Rationale and Design of The Congenital Cardiac Research Collaborative Pulmonary Vein Stenosis Registry

Historically, congenital/childhood-onset pulmonary vein stenosis (PVS) was associated with progressive anatomic narrowing, right ventricular failure, and mortality. Comprehensive care of PVS with intensive surveillance, serial reintervention, and medical therapy have shown potential to improve outcomes. Important knowledge gaps persist in measuring severity of PVS, optimal patient- and vein-level outcomes, and which treatment(s) and comorbid conditions influence outcomes. The Congenital Cardiac Research Collaborative Pulmonary Vein Stenosis Registry will provide a longitudinal dataset of pediatric patients with multi-vessel pulmonary vein stenosis treated at 19 US centers with ambispective data collection creating a cohort of patients beginning on 1/1/2022 with ongoing longitudinal follow-up. Data beginning at their initial evaluation and intervention (including clinical and demographic factors) and continued with longitudinal follow-up, resource utilization, reintervention(s), medical therapy, and (patient- and vein-level) outcomes will be collected. The registry will provide an efficient means of improving outcomes in this vulnerable patient population. Evaluating current outcomes, resource utilization, and practice variation in this population will guide quality improvement efforts, and the registry may serve as a structure for future research and quality improvement efforts.