Successful maternal and fetal outcomes have been demonstrated with an aggressive team management approach, early delivery, and detailed postpartum care.

Pulmonary arterial hypertension (PAH) is a rare disease characterized by progressive dyspnea and exercise intolerance, with death due to right-sided heart failure. Before 1985, median survival was 2.8 years from diagnosis, with no known effective therapies.11 Epoprostenol was the first medication approved in 1996, and since that time, significant advances in treatment have occurred, including the addition of multiple medication therapies leading to improved management of symptoms.22,44 The prognosis of PAH, although improved, remains poor, with a 15% mortality during the first year of treatment.55,66 

The pathophysiology of PAH includes complex endothelial dysfunction leading to remodeling of the wall of the pulmonary artery, causing exaggerated vasoconstriction and impaired vasodilatation. This...

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