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. 2022 Oct 1;12(4):e12152.
doi: 10.1002/pul2.12152. eCollection 2022 Oct.

Prognostic value of longitudinal vasoreactivity in pediatric pulmonary hypertension

Affiliations

Prognostic value of longitudinal vasoreactivity in pediatric pulmonary hypertension

Patrick D Evers et al. Pulm Circ. .

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.

Keywords: hemodynamics; hypertension; pediatrics; pulmonary.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Schematic representation of the longitudinal acute vasoreactivity testing (AVT) results for individual subjects in the years following diagnosis separated into study cohorts
Figure 2
Figure 2
Box and whiskers plot for baseline hemodynamics up to 1 year following discharge, 5 years following discharge and 10 years following discharge. Groups compared are those who at any catheterization subsequent to the first were AVT positive (AVT−/+) compared to those who never illustrated AVT positivity (AVT−/−)
Figure 3
Figure 3
Kaplan–Meier of freedom from composite adverse outcomes based on acute vasoreactivity testing (AVT) results at initial diagnostic catheterization. Log‐rank = 0.149
Figure 4
Figure 4
Kaplan–Meier of freedom from composite adverse outcomes. Groups compared are those who were acute vasoreactivity testing (AVT) positive at the initial catheterization, those who were AVT negative at the initial catheterization but at any subsequent catheterization were AVT positive, and compared to those who never illustrated AVT positivity, AVT(+), AVT(+/−), and AVT(−/−), respectively. Log‐rank = 0.002

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