Key result
CPET parameters do not correlate with quality-of-life scores in pediatric pulmonary arterial hypertension.
Why the study?
Idiopathic and heritable PAH impair functional capacity and HRQoL, yet the relationship between CPET parameters and HRQoL had not been investigated in pediatric PAH.
Observational (n=19)
Yes
Children with idiopathic or heritable PAH exhibit reduced exercise capacity and impaired quality of life, though objective CPET parameters did not significantly correlate with patient-reported quality of life scores in this small cohort.
Answers are grounded in this paper and cite their sources.
No CPET-HRQoL correlation in small pediatric IPAH cohort; leaves open whether exercise testing informs quality-of-life assessment.
BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive disease causing elevated pulmonary vascular resistance, right ventricular (RV) failure, and premature death. Idiopathic and heritable PAH impair functional capacity and health-related quality of life (HRQoL). Cardiopulmonary exercise testing (CPET) assesses exercise limitation and disease severity, yet its relationship with HRQoL has not been investigated in pediatric PAH. RESEARCH DESIGN AND METHODS: A retrospective multicenter study across three French tertiary pediatric cardiology centers was conducted (June 2023-April 2025). Children with idiopathic or heritable PAH who underwent CPET and completed the Pediatric Quality of Life Inventory (PedsQL 4.0) were included. Key CPET parameters were analyzed, and associations to HRQoL scores assessed via Spearman correlation. RESULTS: Among 19 children (median age 11 years, median follow-up 32 months), exercise capacity was reduced (peak VO2 29 ml/kg/min; 74.5% predicted; Z-score -2.9), with ventilatory inefficiency (VE/VCO₂ slope 40). QoL scores were lower than normative values. No significant correlation was found between CPET parameters and HRQoL scores. CONCLUSION: Children with idiopathic or heritable PAH present exercise limitation and impaired HRQoL, though CPET parameters were not correlated with HRQoL scores, reflecting limited statistical power. Larger studies are needed to clarify this relationship.
No takes yet. Share an insight, caveat, or question.
DUBOIS et al. (2026) conducted an observational in Idiopathic or heritable pulmonary arterial hypertension (n=19). Cardiopulmonary exercise testing (CPET) parameters vs. Normative values was evaluated on Correlation between CPET parameters and health-related quality of life (HRQoL) scores. CPET parameters showed reduced capacity in children with PAH but did not correlate with HRQoL scores.
Synapse has enriched 10 related papers. Consider them for comparative context: