Double outlet left ventricle is an extremely rare anomaly. Until recently,
the diagnosis was usually established by angiography or at postmortem. Ther
e are only a few reports describing the echocardiographic findings in this
lesion, and as far as we know, no report showing the anatomy as well as the
velocity and pattern of flow by color Doppler echocardiography. The patien
t reported here underwent surgery at the age of four years, when an aortic
homograft was placed between the right ventricle and the pulmonary trunk. T
his biventricular repair had to be changed into a Fontan-type procedure, 15
years later since the hypoplastic right ventricle did not grow adequately.