Isolated double chambered right ventricle as a rare cause of sudden death in infancy

Abstract

Isolated double-chambered right ventricle (DCRV) is a rare form of congenital heart disorder in which the right ventricle is divided into a high-pressure inlet portion and a low-pressure outlet portion by an anomalous muscle bundle. Rarely, autopsy cases describing this congenital heart disease have been reported in the world literature. We report the case of a 50-day-old male who died seven days after been suffering from fever and dyspnea. The autopsy revealed essentially an isolated double-chambered right ventricle (DCRV) and an important pulmonary edema. In this paper, we present the first reported case of sudden unanticipated death in infancy due to isolated double-chambered right ventricle.

Keywords

• Sudden death
• Congenital heart disease
• Double chambered right ventricle
• Autopsy