Potential impact of specific therapy on pregnant women with pulmonary arterial hypertension without cardiac shunt: a descriptive study in northern China

Abstract Background Pregnancy in women with pulmonary arterial hypertension (PAH) is a fatal condition, despite the effectiveness of PAH-specific therapies. The coverage status and effect of specific therapies in pregnant patients with PAH without cardiac shunts in China remain unclear. To investigate this issue, we conducted a multicenter retrospective study in northern China. Methods The study included 85 patients who were admitted to 4 clinical centers in Shandong Province between October 2010 and August 2020. Maternal endpoint events included (1) maternal death and/or (2) major adverse cardiac events, both occurring during pregnancy or within 6 weeks postpartum. Results Although the overall mortality rate was encouraging (11.8%), the number of patients receiving PAH-specific therapies was extremely low (28.2%). Moreover, only 15.3% of patients received adequate duration of PAH-specific therapy (≥4 weeks) before delivery, and this subgroup showed the lowest major adverse cardiac events rate (7.7%) compared with that in the untreated (19.7%) and short-time treated groups (<4 weeks; 54.5%). Conclusion Pregnant patients with PAH without cardiac shunts face significantly increased mortality risks. Short-term PAH-specific therapy does not guarantee favorable maternal outcomes. Prepregnancy screening, early identification, and timely intervention are expected to improve maternal outcomes in pregnant women with PAH.