RESUMO
Long-segment pulmonary atresia (PA), non-confluent branch pulmonary arteries, ventricular septal defect, tricuspid valve atresia (type 1A), and single ventricle physiology is a relatively rare and extremely heterogeneous form of congenital heart disease. This subset of patients having pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (MAPCAs) have to undergo multiple unifocalization staging operations before a complete repair is attempted. Most of the patients were deemed inoperable. We report a rare case of a concomitant single-stage unifocalization and cavopulmonary anastomosis (bi-directional Glenn procedure) in an adolescent cyanotic girl with tricuspid valve atresia (type 1 A), long-segment pulmonary atresia, non-confluent branch pulmonary arteries, bilateral patent ductus arteriosus, MAPCAs, and single-ventricle physiology. Reconstruction of the absent central pulmonary artery and non-confluent branch pulmonary arteries was achieved by dividing the bilateral patent ductus arteriosus feeding the bilateral pulmonary arteries.
