Truncus arteriosis is a rare cyanotic congenital heart disease. It is a conotruncal heart anomaly characterized by a ventricular septal defect (VSD), a single truncal valve, a common ventricular outflow tract and pulmonary arteris arising directly from the truncus. In this case, truncal valve is quadricuspid, with a severe stenosis. Without surgical intervention, severe pulmonary hypertension and death in infancy is the rule. Definitive surgical correction within the first month of life must be performed. It consists of mobilization of pulmonary arteries from the truncus to the right ventricle with conduit and closure of the VSD with a patch.
