Experience with operation for total anomalous pulmonary venous connection in infancy

Twenty patients presenting with total anomalous pulmonary venous connection (TAPVC) in infancy underwent cardiac repair. Four had associated major intracardiac anomalies (complex TAPVC) and 16 had isolated TAPVC. All patients with complex lesions died during or shortly after the operation; they all had pulmonary venous obstruction (PVO). The associated malformations were critical pulmonary stenosis (one case), multiple ventricular septal defects (VSDs) (one case), mitral atresia (one case), and asplenia syndrome with common atrioventricular valve, double-outlet right ventricle, and pulmonary stenosis (one case). Among the patients with isolated lesions there were two surgical deaths, both in infants less than 1 month of age, with severe PVO and subdiaphragmatic drainage. Statistical analysis of these data shows a strongly incremental risk of surgical death due to the presence of associated malformations. Analysis of just isolated TAPVC shows a possible incremental effect due to neonatal age (less than 1 month) and PVO, these two factors being not clearly separated. There have been no late deaths and no late complications among the 14 survivors followed from 7 months to 10 years (mean 44 months). An aggressive surgical approach regardless of the age, degree of pulmonary hypertension, and type of anatomic connection is advised for isolated TAPVC. Some caution is recommended for complex TAPVC, in which a much higher risk is anticipated, particularly when a palliative pulmonary vein-to-left atrium anastomosis is performed.