马方综合征的外科治疗

SURGICAL TREATMENT OF MARFAN SYNDROME

目的：总结４８例马方综合征（旧译名马凡氏综合征）的外科治疗与结果。方法：分析了４８例马方综合征病人的临床资料，男性４１例，女性７例，年龄分布２０～７３岁，平均（３８±３）岁。马方综合征升主动脉瘤直径４．５～１１．８ｃｍ，平均（６．９±２．５）ｃｍ，全部病人有中重度主动脉瓣关闭不全，均由主动脉瓣及主动脉窦高度扩张所致。病理检查示主动脉壁中层均呈囊性坏死，弹力纤维断裂及平滑肌细胞退行性变。结果：４１例进行了手术治疗，其中Ｂｅｎｔａｌ’ｓ手术３８例，Ｗｈｅａｔ’ｓ手术３例。手术死亡３例。非手术组７例，其中死亡５例（心力衰竭死亡２例，动脉瘤破裂２例，恶性室颤１例），自动出院２例。结论：马方综合征合并有升主动脉瘤及严重主动脉瓣关闭不全时，均应及时早期手术治疗。

ABSTRACT Objective:To review the experiences and results of the surgical treatment in 48 cases of Marfan syndrome.Methods: The clinical status of 48 patients, including 41 males and 7 females was analyzed. Their ages ranged from 20 to 73 [mean (38±3)] years old and the diameters of ascending aortic aneurysm with Marfan syndrome ranged from 4.5 to 11.8 cm [mean (6.9±2.5) cm]. All the patients had mild to severe aortic insufficiency despite normal aortic leaflets. The aortic valve dysfunction was caused by dilation of the sinus of Valsalva and annuloaortic ectasia. The pathological examination showed cystic change, elastic fragmentation and the degenerated smooth muscle cells of the media of aortic wall.Results: Of the 41 patients in surgical group, 38 received Bentall's operation and 3 Wheat's, with 3 operative deaths. 7 patients were in nonsurgical group (heart failure in 2, aneurysm rupture in 2 and ventricular fibrillation in 1). Conclusion: Marfan syndrome with ascending aortic aneurysm and severe aortic regurgitation obviously indicate prompt surgical treatment. The operative results are better than medical therapy.