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Reconstruction of superior vena cava-right atrial continuity and "anatomic" repair of transposition of great arteries with ventricular septal defect.

A superior vena cava-to-right pulmonary artery anastomosis (Glenn procedure), used to palliate certain types of cyanotic congenital heart defects, has usually been left intact at the time of definitive intracardiac repair. Late failure of this shunt has prompted attempts to reestablish superior vena cava-to-right atrium and right ventricle-to-right pulmonary artery continuity. The present patient, a 10-year-old boy with transposition of the great arteries, ventricular septal defect, and mild pulmonic stenosis, had had a Glenn procedure performed at 3 years of age. Angiography demonstrated a patent superior vena cava-to-right pulmonary anastomosis. The appearance of the main left pulmonary artery and of its peripheral branches suggested the presence of pulmonary vascular obstructive disease in the left lung. Therefore, it was considered advisable to reconnect the right ventricle to the right pulmonary artery, which was "protected" by the long-standing low-pressure shunt. The operative result, the intraoperative hemodynamics, and the postoperative assessment of the pulmonary blood flow by means of lung scanning, which clearly demonstrated a preferential perfusion of the right lung, support the validity of this approach.