RESUMO
Patients with a single ventricle represent a rare abnormality found in 1% of patients with congenital heart disease, often discovered during childhood. Without pulmonary stenosis, the disease can progress to fixed pulmonary hypertension. Both pregnancy and delivery are risky events capable of increasing the right-to-left shunt. Pregnancy is contraindicated. We report the case of a 27-year-old woman with a single ventricle without pulmonary protection and fixed pulmonary hypertension at 60 mmHg, discovered during a pregnancy. The delivery was obtained by cesarean section with epidural anesthesia and the patient was perioperatively treated with nitric oxide. Though contraindicated, pregnancy and delivery were successfully achieved in this patient. Patients with single ventricle and Eisenmenger syndrome rarely reach adult life. Pregnancy with this condition is exceptional and fundamentally perturbs hemodynamic stability. In spite of the development of anesthesia and resuscitation and the description of some cases in literature, pregnancy with Eisenmenger syndrome is contraindicated.