Severe form of Ebstein's anomaly associated with ventricular septal defect.

Although the ventricular septal defect (VSD) that coexists with the severe form of Ebstein's anomaly was thought to be beneficial for hemodynamics, it is unclear whether biventricular repair is always possible. Ebstein's anomaly with VSD was diagnosed at 4 days of age based on a heart murmur and mild cyanosis. The cardiothoracic ratio was 78% and the Celermajer index was 1.6 (grade 4), but the hemodynamics were stable and tricuspid valve regurgitation was less than mild. Pulmonary overcirculation developed, and therefore, biventricular repair was attempted at 4 months of age, consisting of VSD patch closure, atrial septal defect partial closure, and the cone reconstruction of the tricuspid valve. Due to persistent cyanosis, however, nitric oxide inhalation and high flow nasal oxygen inhalation could not be discontinued. Catheter examination showed a cardiac index of 2.1 L/min/m2, moderate tricuspid regurgitation, and a pulmonary-to-systemic blood flow ratio of 0.64, and thus, takedown to one and one-half ventricular circulation using bidirectional superior cavopulmonary anastomosis, atrial communication closure, and right pulmonary artery banding was performed. The patient was discharged on postoperative day 25. Four months after discharge, the patient is doing well with home oxygen therapy.