Outcomes in 45 children with ductal origin of the distal pulmonary artery

Background. There are no large series describing the morphologic spectrum and the clinical outcomes of children with ductal origin of the distal pulmonary artery (PA). Methods. Medical records were reviewed for all children presenting between 1970 and 2001. Angiograms were reviewed at presentation and at last available follow-up. Results. Forty-five patients were identified with median presenting age of 14 days (range, birth to 6.5 years). Ductal origin of the distal PA occurred as an isolated finding in 16 patients (36%), with tetralogy of Fallot in 12 (27%), with pulmonary atresia-ventricular septal defect in 13 (29%), and with heterotaxy in 4 (9%). Diagnostic pulmonary venous wedge angiography was performed in 21 patients (47%). Surgical procedures were undertaken in 31 patients, and were initial systemic-ductal PA shunt in 13 patients, interposition graft in 6, direct anastomosis to the main PA in 2, ductal PA banding in 2, unifocalization of the ductal PA with complete or staged pulmonary atresia-ventricular septal defect repair in 7, and heart transplantation in 1 patient. Surgical revision was required in 3 patients and catheter interventions in 12 patients. Overall 20-year survival was 70% and was improved among patients without congestive heart failure at presentation (p = 0.08, hazard ratio: 2.81). Reconstruction of the ductal PA decreased the prevalence of pulmonary parenchymal hypoplasia (p < 0.001) and scoliosis at last available follow-up. Conclusions. Ductal origin of the distal PA is associated with important multisystem morbidity and mortality. Early diagnosis and repair of the ductal PA, especially in children presenting with pulmonary overcirculation, may improve outcomes.