Pregnancy in women with known and treated Budd–Chiari syndrome: Maternal and fetal outcomes

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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.

Methods

A retrospective study of pregnancy in women with known and treated BCS.

Results

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.

Conclusions

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.

Introduction

Budd–Chiari syndrome (BCS) is a rare disorder caused by the obstruction of hepatic venous outflow, leading to sinusoidal congestion, ischemic injury to the liver and portal hypertension. The main mechanisms for BCS is thrombosis of the hepatic veins, or of the terminal portion of the inferior vena cava [1], [2]. Multiple risk factors for venous thromboembolism, including various prothrombotic conditions, are usually present in BCS patients [1], [3], [4], [5].

Pregnancy is a crucial issue in women with BCS since the desire for pregnancy is increasingly expressed by these young patients once their condition has greatly improved [6]. However, these women could be at risk of developing severe exacerbation of their disease during pregnancy [7], [8]. As available reports on pregnancy in women with known BCS are scarce, it is unclear whether or not pregnancy should be contraindicated in patients with known and treated BCS [9], [10], [11], [12], [13], [14], [15], [16]. This prompted us to assess the fetal and maternal outcome in a group of women with a desire for pregnancy and who became pregnant after BCS had been recognized and treated.

Section snippets

Definitions

Date of diagnosis of BCS corresponded to the first imaging procedure showing an obstructed venous outflow tract [2]. Rotterdam prognostic index was calculated as initially described [17]. Miscarriages were defined as a spontaneous termination of pregnancy before 20 weeks’ gestation.

In order to identify its prognostic factors, pregnancy outcome was classified into 2 categories: (a) favorable outcome, when live birth occurred at 32 or more completed weeks of gestation, with a healthy infant and

General characteristics

Sixteen patients had 24 pregnancies during follow-up after BCS was diagnosed in one of the three referral centers from 1985 to 2005. Median age at conception was 33 years (range 21–41). Median time between diagnosis of BCS and conception was 57 months (range, 4–184). During the period preceding pregnancy, 9 out of the 16 patients underwent 11 liver decompression procedures, a median of 65 months (range, 5–174) before conception. Procedures included angioplasty in 4 patients, TIPS in 2, and

Discussion

A majority of the patients affected with BCS in Western countries are women of child-bearing age [1], [17], [29]. Many of these young women insistently express their desire for pregnancy. However, several previously reported observations suggest that pregnancy in BCS women could cause liver disease to deteriorate. Pregnancy was reported to induce the development of ascites in several women with known BCS [9], [12], [16]. Moreover, in older series of patients in India, women presenting with BCS

Acknowledgements

We thank Dr. Noëlle Bendersky, Alice Marot and Mohamed Achahboun for their help identifying patients, Béatrice Michel and Saida Roussin for their secretarial work.

Ciberehd is funded by Instituto de Salud Carlos III.

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    The authors who have taken part in this study declared that they do not have anything to disclose regarding funding from industry or conflict of interest with respect to this manuscript.

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