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Abstract
Double-chambered right ventricle is a form of right ventricular outflow tract obstruction
that develops over time, often in patients with an abnormally short distance between
the moderator band and pulmonary valve. This lesion typically presents in childhood
or adolescence and is often accompanied by a ventricular septal defect. Only a handful
of previous cases have been described in which double-chambered right ventricle occurred
in adulthood.
Since 1992, three patients more than 30 years old (38, 43, and 66 years of age) have
presented at our institution with unusual symptoms or a previous incorrect diagnosis.
We reviewed the clinical data in these patients.
Presenting symptoms included syncope, angina, and severe dyspnea resembling pulmonary
hypertension. In 1 patient, disease was categorized as New York Heart Association
class IV, and in the other 2 as class III. Coexisting anomalies included a patent
foramen ovale or secundum atrial septal defect in 2 patients, a small ventricular
septal defect in 1 (with a probable history of ventricular septal defect in another),
and mild aortic regurgitation in 1. All patients required urgent or emergent operations,
with peak pressures in the proximal right ventricular chamber of 135 to 180 mm Hg
and severely depressed left ventricular function in 1 patient. Resection of the anomalous
right ventricular muscle bundles was achieved through a right atrial approach in all
patients. All patients were alive with improved functional status at follow-up, which
was between 15 and 40 months.
Right ventricular outflow tract obstruction resulting from a double-chambered right
ventricle is rare in adults, but when it does occur it can present with unusual symptoms.
When evaluating the patient with signs or symptoms of primary right heart failure,
cardiologists should make an effort to image the entire right heart complex. Subcostal
echocardiography can facilitate adequate visualization of the right ventricle when
it is difficult to distinguish the subpulmonary outflow tract from the parasternal
and apical windows.