Homograft Durability after Correction of Pulmonary Atresia and Ventricular Septal Defect with or without Systemic Pulmonary Collateral Arteries

Abstract Background Pulmonary atresia and ventricular septal defect (PA-VSD), with or without systemic pulmonary collateral arteries (SPCA), represent a complex anatomical and surgical spectrum of congenital heart disease. Currently, there is limited evidence about homograft durability after complete correction which could potentially be affected by anatomical differences in pulmonary vasculature. Material and Methods A retrospective single center study was performed including all 69 consecutive PA-VSD patients (46 with SPCA, 23 without SPCA) operated between 1978 and 2018. Primary interest was homograft durability after complete repair. Longitudinal echocardiographic homograft function and right ventricular systolic pressure (RVSP) were analyzed with linear mixed effects models. Results The median follow-up time was 20 years. Of 46 patients with SPCA, 37 (80.4%) underwent biventricular correction at a median age of 2.7 years (inter quartile range 1.8-6.3 years). Two patients are currently awaiting unifocalization and correction. All 23 patients without SPCA underwent successful complete correction at a median age of 1.6 years (inter quartile range 1.1-3.6 years). Freedom from any reintervention after 20 years was 15%. In case a homograft was used during correction, freedom from homograft replacement after 20 years was comparable (p=0.925) with 32±11% in the SPCA group, and 32±13% for patients without SPCA. Indications for homograft replacement were isolated stenosis (n=7; 46.7%), isolated regurgitation (n=3; 20.0%) and mixed stenosis and regurgitation (n=5; 33.3%) in the SPCA group. In patients without SPCA, isolated stenosis was the indication in 8 (88.9%), and 1 (11.1%) patient had both stenosis and regurgitation. Peak homograft gradient was significantly (p=.0003) higher in patients without SPCA, with a comparable rate of progression between the groups. The prevalence of severe pulmonary regurgitation was higher in patients with SPCA, however, estimated at 35% at 10 years versus 15% in patients without SPCA. Conclusion Homografts used for right ventricular outflow tract reconstruction in patients with PA-VSD, both with or without SPCA, have similarly limited durability. Repeated reintervention is common, and careful follow up with attention to severe pulmonary regurgitation is warranted.