房室管畸形的手术治疗

Surgical Repair of Atrioventricular Canal Defect

为提高房室管畸形手术疗效，总结１９８２年１月至１９９４年１２月纠治房室管畸形１３０例，其中部分性房室管畸形（ＰＡＶＣ）８８例，完全性房室管畸形（ＣＡＶＣ）４２例。ＰＡＶＣ手术方法采用间断缝合二尖瓣裂缺，心包补片关闭原发孔缺损；本组ＰＡＶＣ手术无死亡，１例术后３年发生二尖瓣重度反流，再次手术时死亡。ＣＡＶＣ采用单片心包补片方法；本组ＣＡＶＣ手术死亡１例，死亡率２．４％，死因为重度肺动脉高压；远期死亡２例，均为二尖瓣反流，肺部感染合并心力衰竭。结论：ＰＡＶＣ如无明显二尖瓣反流，手术可在３～４岁时做，而ＣＡＶＣ为了防止肺血管阻塞性病变，应该在６月龄内手术。

Aim:To review the experience of surgical repair of atrioventricular canal defect(AVCD).Clinical material and method:One hundred and thirty patients with atrioventricular canal defect underwent surgical repair under extracorporeal circulation from January 1982 to December 1994.Among them,88 patients had partial atrioventricular canal defect(PAVC)and 42 had complete atrioventricular canal defect(CAVC).The mean age was 5.5±2.8 years for PAVC group and 3.4±2.4 years for CAVC group.A mean body weight in PAVC group was 17.3±5.89 kg and in CAVC group was 11.47±4.17 kg.For patients with PAVC,the cleft of mitral valve was sutured and the primum atrial septal defect was repaired with pericardial patch which was also used for repairing AV septal defect in patients with CAVC.Results:In PAVC group,there was no postoperative death.In CAVC group ,one patient died of severe pulmonary hypertension postoperatively,giving an operative mortality of 2.4% and an overall mortality of 0.77% in the whole series.Two late deaths were caused by respiratory infection associated with heart failure attributed to residual mitral valve regurgitation.Another patient was reoperated on for sevtre residual mitral valve insufficiency 3 years after his first operation and died of DIC postoperatively.Conclusion:The date from this study suggest that the surgical results of AVCD are closely related to severity of mitral valve insufficiency.For patients with slight mitral valve insufficiency,surgical repair can be performed at 3 to 4 years of age.However,for those with CAVC surgical correction should be performed at age of 6 months to 1 year to prevent the development of progressive pulmonary vascular obstructive disease.