1. Double-chambered right
ventricle
Ramachandra

2. Background
DCRV is a rare CHD characterized by right ventricle outflow tract
obstruction by an anomalous muscle band that divides right ventricle
(RV) into proximal high pressure and distal low pressure chamber
 0.5–2% of all cases of CHD
most reported cases in patients less than 20 years old
First case 130 years back
Most well studied in 1970 around
45% of cases had more than one or nondiscrete muscle bundles
Male-to-female ratio is 2:1.

3. Continued…….
Localisation of the obstructive muscle varies and may be either high (or
horizontal), adjacent to the pulmonary valve, or low (or oblique), close to the ape
DCRV is exceptionally rare as an isolated anomaly
 associated[80-90%] lesions VSD,PS, and SAM
DD- TOF/ isolated infundibular stenosis/small VSD
DCRV should strongly suspected in ECHO if RV hypertrophy is present in absence of
infundibular hypertrophy or valvular pulmonary stenosis
Surgical intervention should be considered in all patients who are symptomatic or have
peak gradients of ≥50 mm Hg
 The surgical approach resects the muscle bundles and repairs any associated lesions
There is little recurrence of obstruction after adequate surgical repair
 There have been isolated attempts at percutaneous alcohol ablation of the conal branch
from the right coronary artery and the use of balloon dilatation

4. The next most common associated lesion is pulmonary valve stenosis.
Various other associations have been reported, including double outlet
RV, tetralogy of Fallot, anomalous pulmonary venous
drainage, complete or corrected transposition of great
arteries, pulmonary atresia with intact ventricular septum, and Ebstein
anomaly. Double-chambered right ventricle has also been reported in
patients with Down syndrome and Noonan syndrome, although
differentiation from hypertrophic cardiomyopathy in the latter group is
not straightforward

5. External link
• http://www.youtube.com/watch?v=eoJPM0upjD0&list=LLb0aeWchl0
zwXdPA88uT5Sg&feature=share