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目的总结小儿心脏房室管畸形外科治疗的经验。方法选择手术治疗房室管畸形患儿38例,其中部分型房室管畸形31例,完全型房室管畸形7例。部分型房室管畸形均合并二尖瓣大瓣裂,瓣裂修补采用间断褥式缝合并连续缝合加固27例,反流较轻未处理4例;原发孔型房间隔缺损采用自体心包连续缝合补片修补,20例采用Kirklin的方法将冠状静脉窦开口隔入左心房,11例采用McGoon法将冠状静脉窦开口保留在右心房。完全型房室管畸形7例。按Rastalli分型属A型4例,C型3例,分别采用自体心包加涤纶片的双片法(4例)或自体心包单片法(3例)修复。结果早期死亡1例。术后发生低心排出量综合征6例;Ⅲ度房室传导阻滞1例,7d后恢复窦性心律。术后随访30例,随访时间2个月～6年,心功能均有明显改善,为Ⅰ～Ⅱ级。结论部分型房室管畸形手术治疗的关键是完善修复二尖瓣关闭不全和原发孔型房间隔缺损,避免房室传导阻滞。完全型房室管畸形除注重房室瓣修补完善外,还需将二尖瓣和三尖瓣环纠正至正常解剖位置,以恢复室间隔的面积和增宽左室流出道。
Objective To summarize the experience of surgical treatment of atrioventricular canal in pediatric heart. Methods Thirty-eight children with atrioventricular canal were selected for surgery. Among them, 31 were partial-type atrioventricular canal and 7 were complete-type atrioventricular canal. Some type of atrioventricular deformity were mitral valve mitral valvuloplasty, valvular repair using intermittent mattress suture and continuous suture consolidation in 27 cases, mild reflux unprocessed in 4 cases; primary perforation of atrial septal defect with autologous pericardial continuous Suture patch repair, 20 cases of Kirklin method of coronary sinus openings into the left atrium, 11 cases of McGoon method will be retained in the right atrium of the coronary sinus opening. Complete type of atrioventricular canal in 7 cases. According to Rastalli classification, there were 4 cases of type A and 3 cases of type C, which were repaired by autologous pericardium plus dacron slice (4 cases) or autologous pericardial monolithic method (3 cases). Results of early death in 1 case. Postoperative low cardiac output syndrome in 6 cases; Ⅲ degree atrioventricular block in 1 case, after 7 days to restore sinus rhythm. Thirty patients were followed up for 2 months to 6 years. The heart function improved significantly, which was grade Ⅰ ~ Ⅱ. Conclusion The key to surgical treatment of partial atrioventricular canal malformations is to improve the repair of mitral regurgitation and primary atrial septal defect and avoid atrioventricular block. Complete atrioventricular canal deformity in addition to atrioventricular valve repair attention, but also need to correct the mitral and tricuspid annulus to the normal anatomic position to restore the area of the interventricular septum and widened the left ventricular outflow tract.