A surgical technique for correction of total anomalous pulmonary venous drainage.

A technique was employed successfully for correction of total anomalous pulmonary venous drainage into the upper right superior vena cava. A J-shaped right atriotomy was performed; the posterior flap was sutured to the anterior border of a previously enlarged atrial septal defect. The right superior vena cava was divided above the site of drainage of the pulmonary veins, and its proximal opening closed with a suture. The pulmonary venous return was directed to the left atrium in this way. The right atrial-right superior vena caval continuity was then reestablished by an anastomosis between the previously opened right atrial appendage and the distal end of the right superior vena cava. Finally the remaining atriotomy was closed. The azygos vein must be ligated to avoid systemic unsaturation. For correction of anomalous pulmonary venous drainage into the azygos vein with this technique, ligature of the azygos vein must be placed distally to the site of anomalous drainage. Three patients, aged 2 months, 7 years, and 16 years, respectively, with different anatomic types of the anomaly, were successfully operated on with this procedure. Findings displayed from the postoperative hemodynamic, echocardiographic, and clinical evaluation are encouraging, after a follow-up period that ranges from 4 months to 4 years. The advantages of the repair are discussed.