Pulmonary Vascular Compromise is Associated with Survival in Pediatric Pulmonary Hypertension: A New Computational Model

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Abstract

Background

Pediatric pulmonary arterial hypertension (PAH) has a long asymptomatic period with progressive vascular loss. A recent computational model of simulated PAH in humans has demonstrated that up to 70% of the pulmonary vasculature is lost before clinical PAH criteria are met. We sought to evaluate this model in pediatric subjects with PAH and evaluate whether estimated pulmonary vascular compromise (PVC) can predict survival and other clinical outcomes.

Methods and Results

Retrospective and prospective cohort data were collected for all subjects with PAH between 1999 and 2022 treated at our center. Cardiac catheterization and clinical data were compared with PVC estimated by the computational model. Transplant-free survival was associated with lower PVC (72% vs 88%, p<0.001). Freedom from transplant/death was also associated with a decrease in PVC over time with no significant change in PVC in subjects who died or underwent transplant. By Kaplan-Meier analysis, 10-year survival was 54% (IQR 35%, 81%) when PVC was more than 80%, compared with 100% survival (IQR 100%, 100%) when PVC was less than 80% (p<0.001). By Cox proportional hazard regression, PVC was associated with mortality (HR 1.1, p=0.008). Lower PVC was associated with better percent predicted 6-minute walk distance (-0.25, 95% CI [-0.35, -0.14], p<0.001), lower log brain natriuretic peptide (0.12, 95% CI [0.07, 0.18], p<0.001), and lower estimated 1-year mortality (0.01, 95% CI [0.01, 0.02], p<0.001).

Conclusions

Estimated PVC predicts transplant-free survival and other clinical outcomes in pediatric PAH and provides an adjunctive tool to potentially capture pulmonary vascular loss early in disease.

Clinical Perspective

What Is New?

  • A new computational model can estimate pulmonary vascular area loss or pulmonary vascular compromise (PVC).

  • PVC can detect early vascular loss and is associated with transplant-free survival and clinical outcomes in pediatric pulmonary arterial hypertension.

What Are the Clinical Implications?

  • PVC may detect pulmonary vascular disease early in the disease process and could be used as an adjunct tool for management of patients with pulmonary arterial hypertension.

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