Surgical repair of total anomalous pulmonary venous connection.

The diagnosis of total anomalous pulmonary venous connection (TAPVC) is made when all four pulmonary veins drain anomalously to the right atrium or to a tributary of the systemic veins. It constitutes between 1% and 1.5% of all children with congenital heart disease and can be categorized by the site of drainage into the systemic circulation (supracardiac, 45%; infracardiac, 25%; cardiac, 25%; mixed, 5%). The clinical presentation is different if the pulmonary venous drainage is unobstructed (heart failure, mild cyanosis) or obstructed (respiratory failure, severe heart failure). Surgical management depends on the anatomic type. Obstructed TAPVC requires urgent surgical intervention, whereas unobstructed TAPVC can be dealt with electively; although this is usually operated on once the diagnosis is made. Postoperative pulmonary artery hypertension can be problematic. Recent surgical results with isolated TAPVC have improved, with operative mortality consistently at less than 10%. A particularly challenging group of patients are those with single ventricle physiology and TAPVC with high operative mortality and poor long-term survival.