Prognostic value of longitudinal vasoreactivity in pediatric pulmonary hypertension

Patrick D. Evers, Patrick Quinn, Paul J. Critser, Benjamin S. Frank, Laurie B. Armsby
Oregon Health Sciences University. University of Cininnati. University of Colorado.
United States

Pulmonary Circulation
Pulm Circ 2022; 12: e12152
DOI: 10.1002/pul2.12152

Abstract
Upon diagnosis of pulmonary hypertension in pediatrics, standard practice often involves acute vasoreactivity testing (AVT) in the cardiac catheterization laboratory. However, the importance of repeated AVT testing in a given patient thereafter remains unclear. This study sought to describe serial AVT results in pediatric patients and understand the prognostic significance of longitudinal AVT results in pediatric pulmonary hypertension. A retrospective chart review was performed for pediatric pulmonary hypertension patients diagnosed between 2008 and 2021. Patients were included if they had two or more catheterizations with AVT. The study cohorts were patients who were AVT negative upon initial catheterization then AVT positive at any subsequent catheterization (AVT-/+) compared to those were AVT negative upon initial and all subsequent catheterizations (AVT-/-). A positive AVT was defined by Sitbon criteria. The analyzed outcome was event-free survival. The relationship between study cohorts and event-free survival was analyzed by log-rank Kaplan-Meier survival as well as Cox proportional hazard regression to control for confounders. There were 35 patients who met inclusion criteria in this time period. Patients who were AVT(-/+) had statistically significantly better event-free survival than AVT(-/-) (p = 0.002). In univariate and multivariate Cox regressions, a subsequent AVT positive result amongst those who were initially AVT negative was a positive prognostic factor, hazard ratio 0.03 (95% confidence interval: 0.02-0.35). For patients with negative AVT upon initial cardiac catheterization, this data supports that continuing AVT should be performed as any subsequent AVT positive result may indicate improved expectations for event-free survival.

Category
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing

Age Focus: Pediatric Pulmonary Vascular Disease

Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes

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