Abstract 14502: Contemporary Practices and Outcomes in Primary Pulmonary Vein Stenosis: A PVS Network Study

Abstract

Background and Study Aim: Pediatric pulmonary vein stenosis (PVS) remains a disease associated with high mortality, with studies primarily limited to single center reports. Using the PVS Network Registry, we report on contemporary practices and outcomes for a large cohort of children with primary PVS across North America. Methods: Children with a diagnosis of primary PVS (congenital, normal pulmonary venous connections) in the retrospective PVS Network Registry (2009-2019, n=600, 19 institutions) were analyzed using descriptive statistics, survival analysis and Cox regression analysis. Results: In the PVS Network Registry cohort, 393 patients had a diagnosis of primary PVS. In the primary PVS cohort, 199 (50%) were male, 105 (27%) had a genetic diagnosis and 192 (49%) were premature (median 29 (22-37) weeks). Medical therapies were used in 53(14%) patients, with Imatinib being most common (58%). Pulmonary hypertension medications were used in 182 (46%) patients, with sildenafil most reported (90%). Surgical and catheter-based interventions for PVS were performed in 334 (85%) patients. 203 (60%) patients had at least one PVS surgery and 231(71%) had at least one PVS catheter intervention. At the time of first procedure for PVS, 133 (39%) had bilateral disease, while 100 (30%) had ≥3 pulmonary veins involved. The median age was 0.54 years (IQR 0.91). Catheter based interventions were more common on the left PVs (60%). Repeat procedures were common (53%) with the majority being catheter reinterventions (78%), with an average of 3.24 (±2.75) catheter procedures per patient. The overall survival for children with primary PVS was 76% and 71% at 1 and 2 years. In children with ≥4 veins involved, survival was 59% and 52% at 1 and 2 years. Older age at intervention was protective (HR 0.66, P<0.02), while ≥3 vein involvement was associated with poor outcome (HR 1.8, p=0.05). Conclusion: This is the largest primary PVS cohort reported and demonstrates overall 1 year survival of 76%, however, children with multivessel disease remain at high risk. Reinterventions are common, with many undergoing repeat catheterization procedures in our cohort. The PVS Network retrospective cohort provides insights into clinical practices and will provide benchmarking for future studies.