Double-outlet right ventricle with tunnel from left ventricle to aorta

The successful surgical correction of an unusual form of double-outlet right ventricle (DORV) in a 4-year-old boy is described. A long tunnel-like structure was present between the left ventricle and the aorta and also communicated with the right ventricle. hemodynamic and angiographic evaluation demonstrated restrictive communications between the tunnel, the aorta, and each of the ventricles. A pathological concept explaining the anatomic abnormality seen in this patient is discussed.