Ramsey P S, Hay J E, Ramin K D
Department of Obstetrics/Gynecology, Mayo Medical Center, Rochester, Minnesota 55905, USA.
J Matern Fetal Med. 1998 Sep-Oct;7(5):235-7. doi: 10.1002/(SICI)1520-6661(199809/10)7:5<235::AID-MFM5>3.0.CO;2-H.
Budd-Chiari syndrome is a rare and serious thrombotic event with significant morbidity and mortality. Recommendations regarding future conception and management during pregnancy have not been defined. We present a patient with history of idiopathic Budd-Chiari Syndrome and subsequent orthotopic liver transplantation who was successfully managed during pregnancy. A 24-year-old white female, gravida 1 para 0, status postorthotopic liver transplantation 5 years previously for Budd-Chiari syndrome with post-transplant insulin-dependent diabetes mellitus presented to our clinic at 7 weeks of gestation for initial prenatal evaluation. Maintenance immunosuppressive therapy and prophylactic heparin anticoagulation was administered throughout the pregnancy, which was uneventful until 35 weeks gestation, at which time pre-eclampsia and premature preterm rupture of membranes prompted labor induction. The patient developed no evidence of acute or chronic hepatic rejection and no evidence of recurrent Budd-Chiari syndrome during the pregnancy or post-partum convalescence. Prudent use of prophylactic anticoagulation, close immunosuppressive monitoring, and periodic fetal and maternal surveillance are warranted in patients with previous orthotopic liver transplantation for idiopathic Budd-Chiari syndrome and may reduce risk of recurrence during pregnancy.
布加综合征是一种罕见且严重的血栓形成事件，具有较高的发病率和死亡率。关于未来妊娠及孕期管理的建议尚未明确。我们报告一例既往有特发性布加综合征病史并接受原位肝移植的患者，其孕期管理成功。一名24岁白人女性，孕1产0，5年前因布加综合征接受原位肝移植，术后患有胰岛素依赖型糖尿病，妊娠7周时前来我院门诊进行首次产前检查。整个孕期均给予维持性免疫抑制治疗及预防性肝素抗凝治疗，孕期进展顺利，直至孕35周时，子痫前期和胎膜早破促使引产。孕期及产后恢复期，患者未出现急性或慢性肝排斥反应，也无布加综合征复发迹象。对于既往因特发性布加综合征接受原位肝移植的患者，谨慎使用预防性抗凝治疗、密切监测免疫抑制情况以及定期进行胎儿和母体监测是必要的，这可能会降低孕期复发风险。
