{"doi":"10.1016/j.cjcpc.2025.10.010","title":"Exercise Capacity, Endothelial Function, Muscle Mass, and Strength in Pediatric Patients With Fontan Circulation","abstract":"<h2>Abstract</h2><h3>Background</h3> Fontan circulation carries high morbidity and mortality, driven in part by impaired exercise capacity, muscle deficits, and endothelial dysfunction. In a pediatric cohort with Fontan circulation, we examined associations between exercise capacity, endothelial function, and muscle parameters before enrollment in a randomized exercise intervention. <h3>Methods</h3> Patients aged 8-19 years who have Fontan circulation were included. Exclusion criteria included New York Heart Association class IV, active illness, protein-losing enteropathy, pacemaker, or cognitive delay. Assessments included cardiopulmonary exercise testing (peak VO<sub>2</sub>), endothelial function testing (reactive hyperemia index [RHI]), fasting lipid profile, dual-energy x-ray absorptiometry and dynamometry (muscle mass and strength), and functional movement screening (FMS). <h3>Results</h3> Among 137 participants (median age 12.7 years; 53% single right ventricle), median time since Fontan was 8.6 years. Peak VO<sub>2</sub> was 1402 ± 509 mL/min (75.7% ± 17.9% predicted; 31.1 ± 7.4 mL/kg/min indexed). RHI was 1.45 ± 0.6; NOx 22.2 ± 17.4 μmol/L. The low-density lipoprotein cholesterol level was 65.8 ± 18.9 mg/dL. Length-adjusted leg lean mass, handgrip strength, and leg extension strength <i>z</i> scores were –1.02 ± 1.0, –0.76 ± 0.9, and –0.70 ± 1.0, respectively. The FMS score averaged 11.9 ± 3.0. Peak VO<sub>2</sub> correlated with RHI (<i>r</i> = 0.50), lnRHI (<i>r</i> = 0.51), FMS (<i>r</i> = 0.30) (all <i>P</i> < 0.001), and length-adjusted leg lean mass <i>z</i> score (<i>r</i> = 0.25, <i>P</i> = 0.003). Indexed and percent-predicted peak VO<sub>2</sub> also correlated with FMS (<i>r</i> = 0.31 and 0.30, respectively) and high-density lipoprotein (<i>r</i> = 0.24 and <i>r</i> = 0.25, respectively). <h3>Conclusion</h3> In youth with Fontan circulation, lower aerobic capacity was associated with lower endothelial function, leg lean mass, strength, and mobility, underscoring the interdependence of cardiovascular, vascular, and musculoskeletal health. <h3>Clinical Trial</h3> NCT04195451","journal":"CJC Pediatric and Congenital Heart Disease","year":2025,"id":535306,"datarank":0.16479184330021646,"base_score":1.0986122886681096,"endowment":1.0986122886681096,"self_citation_contribution":0.16479184330021646,"citation_network_contribution":0.0,"self_endowment_contribution":0.16479184330021646,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":2,"citer_count":2,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9419,"is_data_producer":true,"deposit_databanks":{"ClinicalTrials.gov":["NCT04195451"]},"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2025-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":74985,"name":"Latha Palaniappan","orcid":"0000-0002-1245-665X","position":1,"is_corresponding":false},{"id":417333,"name":"Mary B. Leonard","orcid":"0000-0002-8972-6582","position":2,"is_corresponding":false},{"id":143318,"name":"Jin Long","orcid":null,"position":3,"is_corresponding":false},{"id":1262774,"name":"Joseph Dehoney","orcid":null,"position":4,"is_corresponding":false},{"id":276970,"name":"John P. Cooke","orcid":"0000-0003-0033-9138","position":5,"is_corresponding":false},{"id":1086110,"name":"Inger Olson","orcid":null,"position":6,"is_corresponding":false},{"id":615693,"name":"Tulsi Ram Damase","orcid":"0000-0002-6758-9038","position":7,"is_corresponding":false},{"id":326753,"name":"Sharon Chen","orcid":"0000-0002-0576-0856","position":8,"is_corresponding":false},{"id":397973,"name":"Jonathan Myers","orcid":"0000-0003-2592-136X","position":9,"is_corresponding":false},{"id":229036,"name":"Daniel Bernstein","orcid":"0000-0001-7761-5853","position":10,"is_corresponding":false},{"id":969340,"name":"Elif Seda Selamet Tierney","orcid":"0000-0002-4654-3794","position":11,"is_corresponding":false},{"id":1085648,"name":"Mavis C. Lui","orcid":"0000-0003-0555-1672","position":0,"is_corresponding":true}],"reference_count":49,"raw_metadata":null,"created_at":"2026-07-19T02:51:56.297114Z","pmid":"42422410","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}