CAJ | 학술논문

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.
목적 총결주동맥궁중단급합병심장기형적외과치료경험.방법 1997년1월지2008년1월,36례주동맥궁중단환자진행료외과수술치료,기중남성22례,녀성14례.인동환자36례,년령2개월～7세,평균년령2.8세.성인환자1례,년령31세.33례합병심내기형,기중31례정중개흉동시교치주동맥궁중단화심내기형;1례좌측절구교치주동맥궁중단,정중개흉수보심내기형;1례채용고식수술.3례무심내기형적환자2례채용좌후외측절구,1례채용정중흉부가상복부절구.술식포괄16례관도련접,9례직접문합,9례직접문합병보편성형,1례응용좌쇄골하동맥번전.재31례정중절구일기수술중,17례응용선택성뇌관주가하반신정순배,8례채용심저온저류량관주,6례채용전신정순배.결과 주원사망5례,3례사우폐부감염,1례사우폐동맥고압위상,1례사우술후저심배혈량.술후조기유기타중요병발증7례.31례존활환자수방3개월～5년,무원기사망,무수요재차수술적병례.결론 합병심내기형적주동맥궁중단환자가채취선택성뇌관주가하반신정순배혹심저온전신저류량하정중일기수술동시교치.
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.