Various modifications of Fontan-Kreutzer procedure have evolved over the years. The available literature was reviewed in an attempt to evaluate whether one of these operations is preferable to others. Although there is no complete agreement, direct atriopulmonary anastomosis in the absence of a subpulmonary ventricular chamber, and atrioventricular connection via a homograft conduit in patients with a good-sized (greater than 30% of normal), well-formed (with both trabecular and outlet portions) right ventricle appear to be reasonable choices. In patients with more than the usual risk or at low risk for the Fontan-Kreutzer procedure, other alternatives (namely, total cavopulmonary connection for patients with asplenia/polysplenia syndrome and systemic venous anomalies and bidirectional cavopulmonary anastomosis or Fontan-Kreutzer with a small temporary atrial septal defect) could be considered. The role of experimental skeletal muscle augmentation or replacement of right heart chambers and cardiac transplantation in the corrective treatment of tricuspid atresia needs to be defined as more data become available.