Potential Impact of Specific Therapy on Pregnant Women with Pulmonary Arterial Hypertension Without Cardiac Shunt:A Descriptive Study in Northern China

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 preg-nancy or within 6 weeks postpartum. Results:Although the overall mortality rate was encouraging(11.8％),the number of patients receiving PAH-specific therapies was ex-tremely 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 ex-pected to improve maternal outcomes in pregnant women with PAH.