妊娠合并主动脉夹层病情凶险，容易误诊、漏诊，母儿不良结局风险极高，且与夹层分型密切相关，病因主要与原发高血压、先天性和遗传性血管性疾病相关，妊娠是其好发因素。胸痛为主要临床表现，诊断主要依靠影像学检查。治疗包括支持治疗、药物治疗、传统手术治疗、主动脉瘤腔内修复术等，治疗方式、分娩时机、分娩方式根据主动脉夹层累及范围与类型、孕周、患者及家属意愿和医疗条件综合决定。加强孕前与孕期保健、多学科评估与救治是降低不良母胎结局的关键。

Pregnancy with aortic dissection is dangerous and easy to be misdiagnosed and to escape diagnosis. The risk of adverse outcomes in mother and child is extremely high, which is closely related to the classification of the dissection. The cause of aortic dissection in pregnancy is mainly related to primary hypertension, congenital and hereditary vascular disease, pregnancy is the risk factor. Chest pain is the main clinical manifestation, and the diagnosis mainly depends on imaging examination. Treatment includes supportive therapy, drug therapy, traditional surgical treatment, thoracic endovascular aneurysm repair, etc. The treatment, timing of delivery and mode of delivery are determined according to the extent and type of aortic dissection, gestational age, patient and family wishes, and medical conditions. Strengthening pre-pregnancy and pregnancy care, multidisciplinary assessment and treatment are the keys to reduce poor outcome of mother and fetus.