66例部分性房室管畸形的外科治疗

目的总结部分性房室管畸形手术治疗的经验，以提高治疗效果。方法回顾性分析我院1984年1月－2007年12月经外科治疗部分性房室管畸形患者66例的临床资料，对二尖瓣大瓣裂的处理：单纯裂缺缝合52例，缝合加交界折叠缝缩8例，加小瓣成形1例，加缝置St．Jude软质人工成形环3例，人工机械瓣置换术2例；对原发孔型房间隔缺损的修补：采用涤纶补片修补12例，自体心包54例；采用Kirklin法将冠状静脉窦口隔入左心房5例，MeGoon法将冠状静脉窦口隔入右心房61例；同期处理其他合并畸形。结果术后早期死亡2例（3．03％），1例死于心律失常，另1例死于呼吸功能衰竭。术后发生Ⅲ°房室传导阻滞2例，均安装永久性心脏起搏器。术后随访52例（81．3％），随访时间5个月～22年，平均15年，心功能均有明显改善，尤其是术前心功能Ⅲ～Ⅳ级的19例患者，术后改善为Ⅰ～Ⅱ级。再次手术4例，其中1例经再次行二尖瓣置换术治愈；术后死亡3例，1例死于急性。肾功能衰竭，2例死于低心排血量综合征。结论早期手术治疗可以保全房室瓣结构、功能，避免发生肺动脉高压和降低死亡率。手术治疗的关键是消除二尖瓣关闭不全、修补原发孔型房间隔缺损和避免损伤传导组织，术后近、中期疗效良好；有残留中度以上二尖瓣反流者，远期效果不满意。

Surgical Treatment of Partial Atrioventricular Canal Defect in 66 Cases

Objective To summarize the experiences of surgical treatment for partial atrioventricular canal defect. Methods The data of 66 patients of surgical treatment for partial atrioventricular canal defect from January 1984 to December 2007 were analyzed retrospectively. The cleft of mitral valve presented in all of those patients. There were 52 cases with direct suture on cleft, 8 cases with direct suture with commissurroplasty, 1 case with posterior leaflet plasty, 3 cases with direct suture St. Jude ring and 2 cases mitral valve replacement. The ostium primum atrial septal defects were repaired with patches of Dacron in 12 cases and autologous pericardium in 54 cases. Coronary sinus was situated on the left atrium in 5 and ostium primum atrial septal defects were repaired in Kirklin＇s way; the others in MeGoon＇way. Meanwhile other heart abnormalities were done. Results There were two early deaths （3.03 %）, one patient died of heart arrhythmia and one patient died of respiratory failure. Complications of total A-V block was in 2 cases. Both of them were replanted with pace makers. 52 cases were followed up, follow-up time was 5 months to 22 years（mean follow-up 15 years）. All patients had better life. Four patients have been re -operated for different reasons post primary operation. One had good result after re-mitral valve replacement. One case died of acute renal failure and the other two died of low cardiac output syndrome. Conclusions Early operation is definitely recommended when the diagnosis is confirmed. Because the structure or function of mitral valve is saved, pulmonary hypertension is avoided and the mortality is lower in the future. The key points of operation are to rectify the mitral insufficiency, repair ostium primum atrial septal defects and avoid atrioventrieular block. The patients of mild regurgitation of mitral valve have good results. Provided in those have more than middle regurgitation of mitral valve then their tong-term results are poor.