Abstract Number: PB2538
Meeting: ISTH 2020 Congress
Background: Women with Budd Chiari Syndrome (BCS) and/or portal hypertension are at high risk of pregnancy complications and adverse outcomes. Decreased liver function, underlying prothrombotic disorders combined with physiological changes during pregnancy underlie the observed high rates of maternal and fetal morbidity. There is no consensus on optimal treatment strategies throughout pregnancy. Clinical practice with regard to counselling these women planning for pregnancy is heterogenous and some physicians advise against pregnancy.
Aims: To evaluate management and outcomes of pregnancies in women with BCS and/or portal hypertension.
Methods: We performed a retrospective single center cohort study between 2008 and 2019. Women with confirmed BCS and/or portal hypertension who conceived at least once were enrolled and study outcomes were assessed up to 12 weeks after delivery or 6 weeks after pregnancy loss. The primary outcome was maternal pregnancy-related mortality and birth of a living fetus. Secondary outcomes included maternal, fetal and obstetrical complications.
Results: Eighteen women (3 BCS of whom 2 with portal hypertension and 15 with portal hypertension) conceived at least once (24 pregnancies overall). Ten women had a history of venous thrombosis, mostly portal vein thrombosis (N=7). Underlying prothrombotic disorders included myeloproliferative neoplasms (N=4), protein C deficiency (N=2), antiphospholipid syndrome (N=1) and cirrhosis (N=5) [Table 1]. Six of ten women with a history of thrombosis received low-molecular-weight heparin during pregnancy. Of 18 first pregnancies, 12 resulted in live birth, 5 in pregnancy loss and 1 was ectopic [Table 2]. Six women conceived twice. No maternal deaths or thrombotic events were observed.
Conclusions: Adverse pregnancy outcomes in women with BCS and portal hypertension are common. However, in our small cohort, the chance of term live birth of 56% seems too high and maternal mortality too low to consider BCS or portal hypertension an absolute contra-indication.
| Patient Characteristics | All patients (N=18) | Patients with prior VTE (N=10) | Portal vein thrombosis (N=7) | Budd Chiari Syndrome (N=3) | |
| Age at conception (years), mean ± SD | 30.3 ± 6.3 | 30.4 ± 6.3 | 29.1 ± 5.9 | 30.3 ± 4.6 | |
| BMI (kg/m2), mean ± SD | 23.5 ± 3.3 | 23.7 ± 2.9 | 22.1 ± 2.5 | 25.3 ± 1.9 | |
| History of miscarriage, n (%) | 4 (22) | 3 (30) | 2 (29) | 0 | |
| Myeloproliferative neoplasms, Protein C deficiency, Antiphospholipid syndrome, Cirrhosis | 4 (22), 2 (11), 1 (6), 5 (28) | 3 (30), 2 (20), 1 (10), 0 | 2 (29), 2 (29), 0, 0 | 1 (33), 0, 1 (33), 0 | |
| Antithrombotics prior to pregnancy, n (%); LMWH, VKA, DOAC | 6 (33); 1 (6), 5 (28), 0 | 6 (60); 1 (30), 5 (50), 0 | 3 (43); 1 (14), 2 (29), 0 | 2 (67); 0, 2 (67) | |
[Patient Characteristics]
| Pregnancy outcomes | All patients (N=18) | Patients with prior VTE (N=10) | |
| Live birth, n (%); gestational age, median (range) | 12 (67); 39 (27- 41) | 5 (50); 40 (39 – 41) | |
| Pregnancy loss, n (%); early miscarriage <10 weeks GA, late miscarriage 10-20 weeks GA, Fetal death >20 weeks GA | 5 (28); 3 (17), 1 (6), 1 (6) | 4 (40); 3 (30), 1 (10), 1 (10) | |
| Ectopic pregnancy, n (%) | 1 (6) | 1 (10) | |
| Birth weight total (grams), median (range) | 2993 (850 – 4165) | 3035 (2725 – 3250) | |
| Mode of delivery, n (%); vaginal, caesarean section | 8 (67), 4 (33) | 4 (80), 1 (20) | |
| Antithrombotics during pregnancy, n (%); LMWH, Aspirin | 6 (33); 6 (33), 0 | 6 (60); 6 (60), 0 | |
[Pregnancy outcomes]
To cite this abstract in AMA style:
Hamulyák EN, Wiegers HMG, van Duuren JR, Middeldorp S, Ganzevoort W. Pregnancy Outcomes in Women with Budd Chiari Syndrome or Portal Hypertension [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/pregnancy-outcomes-in-women-with-budd-chiari-syndrome-or-portal-hypertension/. Accessed October 1, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/pregnancy-outcomes-in-women-with-budd-chiari-syndrome-or-portal-hypertension/