Pediatric pulmonary hypertension patients had higher composite frailty scores (median 4.0 vs 2.0, p<0.001) and lower functional status (median 80 vs 100, p<0.001) compared to healthy controls.
Cross-Sectional (n=36)
Do frailty and functional status scores correlate with disease severity in pediatric pulmonary hypertension compared to healthy controls?
Frailty and functional status scores are significantly worse in pediatric pulmonary hypertension patients compared to healthy controls and correlate with established markers of disease severity.
Absolute Event Rate: 4% vs 2%
p-value: p=<0.001
Abstract Rationale Pediatric pulmonary hypertension (PH) is a progressive condition with high morbidity and mortality. While several high-risk features have been identified, risk stratification remains challenging. Evaluating frailty and functional status has proven to be a highly valuable, non-invasive tool that significantly enhances risk stratification for patients of various populations, but data is lacking in pediatric PH. Methods This study included PH patients and a group of age and sex-matched healthy controls who underwent exercise testing between January 2020 and July 2024. The five components of Frailty assessed included peak handgrip strength, 2-minute walk distance, skeletal muscle mass assessed using bioelectrical impedance analysis, the Lansky/Karnofsky functional status score, and patient-reported fatigue score. Individual and Composite Frailty Scores (out of 10) were reported. Frailty scores were compared between PH patients and age and sex matched controls using the Student’s t-test and Mann-Whitney U test. Subsequent cross-sectional analysis, using the Spearman correlation and Kruskal-Wallis test determined the association of both scores with established PH severity markers, including six-minute walk distance (6MWD), non-invasive indices of right ventricular (RV) function and afterload, and invasive hemodynamics. Results The study cohort included 36 pediatric PH patients (mean age 12.4 ± 5.4 years, 61% female), categorized as Group 1 (86%) and Group 3 (14%), with World Health Organization Functional Class I in 2 (6%), II in 31 (86%), and III in 3 (8%) patients. PH therapy included monotherapy in 5 (14%), dual in 7 (19%), triple in 19 (53%), and quadruple in 1 (3%) patient(s). Pediatric PH patients had higher composite frailty scores (median 4.0 2.5, 6.0 vs. 2.0 0.5, 3.0, p 0.001) and lower functional status (median 80 70, 80 vs. 100 100, 100, p 0.001) compared to healthy controls. Higher frailty and lower functional status scores correlated with lower 6MWD (p = 0.0046 and 0.00015) and worse RV function (tricuspid annular plane systolic excursion, p = 0.039 and 0.017). Higher frailty scores were associated with higher RV systolic pressure, mean pulmonary artery pressure, and pulmonary vascular resistance (p = 0.014, 0.014, and 0.029 respectively). Conclusions Frailty and functional status scores were worse in PH patients than healthy controls and were associated with standard metrics of PH severity. Integrating these simple non-invasive assessments has the potential to help identify high-risk individuals. Future studies should investigate the predictive power of these scores for adverse clinical outcomes in this vulnerable population. This abstract is funded by: Cincinnati Children’s Hospital Medical Center
Wleklinski et al. (2026) conducted a cross-sectional in Pediatric pulmonary hypertension (n=36). Frailty and functional status assessment vs. Healthy controls was evaluated on Composite frailty scores (p=<0.001). Pediatric pulmonary hypertension patients had higher composite frailty scores (median 4.0 vs 2.0, p<0.001) and lower functional status (median 80 vs 100, p<0.001) compared to healthy controls.