Hirose H, Amano A, Takahashi A, Nagano N, Kohmoto T
Department of Cardiovascular Surgery, Shin-Tokyo Hospital, Chiba, Japan.
Ann Thorac Cardiovasc Surg. 2000 Aug;6(4):275-80.
Cardiovascular malformations are frequently observed in Turner's syndrome. Bicuspid aortic valve and coarctation of the aorta are commonly associated with Turner's syndrome whereas aortic dissection is rare but its rupture results in death. We experienced a case of ruptured dissecting aneurysm (Stanford type A) in a 30-year-old female with Turner's syndrome. Emergent total arch replacement was performed successfully. A literature review revealed 32 cases of aortic dissection in patients with Turner's syndrome, including 15 cases of rupture. However, survival after rupture was reported only two cases. To our knowledge, this report descries the third known case of successful surgical management of ruptured aortic dissection in Turner's syndrome.
心血管畸形在特纳综合征中经常被观察到。二叶式主动脉瓣和主动脉缩窄通常与特纳综合征相关，而主动脉夹层很少见，但其破裂会导致死亡。我们遇到一例30岁患有特纳综合征的女性发生了夹层动脉瘤破裂（斯坦福A型）。成功进行了急诊全弓置换术。文献回顾显示，有32例特纳综合征患者发生主动脉夹层，其中15例破裂。然而，仅报道了2例破裂后的存活病例。据我们所知，本报告描述了第三例已知的成功手术治疗特纳综合征患者主动脉夹层破裂的病例。
