Changes of management in a patient with double outlet left ventricle

Double outlet left ventricle is an extremely rare anomaly. Until recently, the diagnosis was usually established by angiography or at postmortem. There 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 patient reported here underwent surgery at the age of four years, when an aortic homograft was placed between the right ventricle and the pulmonary trunk. This biventricular repair had to be changed into a Fontan-type procedure, 15 years later since the hypoplastic right ventricle did not grow adequately.