Restrictive interatrial communication in total anomalous pulmonary venous connection

Restriction to systemic blood flow at the atrial level in total anomalous pulmonary venous connection (TAPVC) may play a role in the early development of congestive heart failure in some patients. Over a 4-year period, 21 patients with TAPVC without extracardiac obstruction presented from 1 day to 10 months of age (mean 2.6 months). Of 21 patients, 17 (81%) presented after 1 month of age with moderate to severe congestive heart failure. In the first week after birth 4 patients presented with minimal symptoms of congestive heart failure. A small patent foramen ovale (3 mm or less in diameter) was found in 19 of 21 patients (90%) by 2-dimensional echocardiography or angiography. Balloon or blade and balloon atrial septostomy was performed in these 19 patients and resulted in significant decreases in mean right to left atrial pressure gradient (from 2.8 to 0.25 mm Hg, p less than 0.001), systolic pulmonary to femoral artery pressure ratio (from 0.80 to 0.60, p less than 0.001), and systemic arterial oxygen saturation (from 84% to 79%, p less than 0.01). One patient had nonfatal complications. Surgery was performed in 19 patients from 2 weeks to 29 months (mean 12 months) after catheterization, with 1 operative death (5% mortality). Four patients required early total correction because of persistent heart failure; 15 had elective surgical repair. One of 2 unoperated patients died of pneumonia at 2.5 years of age. A restrictive interatrial communication develops after the first month of extrauterine life in most patients with TAPVC. Atrial septostomy results in improved hemodynamic conditions and clinical palliation.(ABSTRACT TRUNCATED AT 250 WORDS)