妊娠合并马方综合征增加不良妊娠结局风险，其最严重的并发症是主动脉夹层，危及母胎安全。现综述近年来国内外妊娠合并马方综合征的诊治进展，以期提高认识，加强孕前咨询和产前诊断，从而有助于早期识别和早期诊断，及时提供孕前咨询和围产期多学科规范化管理，改善母儿预后。“,”Pregnancy complicated by Marfan syndrome (MFS) could increase adverse pregnancy outcomes. The most serious MFS-induced complication is aortic dissection (AoD), which could endanger both the mother and the fetus. This article summarizes the research progress in China and overseas in the diagnosis and treatment of pregnancy complicated by MFS in recent years in order to raise awareness and improve prenatal diagnosis and pre-conception counseling, thereby facilitating early diagnosis and timely perinatal multi-disciplinary standardized treatment with a view to improving the prognosis.