摘要目的 总结8例Cantrell五联症病例的手术经验.方法 2007年7月至2009年6月在心外科、胸外科及普外科协作下,采用相应的手术方式矫治8例Cantrell五联症病儿,其中男6例,女2例;平均年龄7.35岁.心内畸形包括室间隔缺损合并房间隔缺损或卵圆孔未闭4例、右室双出口2例,单纯房间隔缺损和单纯室间隔缺损各1例.结果 1例于右室双出口矫治手术后2年二期行心脏回纳手术,手术切开左侧部分肝冠状韧带及部分肝镰状韧带,向下推移肝脏左叶,打开双侧胸膜,切除双侧肋缘部分肋软骨,上拾胸壁,回纳心脏到胸腔,并采用Proceed补片修补膈肌、腹壁缺损外,其余7例均在双侧心包及纵隔胸膜充分打开的同时将第7、8肋软骨横断、闭合两侧肋弓行胸廓成形术,即均一期行心脏畸形矫治及心脏回纳手术.8例心内及心外畸形矫治满意,其中例2术后出现残余漏,经心内科介入封堵后治愈;例4左心室憩室病儿,行憩室切除,残端缝闭术;例6右室双出口者根治手术后出现右心功能不全,对症治疗后恢复.术后随访1～23个月,手术效果满意,无严重不良并发症发生,生活状况良好.结论 精确矫治Cantrell五联症的心脏畸形,打开双侧胸膜,切断肋软骨上抬胸壁,扩大心脏回纳空间,必要时人工补片修复膈肌缺损,本畸形多能达到一期治疗目的 .

abstractsObjective To summarize surgical experience of eight patients with pentalogy of Cantrell. Methods Six male and two female patients with pentalogy of Cantrell,aged from 4 months to 26 years old, average 7.35 years old, underwent surgical therapy for intracardiac anomalies and extracardiac anomalies from July 2007 to June 2009. Eight case with intracardiac anomalies include one case with only VSD, one case with only ASD, two cases with DORV, four cases with VSD and ASD or PTO. Experts majoring in cardiovascular surgery cooperated with doctors majoring in thoracic surgery and general surgery for satisfactory correction of intracardiac anomalies and extracardiac anomalies and repositioning heart to thoracic cavity. Results Ectopic heart of the first patient was simply repositioned into thoracic cavity following surgery of double outlet of right ventricle in another hospital two years before. Correction of introcardiac anomaly and reposition of ectopic heart finished at one time in 7 cases. Eight patients got full recovery except that residual shunt occurred in the second case which also got full recovery after transcatheter therapy. Ventricular diverticulum was removed in the fourth case because of difficult reposition of ectopic heart.With the help of general surgeon and thoracic surgeon, partial coronary ligament of liver and falciform ligament of liver in the left was cut in the first case and the left half lobe of liver was pushed downward. Bilateral pleural and marginal costal costochondral was cut and make thoracic wall upward so that ectopic heart can reset into thoracic cavity. And then, defect of diaphragm and abdominal wall were repaired with Proceed patch. In the other seven cases, bilateral pericardium and mediastinal pleura was cut and the 7th and 8th cartilage was transected and bilateral costal arch was closed so for complete thoracic angioplasty.Left ventricular dysfunction occurred in the fifth case with DORV and also got full recovery after symptomatic treatment. Full recovery was got in all cases after followingup from 1 to 23 months. No adverse complications occurred and every case live a wonderful life. Conclusion Pentalogy of Cantrell can be cured at one time by accurate correction of cardiac anomalies, cutting of bilateral pleural and marginal costal costochondral to make thoracic wall upward and enlarge thoracic space for repositinning of ectopic heart and using artificial patch to repair defect of diaphragm when necessary.