Purpose of Review <p>Pulmonary arterial hypertension (PAH) is a rare but progressive cardiopulmonary disease that disproportionately affects women of childbearing age, and has high associated maternal and fetal morbidity and mortality. We discuss the physiologic changes that account for these poor outcomes. We review the core principles of managing PAH in this population, and more specifically discuss the management by trimester, from pre-conception counseling through the post-partum period.</p> Recent Findings <p>Under non-pregnant states, patients with PAH have elevated pulmonary pressures that predispose them to right heart failure and untimely death. The physiologic changes of pregnancy pose hemodynamic threat to these patients by placing additional strain on the right heart. With advancing therapies, multidisciplinary care, and clinical experience, pregnancy outcomes have somewhat improved, particularly for those with low-risk disease.</p> Summary <p>Early and effective pre- and post-conception counseling are essential for patients with PAH. For those who elect for pregnancy or those who are diagnosed intra-partum, early multidisciplinary care is essential for risk-stratification, mitigation, and treatment.</p>