The clinical presentation and long-term follow-up of 14 cases of tricuspid atresia associated with pulmonary atresia were reviewed. The electrocardiograms, hemodynamic findings and a definition of anatomic types were outlined in order to facilitate therapeutic decisions. In these types of tricuspid atresia the clinical presentation depends on the patent ductus. Despite the caliber of the ductus arteriosus, the poor tolerance to the malformation is frequent and the clinical presentation is similar to those malformations with decreased pulmonary blood flow (cyanosis since birth and hypoxic spells). In considering the age of these patients, the modified Blalock-Taussig anastomosis is the initial procedure. The Fontan procedure should be considered carefully as an alternative in older children.