Intra-atrial repair for total anomalous pulmonary venous connection

The natural history of total pulmonary venous connection (TAPVC) is unfavorable per se. We describe a modified technique of intra-atrial repair in infants with supra- and infracardiac TAPVC. Twenty patients were treated. The median age at repair was 16 (range 3-62) days. Echocardiography and multi-detector row computed tomography were used to confirm the diagnosis. Our policy was to perform surgery on an urgent basis whenever there was a sign of severe pulmonary congestion or hypoxia. The procedures performed in cardiopulmonary bypass (CPB) were established in a standard fashion using bi-caval cannulation and moderate hypothermia. A novel modification of our surgical technique is the H-shaped instead of simple straight incision of pulmonary venous confluence and intra-atrial sewing. H-shaped incision of pulmonary venous confluence can increase the anastomotic area than simply straight-line incision and create a large anastomosis with maximal use of the venous confluence and atrial tissue. Intra-atrial repair can avoid torsion and rotation of the pulmonary veins. There were no operative deaths and no recurrent pulmonary venous obstruction was noted after a mean period of 2 ± 0.8 years (range: 12-20 months). Intra-atrial repair provides excellent results for primary repair of supra- and infracardiac TAPVC in infants.