Pregnancy with uncorrected tetralogy of Fallot (TOF), pulmonary atresia and major aorto-pulmonary collateral arteries (MAPCA)

Pregnancy with uncorrected tetralogy of Fallot (TOF) is a rare event, and increases the risk for fetal growth restriction and adverse outcomes related to worsening right-to-left shunt, cyanosis, pulmonary hypertension and arrhythmia. Obstetric management is made on a case-by-case basis. Patients who remain stable can achieve term pregnancies and the mode of delivery can be based on obstetric indications. We present the case of a 21-year-old primigravida with baseline cyanosis, oxygen saturation 90% at rest (74% with activities), hematocrit 50%, and echocardiogram showing pulmonary atresia, ventricular septal defect (VSD), and major aorto-pulmonary collateral arteries (MAPCA). We managed her with aspirin, metoprolol and oxygen supplementation. She delivered at 38 weeks (fetal growth restriction) via cesarean; she received epidural and endocarditis prophylaxis. Three years later, she presented pregnant and had a repeat cesarean at term. Both newborns had normal structural hearts. Knowledge of the cardiovascular physiology during pregnancy and baseline assessment, and a multidisciplinary management are key aspects in the management of these cases during pregnancy.