Isolated Double-chambered Right Ventricle with Intact Interventricular Septum

Double-chambered right ventricle (DCRV) is a developmental cardiac anomaly in which anomalous muscle bundles divide the right ventricular (RV) cavity into two chambers. It is usually associated with other congenital cardiac defects, of which ventricular septal defect is the most common association. Isolated DCRV with an intact interventricular septum is very rarely reported. It manifests itself usually in adolescence and adults as the obstruction progresses gradually. It is important to recognize this anomaly as it can progress to severe RV failure if unaddressed. We report a 13-year-old male who presented to us with an isolated DCRV. He successfully underwent a resection of the RV cavity anomalous muscle bundles. We also stress on the utility of transesophageal echocardiography intraoperatively which accurately delineates the location of the muscle bundles and helps in evaluating the adequacy of resection postsurgery.