Staged Fontan's operation for unguarded tricuspid orifice with pulmonary atresia.

We report a rare case of unguarded tricuspid orifice with pulmonary atresia, which is devoid of any guarding valvular tissue at tricuspid annulus. A left Blalock-Taussig shunt was constructed on the 17th day of life. At 4 years of age, a bidirectional Glenn's operation was performed for the further growth of the pulmonary bed and the volume reduction of the left ventricle. Finally, an extracardiac cavo-pulmonary connection was created using a ringed Gore-Tex graft at 6 years of age. Staged Fontan's strategy may contribute to a good surgical outcome for this highly lethal anomaly.