主动脉弓中断的外科治疗

目的 总结主动脉弓中断及合并心脏畸形的外科治疗经验.方法 1997年1月至2008年1月,36例主动脉弓中断患者进行了外科手术治疗,其中男性22例,女性14例.儿童患者36例,年龄2个月～7岁,平均年龄2.8岁.成人患者1例,年龄31岁.33例合并心内畸形,其中31例正中开胸同时矫治主动脉弓中断和心内畸形;1例左侧切口矫治主动脉弓中断,正中开胸修补心内畸形;1例采用姑息手术.3例无心内畸形的患者2例采用左后外侧切口,1例采用正中胸部加上腹部切口.术式包括16例管道连接,9例直接吻合,9例直接吻合并补片成形,1例应用左锁骨下动脉翻转.在31例正中切口一期手术中,17例应用选择性脑灌注加下半身停循环,8例采用深低温低流量灌注,6例采用全身停循环.结果 住院死亡5例,3例死于肺部感染,1例死于肺动脉高压危象,1例死于术后低心排血量.术后早期有其他重要并发症7例.31例存活患者随访3个月～5年,无远期死亡,无需要再次手术的病例.结论 合并心内畸形的主动脉弓中断患者可采取选择性脑灌注加下半身停循环或深低温全身低流量下正中一期手术同时矫治.

Outcome of surgery for interrupted aortic arch combined cardiac anomaly

Objective To retrospectively review the experience in repair of interrupted aortic arch (IAA) and associated cardiac anomaly. Methods From January 1997 to January 2008, 36 patients with interrupted aortic arch and associated cardiac anomaly underwent surgical treatment. There were 22 male and 14 female. Mean age of the 35 children patients was 2. 8 years, with a range from 2 months to 7 years. There was a 31 years old adult patient. Types of interrupted aortic arch include 30 cases of type A and 6 cases of type B. In all 36 patients, 33 cases had patent ductus arteriosus (PDA) and intracardiac abnormality, including 28 cases of simple anomaly as ventricular septal defect and 5 cases of complex anomaly, two cases were single IAA arch without PDA and other cardiac defect, one case had no intracardiac anomaly but PDA. For 33 patients with PDA and intracardiac anomaly, median sternotomy was used to simultaneously repair interrupted aortic arch and intracardiac defect in 31 cases, left thoracotomy and median sternotomy were applied to repair IAA and intracardiac anomaly respectively in one case, one patient had palliative repair. For three patients without intracardiac anomaly, left thoracotomy was applied in two cases, median sternotomy and abdominotomy were used in one adult patient. Techniques of operation for interrupted aortic arch include 16 cases of conduit connection, 9 cases of direct anastomosis, 9 cases of direct anastomosis with patch augmentation, 1 case of subclavian flap aortoplasty. In all 31 cases of one-stage operation through median sternotomy, selective cerebral perfusion was used in 17 patients, deep hypothermia and low flow were applied in 8 cases, deep hypothermia circulatory arrest was performed in 6 patients. Results There were 5 hospital deaths. Three cases died of pulmonary infection, 1 case died of of pulmonary hypertension crisis, and another case died of postoperative low cardiac output, which was misdiagnosed before operation. Seven cases had other main postoperative complications. Thirty-one survivors were followed up from 3 months to 5 years, there was no late death and reoperation. Conclusion One-stage repair through median sternotomy using selective cerebral perfusion or deep hypothermia and low flow can be applied to most of the cases with associated cardiac anomaly.