Maternal and perinatal outcomes of sickle cell disease in pregnancy: A nationwide study in France

Abstract

This nationwide cohort study provides a comprehensive overview of maternal and perinatal outcomes associated with sickle cell disease (SCD) during pregnancy. Using the French national health database, all singleton pregnancy-related hospital discharges from 2013 to 2020 in women aged 15-55 (n = 5 752 080) were selected. Of these, 1022 births were to women with SCD, 308 of whom were on long-term treatment, that is, hydroxyurea (HU) and/or transfusion programme. Pregnancies with SCD were more likely to involve pre-eclampsia (9.6% vs. 1.7%; p < 0.001), pulmonary embolism (0.70% vs. 0.02%; p < 0.001), caesarean sections (52.8% vs. 18.2%; p < 0.001) and postpartum haemorrhage (8.3% vs. 4.1%; p < 0.001) compared to pregnancies without SCD. Preterm birth (<37 weeks) was much more common in women with SCD (28.5% vs. 5.6%). Infants born to women with SCD faced greater adverse neonatal outcomes (22.4% vs. 8.0%; p < 0.001). Although untreated SCD was linked to fewer complications than long-term treated SCD, both conditions presented greater risks compared with pregnancies without SCD. Unexpectedly, babies born to women with SCD had a higher incidence of congenital abnormalities (6.3% vs. 3.4%; p < 0.001), not attributed to HU use. Overall, despite advances in SCD management, pregnancy in SCD remains a high-risk condition, for both mothers and babies.

Keywords: adverse neonatal outcome; aspirin; congenital abnormalities; hydroxyurea; perinatal and maternal outcomes; pregnancy; prematurity; pre‐eclampsia; sickle cell disease; transfusion.

FIGURE 1

Flow chart of the study population. Figure shows the flowchart of the study and the number of women included in the SCD and non‐SCD population. SCD, sickle cell disease.

FIGURE 2

Evolution of transfusion practices before, during, and after pregnancy in women with sickle cell disease (2013–2020). The bar chart displays the percentage of sickle cell disease women that received transfusions before, during and after pregnancy from 2013 to 2020. The orange trend line shows a declining trend in the percentage of pregnancies with transfusions over time (R ² = 0.6928). Each bar represents a specific year and is subdivided to highlight the timing of transfusions: Dark grey: Transfusions before pregnancy. Medium grey: Transfusions during pregnancy. Light grey: Transfusions after pregnancy.