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目的探讨马方综合征(MFS)患者孕产期及产后心血管系统并发症的发生情况及处理方法。方法对1994年至2006年在北京安贞医院心外科就诊的30例 MFS 妊娠妇女的临床资料进行回顾性分析。结果 (1)30例 MFS 妊娠妇女中,3例产前检查中发现主动脉根部直径分别为50、53和58 mm;2例分别在孕24周和27周时发生主动脉根部瘤或夹层合并重度主动脉瓣反流、重度心力衰竭;产时和产后出现心血管并发症2例。1例足月分娩中发生急性 DeBakeyⅡ型主动脉夹层动脉瘤,另1例在产后第2天发现主动脉根部瘤、急性 DeBakeyⅢb型主动脉夹层动脉瘤、二尖瓣脱垂合并重度反流和左心功能不全;1例患者在接受二尖瓣机械瓣膜置换术(23岁)后10年妊娠,剖宫产前3 d 停用华法林,改用低分子肝素(其他名称:克赛)2000 U,抗凝治疗,每日2次皮下注射,母婴安全,未发生产后大出血、瓣膜血栓和细菌性心内膜炎,产后1年因主动脉根部瘤和主动脉瓣反流再次接受带瓣管道主动脉根部移植术;其余均为妊娠后4～28年接受心血管手术。(2)30例 MFS 妇女共有52例次妊娠,其中自然流产1例次、人工流产3例次;因心血管系统并发症而终止妊娠者2例次,经阴道足月分娩41例次(85%,41/48),因心血管系统并发症剖官产分娩5例次(10%,5/48)。产时和产后出现心血管系统并发症2例。(3)1例瓣膜置换术后患者安全妊娠和分娩,但1年后再次发生主动脉瘤变。(4)分娩至接受心血管手术的平均时间为(15±9)年,其后代 MFS 的遗传发生率为26%。结论 MFS 妇女妊娠后,如果不合并或合并轻度心血管系统病变,经阴道分娩是安全的;当合并重度心血管系统病变(主动脉夹层动脉瘤等)时,则以剖宫产分娩为宜。
Objective To investigate the incidence and treatment of cardiovascular complications during maternal and postnatal period in patients with Marfan syndrome (MFS). Methods The clinical data of 30 pregnant women with MFS who were treated in cardiac surgery at Anzhen Hospital of Beijing from 1994 to 2006 were analyzed retrospectively. Results (1) Of the 30 MFS pregnant women, the diameter of the aortic root was found to be 50, 53 and 58 mm in 3 prenatal examinations respectively. Two cases had aortic root tumor or dissection at 24 and 27 weeks’ gestation, respectively Severe aortic regurgitation, severe heart failure; intrapartum and postpartum cardiovascular complications in 2 cases. One case of acute DeBakey II aortic dissection occurred in term delivery and the other 1 case of aortic root tumor, acute DeBakey IIIb aortic dissection aneurysm, mitral valve prolapse with severe reflux and left Cardiac insufficiency; 1 patient underwent 10 years of gestation after mitral valve replacement (23 years), warfarin discontinuation 3 days before cesarean section, and low molecular weight heparin (other name) U, anticoagulant therapy, 2 times a day subcutaneous injection, maternal and child safety, no postpartum hemorrhage, valve thrombosis and bacterial endocarditis, 1 year postpartum due to aortic root tumor and aortic regurgitation again with valve Pipeline aortic root transplantation; the rest are 4 to 28 years after pregnancy for cardiovascular surgery. (2) A total of 52 pregnancies were found in 30 MFS women, including 1 spontaneous abortion and 3 induced abortion; 2 women who terminated pregnancy due to cardiovascular complications and 41 women who underwent vaginal delivery %, 41/48). Cesarean delivery due to cardiovascular complications occurred in 5 cases (10%, 5/48). Two cases of cardiovascular complications occurred during and after delivery. (3) One patient underwent valve replacement surgery was safe for pregnancy and childbirth, but aortic aneurysm occurred again after one year. (4) The mean time between delivery and receiving cardiovascular surgery was (15 ± 9) years, and the genetic incidence of MFS in its offspring was 26%. Conclusions Transvaginal delivery is safe if pregnant women with MFS do not have or merge with mild cardiovascular disease after pregnancy. When complicated with severe cardiovascular disease (aortic dissection aneurysm, etc.), cesarean section delivery is appropriate .