Primary pulmonary hypertension in pregnancy; a role for novel vasodilators.

We describe the case of a 28-week pregnant woman presenting with severe primary pulmonary hypertension (PPH). She had an elective Caesarean section under general anaesthesia at 32 weeks gestation. Pulmonary artery pressures (PAP) measured from a pulmonary artery catheter before anaesthesia were in excess of 100 mm Hg. Intraoperative nitric oxide was used to reduce PAP. After the delivery of a healthy infant PAP was controlled with nebulized iloprost and a prostacyclin infusion. Seven days later she was discharged from intensive care taking an oral calcium antagonist and warfarin. She developed intractable right heart failure and died 14 days after delivery. Despite increasing experience in the use of drugs to reduce PAP, the clinical course of pregnancy complicated by severe PPH is usually fatal.