Fatal outcome of pregnancy in the Eisenmenger syndrome

A mildly symptomatic 28-year-old woman with Eisenmenger syndrome bt came pregnant against advice, and presented at 17 weeks insisting on continuing the pregnancy. She underwent an emergency Caesarean section fur pre-eclampsia at 26 weeks, delivering a live 620 g boy, who survived to leave the hospital. In the post-partum period, she presented increasing hypoxemia, despite treatment with noradrenaline, nitric oxide and prostacyclin. She died 2 weeks after delivery from pulmonary haemorrhage. Necropsy showed histological signs of severe pulmonary vascular disease, with evidence of diffuse fibrinoid necrosis, vasculitis and haemorrhage.