Abstract
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. RESULTS: Among 56 children with pulmonary vein stenosis (33 male children, 59%), 20 (36%) died at a median age of 10 months (interquartile range, 4-24 months). All patients underwent cardiac catheterization, with 45 (80%) undergoing at least 1 interventional procedure. Causes of death included multiorgan failure (35%), progressive respiratory failure (20%), and sudden cardiac death (15%). Prematurity, chronic lung disease, a genetic syndrome, or the number of affected pulmonary veins did not significantly correlate with mortality. However, right ventricular (RV) systolic pressure greater than half systemic pressure was associated with mortality (hazard ratio [HR], 5.5 [95% CI, 2.2-14.1]; P<0.001). The final predictive model for mortality included RV systolic pressure greater than half systemic pressure (HR, 4.0 [95% CI, 1.6-10.4]; P=0.004), moderately or severely diminished RV systolic function (HR, 3.6 [95% CI, 1.1-11.5]; P=0.032), and the presence of congenital heart disease (HR, 2.4 [95% CI, 0.9-6.7]; P=0.084). 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.