Kristen Oelschlager, RN

Patients with congenital heart disease and pulmonary arterial hypertension (PAH) are at risk for severe deterioration during pregnancy and delivery. We discuss the case of a 38-year-old woman who presented to the emergency department complaining of dyspnea 6 days after giving birth to her first child via cesare- an section. When PAH is untreated, maternal mortality may exceed 50%, but aggressive PAH treatment offers improved outcomes. Moreover, initial improvement in functional status made with parenteral prostanoids can be maintained with combination oral therapy.