ABSTRACT
Nine-year-old female patient presented with cianosis since she was born, fatique and dyspnea when sucking. The diagnosis was univentricular heart with left ventricular morphology, transposition of the great arteries, moderate pulmonary valve stenosis and atrial septal defect. Submitted to surgical correction with superior vena cava-right pulmonary artery anastomosis, inferior vena cava anastomosis using lateral tunnel, with cardiopulmonary bypass. After surgical correction, the clinical and laboratorial (echocardiogram and cardiac catheterization) evaluation showed Fontan operation with good surgical results. Total cavopulmonary connection was proposed as a modification of the Fontan procedure that might have greater benefits than previous proposed techniques. The results demonstrate that this modification provides excellent early definitive treatment, increasing hemodynamic profile, with low morbidity and mortality, for a variety of complex congenital heart lesions.