Rautou Pierre-Emmanuel, Angermayr Bernhard, Garcia-Pagan Juan-Carlos, Moucari Rami, Peck-Radosavljevic Markus, Raffa Sebastian, Bernuau Jacques, Condat Bertrand, Levardon Michel, Yver Carine, Ducarme Guillaume, Luton Dominique, Denninger Marie-Hélène, Valla Dominique, Plessier Aurélie
Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Hôpital Beaujon, AP-HP and INSERM U773, Centre de Recherche Biomédicale Bichat Beaujon CRB3, Université Paris 7-Denis-Diderot, Clichy, France.
J Hepatol. 2009 Jul;51(1):47-54. doi: 10.1016/j.jhep.2009.02.028. Epub 2009 Apr 16.
BACKGROUND/AIMS: Budd-Chiari syndrome (BCS) mainly affects women of childbearing age. We aimed to clarify whether pregnancy, a thrombotic risk factor, should be contraindicated in patients with known and treated BCS.
A retrospective study of pregnancy in women with known and treated BCS.
Sixteen women had 24 pregnancies. Nine women had undergone surgical or radiological treatment. Anticoagulation was administered during 17 pregnancies. Seven fetuses were lost before gestation week 20. Deliveries occurred between week 20 and 31 in two patients, week 32 and 36 in eleven and after week 37 in four. There was one stillbirth, but 16 infants did well. Factor II gene mutation was a factor for a poor outcome of pregnancies. In two patients, symptomatic thrombosis recurred during pregnancy or postpartum. All patients were alive after a median follow-up of 34 months after the last delivery. Bleeding at delivery, although non-lethal, occurred only on anticoagulation therapy.
When known and treated BCS is well controlled, pregnancy should not be contraindicated as maternal outcome, and fetal outcome beyond gestation week 20, are good. The risk-benefit ratio of anticoagulant therapy needs to be further clarified. Patients should be fully informed of the persistent risks of such pregnancies.
背景/目的：布加综合征（BCS）主要影响育龄女性。我们旨在明确对于已知患有BCS且已接受治疗的患者，作为血栓形成危险因素的妊娠是否应被列为禁忌。
对已知患有BCS且已接受治疗的女性的妊娠情况进行回顾性研究。
16名女性共有24次妊娠。9名女性接受了手术或放射治疗。17次妊娠期间进行了抗凝治疗。7例胎儿在妊娠20周前流产。2例患者在20至31周分娩，11例在32至36周分娩，4例在37周后分娩。有1例死产，但16名婴儿情况良好。凝血因子II基因突变是妊娠结局不良的一个因素。2例患者在妊娠期间或产后出现有症状的血栓复发。末次分娩后中位随访34个月，所有患者均存活。分娩时出血虽未致命，但仅发生在抗凝治疗期间。
当已知患有BCS且已接受治疗的病情得到良好控制时，妊娠不应被列为禁忌，因为母亲的结局以及妊娠20周后的胎儿结局良好。抗凝治疗的风险效益比需要进一步明确。应让患者充分了解此类妊娠持续存在的风险。
