内脏异位综合征合并心脏畸形的外科治疗

摘要

Objective To evaluate the results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly.Methods From September 2008 to October 2013,34 patients with heterotaxy syndrome underwent 46 cardiac operations at Guangzhou Women and Children Medical Center.22 were male,and 12 female.Mean age at operation was 22 months (4 months to 14 year).There were right atrial isomerism 24 cases,left atrial isomerism 10 cases.Only one had a single VSD,and others were all with complex cardiac anomaly,including complete atrioventricular canal defect 26 cases,tricuspid atresia 4 cases,mitral atresia 1 case,anomalous pulmonary venous connection 18 cases,pulmonary venous stenosis 4 cases,double superior vena cava 17 cases,and interrupted hepatic portion of the inferior vena cava 5 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results There were 4 died during hospitalization.The follow-up duration was 1 month to 5 years.There were 2 died.The early to middle term mortality was 17.6％ (6/34).In the same period the mortality for 103 patients without heterotaxy syndrome underwent univentricular repair was 4.8％ (5/103).There was significant statistical difference (P ＜ 0.05).Conclusion Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.%目的 评价合并内脏异位综合征先天性心脏病外科治疗的效果.方法 2008年9月至2013年10月,34例内脏异位综合征合并心脏畸形患儿(婴)行手术治疗46例次.男22例,女12例;年龄4个月～14岁,中位年龄22个月.右心房异构24例,左心房异构10例;1例为单纯室间隔缺损,33例伴复杂心内畸形,其中完全性房室间隔缺损26例,肺静脉异位引流18例,双上腔静脉17例,下腔静脉中断5例,三尖瓣闭锁4例,肺静脉梗阻4例,二尖瓣闭锁1例.32例采用单心室矫治手术,其中双向Glenn术13例次,肺动脉环缩术5例次,Fontan手术5例次,瓣膜置换术5例次,双侧双向Glenn术4例次,Kawashima手术3例次,中央分流术1例次;9例采用多种术式.结果 住院死亡4例;随访1个月～5年,死亡2例,早、中期死亡比例17.6％(6/34),与同期103例未合并内脏异位综合征行单心室系列手术患儿(婴)早、中期死亡比例4.8％(5/103)差异显著(P＜0.05).结论 单心室矫治系列手术是治疗内脏异位合并心内复杂畸形的主要手术方式,术后早、中期效果良好.