Objective: To analyze the clinical characteristics of pregnant women with different risk factors of Stanford type A aortic dissection (TAAD), so as to provide evidence for early warning and stratified management. Methods: A total of 42 pregnant or puerperal women with pregnancy-associated TAAD admitted to Beijing Anzhen Hospital, Capital Medical University from January 2010 to May 2026 were retrospectively enrolled. They were divided into three groups according to risk factors: Marfan syndrome (MF) group, hypertension (HBP) group, and non-identified high-risk factor (N) group before TAAD onset. The onset characteristics, aortic diameter and maternal-fetal outcomes were compared among the three groups. Results: All 42 patients with TAAD underwent aortic surgery, and 4 cases (10%, 4/42) died. The neonatal survival rate was 100% (26/26) in pregnant women with onset in the third trimester of pregnancy, while the fetal loss rate reached 6/9 in those with onset in the second trimester. Typical clinical symptoms, such as sudden severe tearing or knife-like chest and back pain, with or without radiating pain were observed in 74% (31/42) of patients. There were 10 cases (24%) in the MF group, 12 cases (28%) in the HBP group, and 20 cases (48%) in the N group. The median aortic root diameter was 38.5 mm (31.0, 48.3 mm) in the HBP group, which was significantly smaller than 51.5 mm (43.5, 63.5 mm) in the MF group and 50.5 mm (44.5, 67.0 mm) in the N group (all P<0.05). No statistically significant difference was detected between the MF group and the N group (P>0.05). In the HBP group, 6/12 of pregnant women had an aortic root diameter less than 40 mm, whereas all patients in the MF group and N group had an aortic root diameter of 40 mm or above. The mortality rates of TAAD in MF group, HBP group and N group were 1/12, 0/10 and 15% (3/20), respectively, and the live birth rates were 10/12, 10/11 and 71% (15/21), respectively, without statistically significant differences among groups (all P>0.05). Conclusions: Pregnancy-related TAAD is fundamentally based on underlying aortic structural abnormalities. Hypertension could lower the critical aortic diameter for dissection. Occult aortic lesions are common in TAAD patients without risk factors. It is suggested that aortic ultrasound screening should be advanced to pre-pregnancy or early pregnancy, and more active monitoring and intervention strategies should be taken for hypertensive patients with aortic lesions. 目的： 分析不同高危因素的妊娠合并Stanford A型主动脉夹层（TAAD）孕产妇的临床特征，为早期预警及分层管理提供依据。 方法： 纳入2010年1月至2026年5月首都医科大学附属北京安贞医院收治的42例妊娠合并TAAD孕产妇的临床资料进行回顾性分析，根据TAAD发病时的危险因素分为马方综合征组、高血压相关组、未见高危因素组，比较3组孕产妇的发病特点、主动脉根部直径及母儿结局。 结果： 42例妊娠合并TAAD孕产妇均行主动脉修复手术，其中4例（10%，4/42）死亡。妊娠晚期发病者新生儿存活率达100%（26/26），妊娠中期发病者胎儿丢失率为6/9。74%（31/42）的孕产妇表现为突发剧烈撕裂样或刀割样胸背痛，可伴或不伴放射痛等典型临床症状。42例妊娠合并TAAD孕产妇中，马方综合征组10例（24%）、高血压相关组12例（28%）、未见高危因素组20例（48%）。高血压相关组TAAD孕产妇的中位主动脉根部直径为38.5 mm（31.0，48.3 mm），显著低于马方综合征组［51.5 mm（43.5，63.5 mm），P=0.008］和未见高危因素组［50.5 mm（44.5，67.0 mm），P=0.002］，但马方综合征组与未见高危因素组TAAD孕产妇的中位主动脉根部直径比较，差异无统计学意义（P=0.887）。高血压相关组TAAD孕产妇主动脉根部直径<40 mm者占6/12，而马方综合征组和未见高危因素组所有孕产妇的主动脉根部直径均≥40 mm。马方综合征组、高血压相关组、未见高危因素组妊娠合并TAAD孕产妇的病死率分别为1/12、0/10、15%（3/20），活产率分别为10/12、10/11、71%（15/21），分别比较，差异均无统计学意义（P均>0.05）。 结论： 妊娠相关TAAD以主动脉结构异常为核心病理基础，高血压可降低主动脉夹层发病的临界直径。TAAD发病前未发现高危因素的孕产妇多存在隐匿性主动脉病变。建议将主动脉超声筛查前移至妊娠前或妊娠早期，对高血压合并主动脉病变者采取更积极的监测及干预策略。.