Maternal and fetal outcomes of pregnant women with pulmonary arterial hypertension associated with congenital heart disease in Beijing, China: A retrospective study

As pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) may increase maternal and fetal risk, this study explored the pregnancy outcomes of Chinese women with PAH-CHD. The clinical data of pregnant women with PAH-CHD admitted to the Beijing Anzhen Hospital from 2010 to 2019 were retrospectively analyzed; these patients and their offspring were followed up, with a mean period of 5.9 +/- 2.7 years. Overall, 260 patients with PAH-CHD were included. The mean maternal age was 27.7 +/- 4.1 years, and 205 (78.8%) patients were nulliparous. The estimated systolic pulmonary artery pressure was 40-50 mmHg in 34.6% of the patients, 50-70 mmHg in 23.1%, and >70 mmHg in 42.3%. More than 96% of patients were diagnosed with PAH-CHD before pregnancy. During pregnancy, heart failure occurred in 19.2% of the patients. Cesarean delivery was performed in 88.1% (15.0% emergency) of the patients. Complications included fetal distress (5.8%), preterm delivery (34.2%), and low birth weight (33.8%). A total of 15 mothers (5.8%) died, with the highest mortality rate in those with Eisenmenger syndrome (10/43, 23.3%), and 10 offspring died (3.8%), two (0.8%) following hospital discharge and eight (3.1%) while in hospital. Although most pregnant women with PAH-CHD were able to have children, PAH increased the maternal and fetal risk. Thus, an individualized risk-based approach with shared decision-making may be more appropriate in pregnant women with PAH-CHD.