Long-term survival of Uhl's anomaly with total cavopulmonary conversion.

We report a long-term survivor with Uhl's anomaly who underwent one and a half ventricle repair combined with a partial right ventriculectomy in infancy, followed by successful total cavopulmonary conversion with right ventricular exclusion 5 years later. The combination of total cavopulmonary connection and right ventricular exclusion could be the optimal surgical option for a critically ill infant with Uhl's anomaly.