Pregnancy outcome in a pregnant patient with idiopathic Pulmonary Arterial Hypertension: a case report and review of the literature.

Abstract

Idiopathic pulmonary arterial hypertension is a rare and progressive condition which is aggravated by the physiologic changes during pregnancy. Because of high mortality rate, most physicians recommend early termination of pregnancy in patients with idiopathic pulmonary arterial hypertension.Here we describe a case of a 30-year-old primigravida Caucasianآ housewife with functional class 1 idiopathic pulmonary arterial hypertension and a positive vasoreactive response to adenosine who had a full-term non-complicated delivery. Right-sided heart catheterization before the pregnancy showed severe pulmonary hypertension with mean pulmonary arterial pressure of 60آ mmHg, and pulmonary vascular resistance of 12.2 WU. Vasoreactivity was positive after infusion of 200آ ?g/kg per minute adenosine. During pregnancy, she did not receive medication other than prophylactic enoxaparin. She had an elective cesarean section under general anesthesia at 39آ weeks of gestation without complication and delivered a healthy baby. After delivery, her hemodynamic status was stable. One month postpartum, she was in a stable clinical condition in functional class 1.In pregnant patients with pulmonary arterial hypertension, decreased mortality has been observed over recent yearsآ particularly in patients with well-controlled pulmonary pressure and a positive vasoreactivity test.