Successfully completed total cavopulmonary connection with a right-sided maze procedure after a modified Starnes' operation in a neonate with Ebstein's anomaly.

The symptomatic newborn infant with Ebstein's anomaly is in critical condition and is difficult to treat successfully. Furthermore, supraventricular tachyarrhythmia in patients with Ebstein's anomaly may determine the early and late results. We report a successfully treated case of extracardiac total cavopulmonary connection with a right-sided maze procedure after a modified Starnes' operation during the neonatal period.