不同类型先天性心脏病中，ＰＬＳＶＣ的发生率、引流部位的类型及其对手术处理原则的影响。方法：统计不同类型先天性心脏病手术３１６８例，发现ＰＬＳＶＣ６７例，发生率２．１１％（１．２７％～１３．９５％）。ＰＬＳＶＣ引流部位：①经冠状静脉窦引流入右心房５７例；②经冠状静脉窦引流入右心房伴右上腔静脉缺如１例；③直接引流入右心房１例；④引流入冠状静脉窦开口于左心房２例；⑤直接引流入左心房６例。ＰＬＳＶＣ６７例中：术中暂时阻闭５２例，结扎５例，切开右心房插管１０例，其中单纯为了引流ＰＬＳＶＣ７例，建立心房内隧道将ＰＬＳＶＣ导入右心房２例，修补原发孔型心房间隔缺损时补片下缘向左心房后壁弯曲缝合将ＰＬＳＶＣ折流向右心房１例。结果：２例患者因ＰＬＳＶＣ漏诊和术中处理失误而死亡，死亡率３％。结论：①越是复杂的先天性心脏畸形中，合并ＰＬＳＶＣ的发生率越高；②ＰＬＳＶＣ术前确诊常较困难，术中探查甚为重要。漏诊常会带来严重后果；③ＰＬＳＶＣ的手术处理原则固然取决其引流部位类型，但ＰＬＳＶＣ与右上腔静脉之间有无?

Objective:As persistent left superior vena cava(PLSVC)causes difficulties in the diagnosis and treatment of cardiovascular diseases,increasing attention has been paid to the clinical significance of PLSVC.This study was aimed to assess the incidence,types of drainage position of PLSVC and its influence on the operative principles in various kinds of congenital heart diseases(CHDs).Methods:Among 3 168 patients with CHDs treated by open-heart operation,67 cases were found to have PLSVC with an incidence of 2.11%(1.27%-13.95%). The PLSVC drainage positions included:①into the right atrium (RA) through the coronary sinus(CS),57 cases;②into the RA through the CS without right superior vena cava(RSVC),1 case;③directly into the RA,1 case;④into the CS whose opening located in the left atrium(LA)complicated with atrio-ventricular septal defect, 2 cases;⑤directly into the LA, 6 cases. During the open-heart operations,the PLSVCs were dealt with:temporary occlusion in 52 cases,ligation in 5 cases,PLSVC cannulation after RA incision in 10 cases including:only for drainage from PLSVC in 7 cases,a tunnel was created from PLSVC to the RA in 2 cases,reconstruction of the intra-atrial septum with a patch(as a baffle) to guide PLSVC to RA in 1 case.Results:2 patients died due to incorrect treatment of PLSVC,the mortality was 3%.Conclusions:①The more complex the CHD, the higher incidence of the PLSVC;②Preoperative diagnosis of PLSVC is often difficult and intraoperative exploration is very important.The outcome of undetected PLSVC is serious;③The principle of PLSVC treatment depends on its types of drainage position,however,the presence or absence of free communication beween the RSVC and PLSVC has decisive effect on the operative methods chosen,especially for PLSVC draining into the LA.