Successful Management of Pregnancy in a Patient with Systemic Lupus Erythematosus-associated Pulmonary Arterial Hypertension

Abstract

Pregnancy in women with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) remains a high risk. We successfully managed a pregnancy in a patient with SLE-PAH. A 31-year-old pregnant woman with SLE-PAH had worsening PAH and SLE flare-up during pregnancy and a sudden increase in pulmonary arterial pressure after delivery. SLE-PAH was controlled by continuous intravenous epoprostenol and inhaled nitric oxide therapy combined with high-dose corticosteroids under close hemodynamic monitoring. Women with SLE-PAH should avoid pregnancy. However, in case of a similar event, we recommend our case as a good reference for improving the outcome of pregnancy with SLE-PAH.

Impact of pregnancy in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: case series and literature review

“…A review of the literature included 14 pregnancy cases complicated by SLE-PAH in 11 studies from 2002 to 2021 13–18 24–28. Most patients (except for the case in Zhou et al 24) delivered via caesarean section (76.92%); the others delivered vaginally (23.08%).…”

“…Additionally, few publications and guidelines have focused on decision-making and management of pregnancy in women with SLE-PAH. Several recent case reports have shown that pregnancy is feasible in patients with SLE-PAH 13–20. These investigations suggested the possibility of successful pregnancy complicated by SLE-PAH and served as the foundation for future management and treatment guidelines.…”

“…Several recent case reports have shown that pregnancy is feasible in patients with SLE-PAH. [13][14][15][16][17][18][19][20] These investigations suggested the possibility of successful pregnancy complicated by SLE-PAH and served as the foundation for future management and treatment guidelines. This is the first study of pregnant women with SLE-PAH confirmed by right heart catheterisation (RHC) and the first systematic review of all eligible pregnancy cases complicated by SLE-PAH reported in previous studies.…”

“…Among the other six pregnancies without induced labour (including both planned pregnancies, cases 3 and 9), five patients delivered their neonates via caesarean section and one delivered vaginally. The median time of pregnancies completed in all patients, patients with induced labour and patients without induced labour was 31 weeks, 22 (15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25) weeks and 33 (31)(32)(33)(34)(35)(36)(37)(38) weeks, respectively. Of the 10 patients, 40% were administered general anaesthesia and 60% were administered continuous epidural anaesthesia.…”

Epoprostenol Exposure During Pregnancy

“…Importantly, in the last 2 decades, there have been multiple modern case reports and case series that have reaffirmed the safety and efficacy with the use of epoprostenol in parturients with pulmonary hypertension without evidence of preterm labor, bleeding complications, and/or detrimental fetal effects (5,(9)(10)(11)(12)(13)(14). The clinical efficacy, evidence for safety, and lack of demonstrated harm with exposure to IV or aerosolized epoprostenol in human studies has led to the recommendation for its use as first line in pregnant women with pulmonary hypertension (15).…”