The combination of discordant atrioventricular and concordant ventriculoarterial connections is an extremely rare entity, with only 8 cases of successful surgical repair being previously reported. Although from the clinical standpoint this anomaly is similar to complete transposition, this unusual combination is clearly suited by its morpho-physiological features to an atrial rather than an arterial switch procedure. A 17-year-old boy with this malformation, associated also with severe pulmonary hypertension, underwent a palliative Senning procedure, leaving the ventricular septal defect open and banding the pulmonary trunk. Postoperatively, hypoxemia was markedly improved, and was associated with a reduction of the level of pulmonary hypertension, contributing to marked symptomatic improvement.
