Successful pregnancy in patient with homozygous sickle cell disease and prior pulmonary embolism – a case report

J. Notter, G. Amstad, B. Drexler, K. Loukidis (Basel, Switzerland)

Women issues in thrombosis and hemostasis
Date: 17.02.2017,
Time: 17:15 - 18:15

Objective: Pregnancy in patients with sickle cell disease (SCD) is associated with high risk of maternal and fetal morbidity and mortality. SCD seems to be an important thrombophilic condition, which is not fully understood yet, with higher risk of deep vein thrombosis and pulmonary embolism (PE), especially during pregnancy and post-partum. There are still unresolved issues regarding the optimal management of pregnancy in SCD. No recommendations were found concerning the management of anticoagulation in patients with prior PE, pregnancy and SCD, a particular high risk combination. To our knowledge , this is the first case report describing this combination. The objective of our paper is to describe one possible approach to patients with SCD, pregnancy and prior PE.

Methods: Case report.

Results: We describe the case and clinical management of a successful pregnancy of a 30-year-old patient with homozygous SCD and status after two miscarriages and PE. A screening for thrombophilia was negative. The patient was prior to the planned pregnancy under therapeutic dosage of low molecular weight heparin (LMWH) due to a PE 8 months prior and Hydroxyurea, which was stopped two months before conception. The LMWH was reduced to a prophylactic dosage during the pregnancy and Aspirin 100mg was added to reduce the risk of preeclampsia. The pregnancy was monitored closely with regular obstetric and hematological checks. The patient suffered several sickle-cell-crises, which were managed with analgesics and adequate hydration. Echocardiography, lung function test and abdominal ultrasound were unremarkable. The Aspirin was stopped at the beginning of the third trimester to reduce birth-related bleeding. Before delivery one exchange transfusion was conducted to minimize bleeding during the C-section and a generous hydration was maintained through the C-section to minimize the chance of a sickle-cell-crisis. A healthy baby girl was delivered full term. The LMWH was discontinued 3 months after delivery. The patient and baby are in a good condition 6 months after the delivery.

Conclusion: A successful pregnancy in a high-risk patient with SCD and prior PE was possible with the aid of LMWH and Aspirin. Interdisciplinary collaboration is recommended to achieve a successful outcome. Further prospective studies are needed to optimize management of pregnancy and PE in patients with SCD.