Approach Considerations
Symptoms of double-chambered right ventricle (DCRV) that require therapy are generally an indication for operative repair.
In the presence of a ventricular septal defect (VSD), a significant left to right shunt can be present, requiring antifailure treatment, particularly if the muscle bundles are not sufficiently obstructive to reduce pulmonary blood flow.
Surgical Care
The first successful surgical repair was reported in 1962. The initial approach was through a ventriculotomy; contemporary series describe both transatrial and transventricular approaches. [10, 11]
Time to intervene naturally depends on the associated lesions; the current practice is to address associated lesions (ventricular septal defect [VSD], subaortic stenosis, pulmonary stenosis) at the time of double-chambered right ventricle repair.
In the absence of a significant associated lesion, observation may be appropriate as long as the intracavitary gradient is not greater than 40 mm Hg and the degree of obstruction is not progressive.
Although attempted, balloon dilatation likely has no role in the management of double-chambered right ventricle. Tsuchikane et al reported a patient who underwent a percutaneous myocardial ablation with an alcohol-induced conus branch occlusion for relief of a significant pressure gradient in double-chambered right ventricle. [12]
Activity
Before repair, according to the degree of right ventricular outflow tract obstruction and associated lesions, exercise tolerance may be impaired and cyanosis may be present. After surgical repair and without significant residual anatomic lesions, activity tolerance should be normal.
Guidelines for physical activity and recreational sports participation in children with genetic cardiovascular diseases have been established [13] ; efforts are ongoing to refine guideline and establish evidence-based recommendations. [14]
Long-Term Monitoring
Prior to surgical therapy for double-chambered right ventricle (DCRV), the follow-up is based on the degree of obstruction, associated lesions, and symptoms. Regular evaluation by a cardiologist is recommended.
The residual lesions determine the follow-up of these patients after surgery. The patients are initially frequently evaluated in order to monitor the progress after repair.
Exercise tolerance and quality of life, endocarditis prophylaxis, and recurrence of obstruction comprise the major issues during the long-term follow-up.
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Double-Chambered Right Ventricle. Electrocardiogram of an 18-month-old boy with double-chambered right ventricle. Note the upright T waves in the right precordial leads.
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Double-Chambered Right Ventricle. Right anterior oblique (RAO) angiogram demonstrating proximal and distal chambers of right ventricle (Image courtesy of R.M. Freedom, MD).
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Double-Chambered Right Ventricle. Lateral right ventriculography of a patient with double-chambered right ventricle. Large arrow indicates the presence of a fibromuscular obstruction with division of the right ventricle; small arrows outline pulmonary valve stenosis (Image courtesy of R.M. Freedom, MD).
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Double-Chambered Right Ventricle. Subcostal right anterior oblique (RAO) echocardiograph view demonstrating right ventricle muscle bundles separating proximal from distal (*) chamber. PV = Pulmonary valve (Image courtesy of J. Smallhorn, MD)
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Double-Chambered Right Ventricle. Subcostal right anterior oblique (RAO) echocardiograph view with color Doppler demonstrating ventricular septal defect jet to proximal chamber. (*) = Distal chamber (Image courtesy of J. Smallhorn, MD).
