Isolated double-chambered right ventricle with intact interventricular septum

Abstract

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.

Adult Double-Chambered Right Ventricle Associated With Ventricular Tachycardia and New-Onset Heart Failure

“…Double-chambered right ventricle (DCRV) is an uncommon form of heart disease in which the right ventricle is divided into two different chambers, a proximal high-pressure chamber and a distal low-pressure chamber, and is characterized by anomalous muscular bands (type 1) or marked parietal and septal muscle hypertrophy (type 2) [1]. Type 1 DCRV patients tend to lack associated defects while type 2 DCRV patients tend to have associated defects [2]. DCRV is often discovered in patients at a young age and corrected in their youth but may also present later on in adulthood.…”

Double-chambered right ventricle: The importance of a long-term follow-up

“…Two cases of double-chambered right ventricle (DCRV) in adult patients have been reported in previous issues of the Journal of Cardiovascular Echography. [ 1 2 ]…”