S265: maternal and perinatal outcomes of sickle cell disease in pregnancy: a nationwide study in france.

Topic: 26. Sickle cell disease Background: Sickle cell disease (SCD) is an inherited disease affecting the ß-chain of hemoglobin. With over 300,000 newborns per year, it is the world’s most common hemoglobinopathy. Improved care for people with SCD has led to higher survival rates, which explains the increasing number of women with SCD who reach childbearing age. Pregnancy in women with SCD has been linked to a high risk of severe maternal and perinatal complications. In the largest and most recent meta-analysis of studies from both low- and high-income countries (encompassing 26,349 pregnant women with SCD), pregnancy was associated with an elevated risk of maternal mortality (relative risk (RR) [95% confidence interval (CI)] = 5.98 [1.94-18.44]), preeclampsia (2.43 [1.75-3.39]), stillbirth (3.94 [2.60-5.96]), preterm delivery (2.21 [1.47-3.31]), and small-for-gestational-age infants (3.72 [2.32-5.98]). In France, the management of pregnancy in SCD follows national guidelines since 2015 and these patients are theoretically referred to centers trained in this disease but recent national data are lacking. Aims: In the present prospective, nationwide study in France, we sought to describe maternal and fetal outcomes of pregnancy in women with SCD vs. women without SCD. Methods: The French national health database (SNDS) was queried for all pregnancy-related hospital discharges between 2013 and 2020. The SNDS is a claim database that links the French national healthcare reimbursement database and the French national hospital discharge database (covering all general hospitals and university medical centers in France). All singleton pregnancies (n=5,752,080) by women aged between 15 and 55 were then selected. Twin pregnancies and women with less than one year of medical history were excluded because of the greater risk of complications and missing data in women newly diagnosed with SCD. We searched for SCD-related “full coverage of medical costs” by the French national health insurance as of beginning of pregnancy using ICD code. We could not distinguish SCD genotypes (SS, SC, Sβ0, Sβ+, etc.) because this information is not specifically coded after hospitalization. Results: There were 1022 deliveries by women with SCD (308 with treated SCD, i.e., hydroxyurea (HU) and/or transfusion program before pregnancy -, and 714 with untreated SCD). Pregnancies among women with SCD were more likely to feature preeclampsia (1.7% vs. 9.6%; p<.001), pulmonary embolism (0.02% vs. 0.7%; p<.001), caesarean section (18.2% vs. 52.8%; p<.001) and postpartum hemorrhage (4.1% vs. 8.3%; p<.001). Preterm deliveries (< 37 week of gestation) were much more frequent in women with SCD (28.5%, vs. 5.6% in other women, p<.001). Children born to women with SCD were more likely to have difficulty adapting to extrauterine life (22.4%, vs. 8% for children born to women without SCD; p<.001). In women with untreated SCD, likely to be mild disease, these adverse outcomes were less common than in women with long-term treatment, but significantly more common than in general population. Despite these, maternal and perinatal mortality were not significantly increased in the SCD population. Unexpectedly, we found that newborns of women with SCD were more likely to have congenital malformations than those in the general population (6.3% vs 3.4% respectively, p<.001). This excess incidence was not related to hydroxyurea prescribed before or during pregnancy in our cohort. Summary/Conclusion: Although they have been major improvements in the management of SCD in and out of pregnancy in a high-income country, SCD remains a high-risk situation for women and the unborn baby. Special attention and care are therefore needed for all women with SCD, whether they have mild or severe disease.Keywords: Pregnancy, Sickle cell anemia, Sickle cell disease