Zagzoog Mohammad M, Crawford Sean A, Davaine Jean-Michel
Department of Vascular and Endovascular Surgery, Pitié-Salpetriere University Hospital, Sorbonne Université, Faculty of Medicine, Paris, France.
Faculty of Medicine, Sorbonne University, Paris, France.
J Vasc Surg Cases Innov Tech. 2024 Jul 2;10(5):101561. doi: 10.1016/j.jvscit.2024.101561. eCollection 2024 Oct.
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.
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。我们报告一例29岁的孕妇，患有急性B型主动脉夹层。由于心肺失代偿和肠道灌注不良，她接受了紧急剖宫产，随后进行了左锁骨下动脉至颈动脉转位术和胸主动脉腔内修复术，但该手术并发了逆行A型主动脉夹层，并通过手术进行了处理。分子检测确诊为马凡综合征。该病例强调，对急性主动脉综合征等具有挑战性的病例进行多学科和混合治疗可取得良好的效果。
