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伴有室间隔缺损和正常左心室的严重主动脉发育不全或闭锁行一期双心室矫治术的早期效果

世界核心医学期刊文摘（心脏病学分册） 2006年第11期2卷 60-61页

作者： Gruber P.J. Fuller S. cleaver K.M. 张步升 Children's Hospital of Philadelphia Philadelphia Pa United States Dr.

Objective: Biventricular repair of aortic atresia (or severe aortic hypoplasia) is possible in the presence of a ventricular septal defect and normal left ventricle. We considered whether primary biventricular repair was a safe alternative in all cases, even in the presence of interrupted aortic arch. Methods: This was a retrospective analysis of patients who underwent primary biventricular repair consisting of a combination Norwood-type reconstruction of the aortic arch, baffle of the left ventricle to both semilunar roots, and conduit placement from the right ventricle to pulmonary arteries. Results: Between January 1995 and January 2005, a total of 21 patients underwent primary biventricular repair at a median age of 5 days and a median weight of 3.0 kg. Aortic atresia was present in 7 and aortic stenosis in 14; 6 had interrupted aortic arch. All patients with aortic stenosis had annular diameters 3 mm or smaller. Median circulatory arrest time was 55 minutes, aortic crossclamp time was 56 minutes, and total support time was 99 minutes. In-hospital survival was 100%. Postoperative echocardiography in 19 patients demonstrated no significant outflow tract obstruction. Total stay was a median of 17 days. At midterm follow-up, there has been 1 late death, and reoperation has been necessary in 10 cases. Conclusion: Primary biventricular repair is a safe alternative to staged repair in all cases of aortic hypoplasia with ventricular septal defect and normal left ventricle.

关键词：室间隔缺损动脉发育不全双心室矫治术左心室主动脉弓离断辅助循环分期修复术动脉闭锁主动

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