Pregnancy and delivery in a patient with Uhl anomaly

We followed throughout pregnancy and delivery a 26-year-old woman suffering from a severe form of Uhl anomaly (congenital arrhythmogenic right ventricular dysplasia with severe enlargement and hypokinesis of the right ventricle, tricuspid valve insufficiency, and mild pulmonary valve insufficiency). Despite severely impaired cardiac function, the patient tolerated pregnancy surprisingly well. At 37.4 weeks' gestation, prophylactic low-dose heparin was started. Three days later she delivered a healthy 2700-g girl by elective cesarean performed under intensive cardiac surveillance.