No abstract

Background: Pregnancy is hazardous with pulmonary arterial hypertension (PAH), but the risks may have improved in recent years. We sought to systematically evaluate PAH and pregnancy-related outcomes in the last decade. Methods: We searched for articles describing outcomes in pregnancy cohorts published between 2008-2018. 3658 titles were screened and 13 studies included for analysis. Pooled incidences and percentages of maternal and perinatal outcomes were calculated.  Results: Out of 272 pregnancies, 214 pregnancies advanced beyond 20 gestational weeks. The mean maternal age was 28±2 years, mean pulmonary artery systolic pressure on echocardiogram was 76±19mmHg. Aetiologies include idiopathic PAH 22%, congenital heart disease 64%, and others 15%. Majority (74%) had good functional class I/II. Only 48% of women received PAH-specific therapy. Premature deliveries occur in 58% of pregnancies at mean of 34±1 weeks, most (76%) had caesarean section. Maternal mortality rate was 12% overall (n=26); even higher for idiopathic PAH aetiology alone (20%). Reported causes of death included right heart failure, cardiac arrest, PAH crises, pre-eclampsia and sepsis. 61% of maternal deaths occur at 0-4 days post-partum. Stillbirths rate was 3% and neonatal mortality rate 1%. Conclusions: PAH in pregnancy continues to be perilous with high maternal mortality rate. Continued prospective studies are needed.