Double-chambered right ventricle in an adult patient diagnosed by transthoracic echocardiography

Abstract

Background: Double-chambered right ventricle is a rare congenital disease frequently misdiagnosed in the adult patient. An anomalous muscle band divides the right ventricle in two cavities causing variable degree of obstruction. Although echocardiography is considered a useful method for the diagnosis of this pathology in children, it has been recognized the transthoracic scanning limitation in adults.

A case of asymptomatic isolated double‐chambered right ventricle in an adult man

“…DCRV is a rare congenital heart disease in which an anomalous muscle band divides the RV into two cavities, causing a variable degree of obstruction . The incidence of DCRV is unclear.…”

“…Forster and Humphries reported one case in 36,000 general autopsies with a 1–2.6% incidence in congenital diseases . There is no uniformity in the position of these anomalous muscle bundles or in the manner in which the RV is divided . The origin of the anomalous muscle bands is unknown .…”

“…It has also been suggested that the right ventricular subdivision and obstruction in this malformation represent an arrested incorporation of the primitive bulbus cordis into the right ventricular body . Associated congenital anomalies are present in approximately 80 to 90%, most commonly a membranous‐type ventricular septal defect . Other associated anomalies include pulmonary valve stenosis, double‐outlet RV, tetralogy of Fallot, anomalous pulmonary venous drainage, complete or corrected transposition of the great arteries, pulmonary atresia with intact ventricular septum, and Ebstein anomaly .…”

“…This may prove to be the most effective noninvasive means of imaging DCRV . Most patients with DCRV initially present with no symptoms . The most common reason for referral is the detection of murmur .…”

“…Double‐chambered right ventricle (DCRV) is a rare congenital heart disease in which an anomalous muscle band divides the right ventricle (RV) in two cavities, causing a variable degree of obstruction . The majority of patients with DCRV have associated congenital anomalies, most commonly a membranous‐type ventricular septal defect or a pulmonary stenosis . Most cases of DCRV are diagnosed and treated during childhood .…”