Uncorrected pentalogy of Fallot with successful pregnancy—A case report

Pentalogy of Fallot (PoF) is a congenital heart disease comprising tetralogy of Fallot plus an atrial septal defect (ASD). Patients are diagnosed early in life and submitted to reparative surgery. Without it, the prognosis is poor. This female patient, initially diagnosed with transposition of great arteries (TGA), ASD and a ventricular septal defect, got pregnant at 26 years old and had an early delivery due to fetal distress. She resumed follow-up, and her last echocardiogram put in doubt the diagnosis of TGA. Cardiac CT then revealed a PoF as well as pulmonary arteriovenous fistulas and a persistent left superior vena cava.