Ascending Aortic Extension for Enlargement of the Aortopulmonary Space in Children with Pulmonary Artery Stenosis

Background Complex reconstruction of the aorta can be complicated by compression within the aortopulmonary space resulting in airway or pulmonary artery narrowing. Pulmonary artery compression is especially problematic in children with single ventricle physiology in which an increase in pulmonary vascular resistance may impair systemic venous flow and reduce cardiac output. Methods We operated on 7 patients (mean age, 2.9 years) with pulmonary artery stenosis presenting after a complex neonatal aortic reconstruction. All 7 patients underwent aortic extension with a polytetrafluoroethylene interposition graft and homograft patch angioplasty of the pulmonary artery to open the aortopulmonary space and relieve pulmonary artery narrowing. Five patients (hypoplastic left heart syndrome, n = 2; transposition of the great arteries with tricuspid atresia and aortic hypoplasia, n = 1; double outlet right ventricle with aortic hypoplasia, n = 2) had previously undergone first stage repairs for single ventricle morphology. Two of the patients had multiple interim procedures, including placement of bilateral pulmonary artery stents, prior to our repair. Results There was 1 early death secondary to fungal sepsis. Six patients were discharged from the hospital. There was 1 late, noncardiac death from aspiration pneumonia in a patient with a severe craniofacial defect. Follow-up echocardiograms in the intermediate term have demonstrated relief of pulmonary artery narrowing and unobstructed aortic flow. Conclusions Aortic extension is an option in children with pulmonary artery compression of structures in the aortopulmonary space after complex aortic reconstruction