Mortality Patterns in Pediatric Pulmonary Vein Stenosis: Insights Into Right Ventricular Systolic Pressure Associations

BACKGROUND: Pulmonary vein stenosis in children is associated with a poor prognosis. However, the cause and risk factors for mortality remain uncertain. METHODS: This retrospective, single-center study identified children with primary and secondary pulmonary vein stenosis through a cardiac catheterization database. Kaplan-Meier analysis, log-rank tests, and Cox regression analysis were performed to assess outcome and identify significant predictors of mortality. CONCLUSIONS: This report is the first to indicate that RV systolic pressure and RV dysfunction are significant independent predictors of mortality in children with pulmonary vein stenosis. A greater understanding of mortality in this population is necessary, particularly in those with RV systolic pressure less than half systemic.