Cardiopulmonary exercise testing and health-related quality of life in children with idiopathic or heritable pulmonary arterial hypertension

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. 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. 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. 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.