Pulmonary arterial hypertension in the setting of pregnancy: a case series and standard treatment approach.

ABSTRACT

Pregnancy in patients with pulmonary arterial hypertension (PAH) is associated with a maternal mortality of 30-50% despite modern treatment modalities. The majority of maternal deaths in PAH patients occur either during labor and delivery or within 1 month postpartum. Cardiovascular collapse is attributed to a mismatch between the physiologic limitations of PAH and the changes that occur with pregnancy and delivery. In the Unites States, there is no consensus on the management of PAH in pregnancy. Several case reports have been published describing improved maternal-fetal outcomes, likely due to new advanced PH therapies, earlier diagnosis of PAH, and an adoption of a multidisciplinary treatment approach. We present five cases of gravid PAH patients successfully managed at our institution with a description of our standardized multidisciplinary treatment approach.