Abstract
Pregnancy in patients with pulmonary arterial hypertension (PAH) is a high-risk condition associated with high morbidity and mortality. Patients with severe PAH are often advised against pregnancy. Still, those patients who pursue pregnancy require a dedicated and multidisciplinary approach since the progression of fetal growth will accompany significant hemodynamic changes, which can be challenging for patients with a poorly functioning right ventricle. In this article, we describe the approach to the unique cardiovascular, respiratory, hematologic, and social challenges that pregnant patients with PAH face throughout pregnancy. We discuss the impact of these physiologic changes on diagnostic studies commonly used in PAH and how to incorporate diagnostic data in making the diagnosis and risk stratifying pregnant patients with PAH. The pharmacologic challenges of pulmonary vasodilators in pregnancy are discussed as well. Pregnant patients with PAH are at particularly high risk of mortality around the time of delivery, and we discuss the multidisciplinary approach to the management of these patients, including the use of anesthesia, inotropic support, type of delivery, and postpartum care, providing clinicians with a practical approach to the management of this difficult condition.
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