Mohammad M. Zagzoog MD , Sean A. Crawford MD , Jean-Michel Davaine MD, PhD
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引用次数: 0
摘要
马凡综合征是一种罕见的遗传性结缔组织疾病,可导致严重的发病率和死亡率。我们报告了一例 29 岁孕妇急性 B 型主动脉夹层的病例。由于心肺功能失代偿和肠道灌注不良,她接受了紧急剖宫产手术,随后进行了左锁骨下至颈动脉转位术和胸腔内主动脉血管修补术,术后并发了逆行性 A 型主动脉夹层,并通过手术进行了处理。分子检测证实了马凡综合征的诊断。该病例突出表明,对具有挑战性的急性主动脉综合征病例进行多学科和混合治疗可获得良好的疗效。
Complicated type B aortic dissection in a pregnant woman with Marfan syndrome
Marfan syndrome is a rare inherited connective tissue disorder that can result in significant morbidity and mortality. We report a case of a 29-year-old pregnant woman presenting with an acute type B aortic dissection. Owing to cardiopulmonary decompensation and intestinal malperfusion, she underwent an emergency cesarean section followed by left subclavian to carotid transposition and thoracic endovascular aortic repair that was complicated by a retrograde type A aortic dissection and was managed surgically. Molecular testing confirmed the diagnosis of Marfan syndrome. This case highlights that multidisciplinary and hybrid management of challenging cases of acute aortic syndromes can result in a favorable outcome.
期刊介绍:
Journal of Vascular Surgery Cases and Innovative Techniques is a surgical journal dedicated to publishing peer review high quality case reports, vascular images and innovative techniques related to all aspects of arterial, venous, and lymphatic diseases and disorders, including vascular trauma, malformations, wound care and the placement and maintenance of arterio-venous dialysis accesses with an emphasis on the practicing clinician. The Journal seeks to provide novel and timely information to vascular surgeons, interventionalists, phlebologists, wound care specialists, and allied health professionals involved with the management of patients with the entire spectrum of vascular disorders.