Total anomalous pulmonary venous connection with dysmorphic pulmonary vein: a risk for postoperative pulmonary venous obstruction.

Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection remains potential and understanding of its mechanisms is warranted. Morphology of the pulmonary vein was qualitatively analyzed in 48 consecutive patients undergoing repair of non-isomeric total anomalous pulmonary venous connection. Pulmonary venous drainage was supracardiac in 26, cardiac in 7, infracardiac in 13, and mixed in 2. Nine had dysmorphic pulmonary venous confluence or tributary veins (Group A). Four had excessive (>or=5) tributary veins with a hypoplastic confluence (Type 1 abnormality). In the other four cases, the vertical vein was atretic (Type 2 abnormality). In a case with cardiac type, pulmonary veins had stenosis at orifices (Type 3 abnormality). The rest (n=39; Group B) had normal pulmonary vein. Eight patients (7 in Group A and 1 in Group B) developed postoperative pulmonary venous obstruction. Overall actuarial survival was 90.0% after 2.3 months up to 10 years. Actuarial freedom from pulmonary venous obstruction was 79.5% after 5.0 months up to 10 years. It was 22.2% at 1 year among Group A compared with 96.7% at 10 years among Group B (P<0.001). Morphological analysis of the pulmonary vein best predicted the incidence of postoperative pulmonary venous obstruction.