升主动脉成形人工血管外包裹治疗儿童马方综合征

目的:探讨升主动脉外人工血管包裹术(Robicsek手术)对儿童马方综合征主动脉根部扩张症的诊治及疗效。方法:1996年9月至2001年7月共收治儿童马方综合征9例,其中6例存在主动脉根部扩张症,治疗采用升主动脉外人工血管包裹术。3例在全麻非体外循环下进行升主动脉外包裹手术;3例在体外循环下行升主动脉外包裹手术,其中2例伴有主动脉瓣关闭不全,1例行同种瓣移植,1例行机械瓣置换。结果:1例术后2 d死于急性左心衰竭,5例存活,随访2~5年效果满意。结论:升主动脉外包裹术是治疗儿童马方综合征主动脉根部扩张症的一种较好选择。

Surgical treatment of aortic root dilatation of Marfan's syndrome in chlildren using Robicsek technique

Objective： To review the experience of surgical treatment of aortic root dilatation of Marfan＇ s syndrome in children using Robicsek technique. Method：From September 1996 to July 2001,9 children with Marfan＇ s syndrome were involved, of which 6 cases with aortic root dilatation underwent Robicsek technique. 3 patients underwent Robicsek technique without cardiopulmonary bypass; 3 with cardiopulmonary bypass, in which 2 existed aortic valve regurgitation, and one underwent homograft, one underwent mechanism valve replacement. Result： 5 patients were survivor, one died for left heart failure after two days since operation, results were satisfactory during the follow-up of 2-5 years, Conclusion： Robicsek technique is a good selection in treating Marfan＇s syndrome of children.