Pulmonary Arterial Hypertension in Pregnancy

PAH is a largely sex-specific disease affecting more women than men. Although the pathophysiology generally includes established pathways involving nitric oxide, prostacyclins, and endothelin, there are also genetic factors involved that predispose women to having PAH. The estrogen paradox is challenging to reconcile given that estrogen is thought to be more protective than harmful in PAH. Pregnancy is generally contraindicated in patients with PAH given the prohibitively high risk for maternal and fetal morbidity and mortality. Thus, preconception counseling and effective contraception are essential in patients with PAH. In patients who desire to proceed with pregnancy, PAH compounded by the physiologic changes of pregnancy can pose a significant hemodynamic burden that requires a multidisciplinary approach for care throughout pregnancy. The highest-risk period is the immediate postpartum period, when close monitoring and management are crucial. Although therapies have advanced, outcomes of patients with PAH and pregnancy are still poor, although they are improving.