马方综合征患者孕产期及产后心血管系统并发症的临床分析  

Clinical analysis of pregnancy and postpartum cardiovascular complications in Marfan syndrome

作　　者：谢进生[1] 李斌[2] 孙衍庆[1] 关欣亮[1] 徐保卫[1] 乔志钰[1] 

机构地区：[1]首都医科大学附属北京安贞医院心外科,100029 [2]首都医科大学附属北京安贞医院妇产科,100029

出　　处：《中华妇产科杂志》2007年第11期742-744,共3页Chinese Journal of Obstetrics and Gynecology

基　　金：北京市科技新星计划(954813700)

摘　　要：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 management of pregnancy and cardiovascular complications in women with Marfan syndrome（MFS）. Methods From October 1994 to September 2006, 30 patients with MFS undergoing cardiovascular surgery were studied retrospectively. Results In the labor of 46 offsprings given birth by 30 women, 5 cases （ 11% ） were performed elective cesarean section because of the existence of aortic complication, and 12 （26%） were diagnosed as MFS. The gestation in two patients was terminated due to deterioration of aortic abnormalities during their third trimester, and they received surgical treatment with Bentall procedure. Two developed acute aortic dissection during labor and post delivery respectively. With the manipulation of anticoagulation peripartum, one who had the implantation with mechanical prosthesis went through pregnancy and delivery uneventfully. The average duration between delivery and cardiovascular surgery was （ 15±9） years. Conclusions Vaginal delivery can be done safely in patients with the MFS who do not have or have mild cardiovascular system abnormalities, aortic dissection, or other important cardiac abnormalities, cesarean section should be the preferred method of delivery. Women with MFS are at increased risk for dissection and congestive heart failure during pregnancy and should be counseled before pregnancy about these risks, as well as the inheritance of the condition.

关 键 词：马方综合征 妊娠并发症 心血管 心血管疾病 

分 类 号：R714.7[医药卫生—妇产科学] R54[医药卫生—临床医学]