主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治

目的 总结主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治经验.方法 2003年2月至2007年8月,一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压病婴6例,男、女各3例.手术年龄18 d～14个月,平均(8.3±4.5)个月;平均体重(8.5 ±2.2)kg.A型4例,B型2例.所有病例术前均有反复呼吸道感染史.手术均采用胸骨正中切口.结果 全组病例均生存.随访3～36个月,恢复良好.结论 一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压效果良好,需同时加强对围术期肺动脉高压的管理.

One stage repair of interrupted aortic arch associated with ventricular septal defect and pulmonary hypertension

Objective To summarize the clinical experience of one-stage repair of interrupted aortic arch (IAA) associated with ventricular septal defect and pulmonary hypertension. Methods Between February 2003 and June 2007, 6 patients (3 male, 3 fe- male) with IAA underwent total repair. The average age of patients was (8.3±4.5) months (18 days to 14 months) and the mean body weight was (8.5±2.2) kg. All patients had repeated respiratory infection. Four patients belonged to IAA type A, and2 to type B. All the patients had median sternotomy. IAA and associated ventricular septal defect was repaired. Results All patients survived and were followed up. No reoperation due to residual anomalies was required. Conehlsion Good results were obtained in one-stage repair of IAA associated with ventricular septal defect and pulmonary hypertension. More attention should pay to perioperative pulmo- nary hypertension and treat it effectively.