Abstract

To delineate factors that contribute to improved surgical outcome in patients with total anomalous pulmonary venous drainage, we reviewed the records of 52 consecutive patients. Venous drainage was supracardiac in 25 (48%), cardiac in 12 (23%), infracardiac in 10 (19%), and mixed in five (10%). Preoperative pulmonary venous obstruction was present in 18 patients (35%). Median age at the time of repair was 35 days and weight, 3.7 kg. Repair was performed with deep hypothermia, low-flow cardiopulmonary bypass, and occasional short periods of circulatory arrest. In patients with coronary sinus drainage, the veins were tunneled to the left atrium through an enlarged atrial septal defect, with a mortality of 8% (1/12) and no postoperative stenosis. The approach in patients with supracardiac, infracardiac, and mixed drainage varied with time. In 16 patients, the condition was managed by apical or right-sided exposure of the common vein, anastomosis of the common vein to the left atrium with continuous sutures, and primary closure of the atrial septal defect (type I repair). In the other 24 patients the common vein was approached from the right side through the right atrium and the interatrial septum. Common vein-left atrium anastomosis was performed with interrupted sutures and a piece of pericardium used to augment the anastomosis, prevent common vein distortion, and close the atrial septal defect (type II repair). Mortality in type I repair was 25% (4/16) and in type II repair, 4% (1/24). Follow-up was 7.86 +/- 4.0 years with no late deaths. Postoperative stenosis occurred in five of 14 (36%) patients who had type I repair versus two of 23 (9%) who had type II repair. Multivariate analysis showed that type I repair was a positive risk factor for hospital mortality (p = 0.05) and restenosis (p = 0.04). The technique of transatrial exposure of the common venous chamber, interrupted suturing of the common vein to the left atrium, and pericardial patch augmentation significantly improves survival and decreases risk of restenosis.

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