Double-chambered right ventricle (DCRV) is a form of congenital heart disease wherein a mid-cavitary obstruction divides the right ventricle into a high-pressure proximal portion and a low-pressure distal portion. However, it has also been reported to be an acquired disease that can develop postnatally from progressive hypertrophy in the crista supraventricularis or other muscle structures within the right ventricular cavity in some patients with small, restrictive VSDs.
DCRV can be classified into 2 main types based on the tissue responsible for the mid-cavitary obstruction. Patients with type 1 DCRV have anomalous muscle bundles that cross the right ventricle, while type 2 DCRV has distinct parietal and septal muscle hypertrophy responsible for the obstruction. The most commonly associated anomaly is a membranous ventricular septal defect (seen in up to 75% of patients with DCRV). DCRV can also occur concomitantly with tetralogy of Fallot, double outlet right ventricle, Ebstein anomaly, transposition of the great arteries, ruptured sinus of Valsalva aneurysm, atrial septal defect, quadricuspid aortic valve, persistent left superior vena cava, and valvar pulmonary stenosis.
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