Introduction: Double-chambered right ventricle (DCRV) is a rare congenital heart defect in which the RV cavity is divided into a proximal high-pressure chamber and a distal low-pressure chamber by anomalous muscle bundles usually in the setting of a ventricular septal defect. Limited data are available on the outcomes following surgical repair in children. Our objective was to describe the immediate perioperative and mid-term outcomes for children who underwent repair for DCRV at our institution.
Methods: Children undergoing repair for DCRV at our institution were identified between December 2010 and December 2021. Surgical repair was performed in 12 patients (7 males, 5 females). Data obtained included demographics and echocardiographic data, in addition to intraoperative and postoperative data. Follow-up data were included when available. Descriptive statistical analysis was performed. Results are reported as median (interquartile range).
Results: Median age at repair was 44 months (3 months – 14 years). VSD was present at the time of repair in all but one patient, who had a VSD that spontaneously closed. Median pre-operative right ventricular outflow tract (RVOT) peak gradient was 40 (16.5 - 65.5) mmHg. Median cardiopulmonary bypass time was 65 (63 - 96) minutes and cross-clamp time was 48.5 (29.5 - 68) minutes. There was no operative mortality. The median hospital length of stay was 4.5 days and the median mechanical ventilation days was 1 day. One patient had postoperative low cardiac output and refractory junctional ectopic tachycardia (JET) requiring ECMO support. The patient was able to be weaned off of ECMO after 7 days with resolution of cardiac dysfunction and was discharged home without further complications after 15 days. The median RVOT peak gradient at the time of dismissal was 9.5 (5 - 12.25) mmHg. Follow-up was obtained in 10 patients with median duration of 6 years (7 months – 7 years). There were no deaths during this time. The median RVOT peak gradient was 8 (5.5-14) mmHg on most recent follow-up. Recurrent RVOT obstruction occurred in one patient. His peak gradient at dismissal was 27 mmHg and 4 years following repair his gradient increased to 85 mmHg requiring surgical intervention. Conclusion Surgical results for DCRV repair are excellent with complete relief of RVOT obstruction. Early mortality is low, and recurrence of obstruction is uncommon.
