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Transposition of the great arteries with total anomalous pulmonary venous connection is a rare anomaly with varied management approach depending on the time of presentation; the management of cases which present early is usually by anatomical surgical approach while late presentation is by physiologic approach. This is due to early left ventricular mass regression as a result of the absence of volume and pressure load to the left ventricle (LV). We report a late presentation (at 1½ years of age) that had a successful anatomic surgical correction because the LV was "prepared" by both pressure and volume load from pulmonary arterial hypertension and large ostium secundum atrial septal defect.

Transposition of the great arteries with total anomalous pulmonary venous connection in a 1½year-old child