Outcomes of surgical repair of double-chambered right ventricle

We reviewed our experience with surgical repair of double-chambered right ventricle and long-term outcome. From November 1970 to February 2008, repair of double-chambered right ventricle was performed in 61 patients (31 males). The median age was 13 years (interquartile range, 2 months to 64 years); 10 patients were infants (16%). Mean preoperative right ventricular outflow tract pressure gradient was 67 ± 37 mm Hg. An associated ventricular septal defect was present in 50 patients (82%). There were 2 (3%) early deaths due to persistence of low cardiac output postoperatively, despite complete relief of the right ventricular gradient. The overall mean postoperative gradient was 2 ± 4.5 mm Hg. Late follow-up was complete in 92% (mean, 7.4 ± 7.9 years; maximum, 37 years). Late survival was 90% at 10 years. There were 3 late deaths due to heart failure in 2 patients and sudden death in 1 patient, all occurring before 1997. No patients required reoperation for residual or recurrent right ventricular obstruction. Surgical correction of double-chambered right ventricle results in excellent functional and hemodynamic long-term results, with complete relief of the right ventricular obstruction. The presence of a double-chambered right ventricle should be considered in anomalies with high or persistent right ventricular outflow tract obstruction.