小儿房室管畸形外科治疗

Surgical Treatment of Pediatric Atrioventricular Canal Defect

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 experiences of surgical treatment for Pediatric atrioventricular canal defect.Methods Thirty-eight patients with surgical treatment for atrioventricular canal defect from May 2005 to July 2010 were analyzed retrospectively,in which 31 had partial atrioventricular canal defect（PACD） and 7 had complete atrioventricular aanal defect（CACD）.The cleft of mitral valve presented in all PACD patients.The cleft of mitral valve was repaired with interrupted suture and strengthened with continuous suture on cleft in 27,and the cleft was not repaired because of light mitral regurgitation in 4.The ostium primum atriaI septal defect was repaired with autologous pericardium in all cases.Coronary sinus was situated on the left atrium in 20 and ostium primum atrial septal defects were repaired in Kirklin＇s way;11 in MeGoon＇s way.Atrioventricular septal defects were repaired in CACD by Double-patches with Dacron and autologous pericardium in 4 cases and patches with autologous pericardium in 3.Results One early death happened because of severe low output syndrome.There were low cardiac output in 6 and Grade Ⅲ°AV Bin 1 case that turned to sinus rhythm after 7 days.Thirty cases were followed up from 2 months to 6 years.Cardiac functions were ranged from class Ⅰ～Ⅱ.Conclusion The key points of operation for PACD are to rectify the mitral insufficiency,repair ostium primum atrial septal defects and avoid atrioventricular block.It is emphasis that annulus of mitral and tricuspid valve should be rectified to correct anatomical abnormalities to recover area of inter-ventricular septum and widen left ventricular outflow tract for CACD.