Maruyama T, Totsuka N, Akahane K, Yoshioka J, Shinohara M, Kouzu S, Fujii N, Yajima H, Shimotori M
Department of Cardiology, Nagano Red Cross Hospital.
Kokyu To Junkan. 1993 Jan;41(1):85-8.
Two women with Marfan syndrome who developed aortic dissection during pregnancy are reported. Case 1: A 38-year-old woman had experienced severe back pain five years before (at the age of 33) when she was in the 38th week of gestation, followed by labour with no problem. Three years ago, she was examined in our hospital for enlarged thoracic aorta pointed out in a somatoscopy and was diagnosed as having Marfan syndrome and aortic dissection of DeBakey type IIIb. Because her aortic dissection has been growing larger recently, surgical repair is scheduled electively. Case 2: A 32-year-old woman, who had been diagnosed as having Marfan syndrome because of ectopia lentis at 10 years old, was referred to our department for a painful tumor with pulsation which had emerged at the right-hand side of her neck in the 32nd week of gestation. Acute aortic dissection of DeBakey type I complicated with acute aortic regurgitation was diagnosed, and emergency operation (Cesarean section, reconstruction of ascending aorta and aortic arch, replacement of aortic valve and aorto-coronary bypass to right coronary artery) was performed. Post-operative course was uneventful and the patient and her baby were discharged safely. In general, aortic dissection during pregnancy of patients with Marfan syndrome is of poor prognosis. Full consent by the patient and her family is necessary to decide whether the pregnancy should go on, and close cooperation among participating departments is indispensable for the treatment for this condition.
报道了两名患有马凡综合征的女性在孕期发生主动脉夹层的病例。病例1：一名38岁女性在5年前（33岁时）妊娠38周时曾经历严重背痛，随后顺利分娩。3年前，她因体格检查发现胸主动脉增宽来我院就诊，被诊断为马凡综合征和DeBakey IIIb型主动脉夹层。由于其主动脉夹层近来增大，计划择期进行手术修复。病例2：一名32岁女性，10岁时因晶状体异位被诊断为马凡综合征，在妊娠32周时因右侧颈部出现搏动性疼痛性肿物转诊至我科。诊断为DeBakey I型急性主动脉夹层并伴有急性主动脉瓣反流，遂行急诊手术（剖宫产、升主动脉和主动脉弓重建、主动脉瓣置换及右冠状动脉主动脉冠状动脉搭桥术）。术后过程顺利，患者及其婴儿安全出院。一般来说，马凡综合征患者孕期发生主动脉夹层预后较差。决定是否继续妊娠需要患者及其家属完全同意，对于这种情况的治疗，各参与科室之间密切合作必不可少。
