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

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.

Keywords: cardiac catheterization; pulmonary vein stenosis; right ventricular systolic pressure.

Figure 1. Institutional approach for treating patients with pulmonary vein stenosis.

This flowchart outlines our current institutional strategy for managing patients with PVS, including criteria for intervention, anatomic assessment, and stepwise therapeutic approaches. *Clinical changes may lead to adjustments in the frequency and type of intervention, determined by the care team based on individual patient response. BPD indicates bronchopulmonary dysplasia; CT, computed tomography; PVS, pulmonary vein stenosis; and RV, right ventricular.

Figure 2. Kaplan‐Meier survival analysis with log‐rank test.

The survival analysis starts at the date of definitive pulmonary vein stenosis diagnosis, with censoring at the last clinic follow‐up or death. A, Overall survival. B, Subgroup analysis with right ventricular systolic pressure (RVSP) at last catheterization. C, Subgroup analysis with right ventricular (RV) systolic function (nondiminished; normal or mildly diminished; or moderately or severely diminished function). D, Subgroup analysis with RVSP at last catheterization among patients with nondiminished RV systolic function.