Two cases of six-chambered hearts

Abstract

Double-chambered right ventricles (DCRV) and left ventricles are rare entities on their own. We present two cases with an unusual combination of double-chambered right as well as left ventricles. One was discovered in a 28-year-old female, while the other was found at birth in a female child. The differing nature of both the patient demographics as well as the presentation with a common morphological background is shown on both computed tomography and magnetic resonance imaging. The oldest description of an obstructive muscular band within the right ventricle was given in 1867. In the literature, there is ample description of the long-term prognosis and management guidelines for DCRV, but no inheritance patterns or risk factors have been identified except for associations with septal defects, tetralogy of Fallot and transposition of the great arteries. A combination of both double-sided left as well as right ventricles has been published in literature a few times with little details about management and prognosis. SIMILAR CASES PUBLISHED: Although many cases of DCRV and DCLV have been published, to our knowledge only 3 cases of combined DCRV and DCLV have been published in literature.

Mid-term Results of Double Chamber Right Ventricle in Association with Genetic Syndromes

“…Double chamber right ventricle is considered an acquired congenital heart defect caused by an abnormal membrane on the hypertrophic muscle band formation inside the RV (3). Various mechanisms of development of DCRV have been suggested.…”

“…Various mechanisms of development of DCRV have been suggested. Superior displacement of the moderator band especially in association with a VSD and flow turbulence in the RVOT may lead to DCRV, or DCRV may occur due to anomalous muscular bands causing obstruction (3). The frequent associations are tetralogy of Fallot, perimembranous VSD or transposition of great arteries (3).…”