Surveillance of pulmonary vascular disease

Pre-existence or gestational occurrence of pulmonary vascular disease, including Eisenmenger's syndrome, primary and secondary pulmonary hypertension, is one of the rare conditions widely considered to pose an extreme risk of maternal death. Four of the nine maternal deaths in women with congenital heart disease reported in the UK in the last triennium were associated with pulmonary vascular disease. Eisenmenger's syndrome is estimated to carry a maternal mortality rate of 40% per pregnancy, with an infant mortality rate of 10-15%.

There were 30 confirmed cases of maternal pulmonary vascular disease in pregnancy; an estimated incidence of 0.6 cases per 100,000 maternities (95% CI 0.4 to 0.9 per 100,000). Two women died, both of whom continued with their pregnancies into the third trimester (overall case fatality 6.7%, 95% CI 0.8 to 22%; case fatality amongst women with continuing pregnancies 8.3%, 95% CI 1.0 to 27%). Neither maternal nor infant mortality appears to be as high as previously reported. However, we cannot exclude the possibility that this is because some women, particularly those with more severe disease, choose not to continue with their pregnancies.

NPEU Contact:
Marian Knight (
Chief Investigators:
Marian Knight (
Last updated:
Friday, 05 February 2016 14:03