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