Abstract

Background: Pediatric pulmonary vein stenosis (PVS) is a progressive and recurrent condition associated with high morbidity and mortality. Long-term outcomes of individual pulmonary veins remain poorly described. Methods: All pediatric (<18 years) PVS cases diagnosed between September 2005 and January 2020 with a minimum follow-up of 5 years were included. Vein-specific outcomes were assessed using competing-risk survival analysis adjusted for patient death and vein atresia. Veins with recurrent disease maintaining patency for ≥3 years were analyzed longitudinally to characterize reintervention burden as a surrogate for disease trajectory. Results: A total of 244 affected veins in 107 patients were identified. The cumulative incidence of vein loss progressively increased during the first 3 years, followed by plateauing thereafter. Overall, 147 of 244 veins (60%) were lost within 3 years of diagnosis, including 75 veins lost secondary to patient death. Vein attrition was greatest during the first year following diagnosis. Ninety-seven veins remained patent for ≥3 years, including 73 with recurrent disease. Among these veins, the median reintervention rate decreased significantly from 2 (IQR: 1–4) during the first 3 years to 1 per year (IQR: 1–3) thereafter (p < 0.01) while maintaining continued patency. Conclusions: In pediatric PVS, the first 3 years after diagnosis represent a critical period of active disease with high rates of vein attrition. Veins maintaining patency beyond this period demonstrate a reduced reintervention rate, suggesting stabilization of disease progression. Aggressive surveillance and intervention during this therapeutic window may improve long-term vein and patient outcomes.

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