Outcomes in 45 children with ductal origin of the distal pulmonary artery
Kalyani R Trivedi1, Tara Karamlou, Shi-Joon Yoo
1Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Ductal origin of the distal pulmonary artery (PA) in children is linked to significant health issues. Early diagnosis and surgical repair can improve survival rates and reduce complications.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Thoracic Surgery
Background:
- Limited large-scale data exists on the morphologic spectrum and clinical outcomes of children with ductal origin of the distal pulmonary artery (PA).
- This condition presents unique diagnostic and therapeutic challenges in pediatric cardiovascular care.
Purpose of the Study:
- To describe the morphologic spectrum and clinical outcomes of children with ductal origin of the distal PA.
- To evaluate the impact of surgical interventions on patient survival and associated morbidities.
Main Methods:
- Retrospective review of medical records for 45 children diagnosed between 1970 and 2001.
- Analysis of angiograms at presentation and follow-up, detailing associated cardiac anomalies and surgical procedures.
Main Results:
- Ductal origin of the distal PA was found isolated in 36% and associated with other defects (Tetralogy of Fallot, PA-VSD, heterotaxy) in 64% of cases.
- Surgical interventions included shunts, grafts, banding, unifocalization, and heart transplantation, with an overall 20-year survival of 70%.
- Reconstruction of the ductal PA reduced pulmonary hypoplasia and scoliosis; survival was better in patients without congestive heart failure at presentation.
Conclusions:
- Ductal origin of the distal PA is associated with significant multisystem morbidity and mortality in children.
- Early diagnosis and timely surgical repair, particularly for those with pulmonary overcirculation, are crucial for improving outcomes.
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.
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