B-18 | Elevated Right Ventricular Systolic Pressure is an Independent Predictor of Mortality in Pediatric Pulmonary Vein Stenosis

Pulmonary vein stenosis (PVS) in children is associated with a poor prognosis. However, the risk factors for mortality remain uncertain. This retrospective, single-center study identified children with primary and secondary PVS 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. Among 56 children with PVS (33 males, 59%), 20 (36%) died at a median age of 10 months (IQR: 4-24). All patients underwent cardiac catheterization, with 45 (80%) undergoing at least one 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 systolic pressure (RVSP) between half and full systemic, and RVSP greater than systemic pressure was associated with mortality (HR 4.8, 95% CI 1.7-13.0, p=0.002, and HR 9.2, 95% CI 2.6-32.8, p<0.001, respectively). The final predictive model for mortality included only RVSP between half and full systemic (HR 4.6, p=0.004) and diminished right ventricular (RV) systolic function (HR 5.5, p=0.030). This report is the first to indicate that RVSP and RV dysfunction are significant independent predictors of mortality in children with PVS. A greater understanding of mortality in this population is necessary, particularly in those with RVSP less than half systemic.