Academic Journal
Examination of elevated myocardial extracellular volume and exercise capacity in pediatric Fontan patients at moderate altitude.
| Τίτλος: | Examination of elevated myocardial extracellular volume and exercise capacity in pediatric Fontan patients at moderate altitude. |
|---|---|
| Συγγραφείς: | Arnouts EC; School of Medicine, University of Colorado Anschutz Medical Campus, 13001 E 17th Pl, Aurora, CO, 80045, USA., Drysdale ND; Department of Surgery, University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Gerstner Saucedo J; School of Medicine, University of Colorado Anschutz Medical Campus, 13001 E 17th Pl, Aurora, CO, 80045, USA. jochen.gerstnersaucedo@cuanschutz.edu.; Department of Pediatrics, Division of Pediatric Radiology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA. jochen.gerstnersaucedo@cuanschutz.edu., Gonzalez de Alba C; Department of Pediatrics, Division of Pediatric Cardiology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Malone LJ; Department of Pediatrics, Division of Pediatric Radiology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Fonseca B; Department of Pediatrics, Division of Pediatric Cardiology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA., DiMaria MV; Department of Pediatrics, Division of Pediatric Cardiology, Michigan Medicine, Ann Arbor, MI, USA., Browne LP; Department of Pediatrics, Division of Pediatric Radiology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Barker AJ; School of Medicine, University of Colorado Anschutz Medical Campus, 13001 E 17th Pl, Aurora, CO, 80045, USA. alex.barker@cuanschutz.edu.; Department of Pediatrics, Division of Pediatric Radiology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA. alex.barker@cuanschutz.edu. |
| Πηγή: | Pediatric radiology [Pediatr Radiol] 2026 Sep; Vol. 56 (10), pp. 2260-2271. Date of Electronic Publication: 2026 Jun 03. |
| Τύπος έκδοσης: | Journal Article |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: Springer-Verlag Country of Publication: Germany NLM ID: 0365332 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1432-1998 (Electronic) Linking ISSN: 03010449 NLM ISO Abbreviation: Pediatr Radiol Subsets: MEDLINE |
| Imprint Name(s): | Original Publication: Berlin, New York, Springer-Verlag. |
| Ιατρικοί όροι (MeSH): | Exercise Tolerance*/physiology , Magnetic Resonance Imaging*/methods , Heart Defects, Congenital*/surgery , Heart Defects, Congenital*/physiopathology , Heart Defects, Congenital*/diagnostic imaging , Myocardium*/pathology , Fontan Procedure* , Altitude*, Humans ; Child ; Female ; Male ; Adolescent ; Retrospective Studies ; Exercise Test ; Fibrosis |
| Περίληψη: | Background: Diffuse myocardial fibrosis, as measured by T1 (longitudinal relaxation time) mapping and extracellular volume, may be associated with ventricular dysfunction in the Fontan circulation. However, the relationship between diffuse myocardial fibrosis and exercise tolerance, an early and clinically meaningful marker of Fontan physiology, remains incompletely characterized, particularly in pediatric populations. Objective: To evaluate the association between CMR-derived markers of diffuse myocardial fibrosis (native T1 and ECV) and exercise tolerance in pediatric patients with Fontan circulation compared with healthy controls. Materials and Methods: A retrospective cohort study of pediatric patients with Fontan circulation who underwent clinically indicated cardiovascular magnetic resonance (CMR) with native and post-contrast T1 mapping between 2019 and 2022 was performed. Global native T1 and ECV were quantified and compared with age- and scanner-matched controls. Cardiopulmonary exercise testing-derived VO₂ max and catheterization parameters obtained within 24 months of CMR were analyzed. Associations between myocardial fibrosis markers and functional measures were assessed using correlation analyses. Results: A cohort of 64 pediatric Fontan patients (median age 11.0 years, interquartile range 7.0-14.0; age range <18 years), and 41 pediatric controls, was stratified by field strength and ventricular dominance. Fontan patients demonstrated increased native T1 values across varying field strengths of 1.5 T (1,035.9 ± 48.7 ms vs 983.8 ± 29.0 ms, P<0.001) and 3 T (1,292.8 ± 53.5 ms vs 1,257.8 ± 41.5 ms, P=0.033), and significantly elevated ECV compared to controls (25.6 ± 3.5% vs 23.2 ± 2.1%, P=0.007). There was no significant difference in native T1 or ECV between dominant right and left ventricles. VO₂ max was markedly reduced in Fontan patients compared to controls (1.2 ± 0.4 liters per minute (LPM) vs 2.5 ± 0.7 LPM, P<0.001). While a broad-inverse correlation between ECV and VO₂ max did not reach statistical significance across the full cohort (R=-0.19, P>0.05), time since Fontan surgery was inversely correlated with ECV (R=-0.27, P<0.05), and localized subgroup correlations emerged between native T1 and exercise capacity. Conclusions: Markers of myocardial fibrosis were elevated among Fontan patients compared to healthy controls. Fontan patients were shown to have reduced exercise tolerance compared to controls as measured by VO2 max. While myocardial ECV is elevated in pediatric Fontan patients, its relationship with impaired exercise capacity is complex and subgroup-specific, suggesting a need for additional study and functional decline may be driven by multifactorial etiologies. (© 2026. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.) |
| Competing Interests: | Declarations. Conflicts of interest: None |
| References: | Circulation. 2019 Aug 6;140(6):e234-e284. (PMID: 31256636) Congenit Heart Dis. 2018 Mar;13(2):181-193. (PMID: 29372588) Heart. 2016 Jul 15;102(14):1081-6. (PMID: 27220691) Acta Biomed. 2021 Nov 03;92(5):e2021260. (PMID: 34738582) Int J Mol Sci. 2020 Sep 03;21(17):. (PMID: 32899304) J Am Coll Cardiol. 2008 Jul 8;52(2):99-107. (PMID: 18598887) J Cardiovasc Magn Reson. 2021 Nov 15;23(1):134. (PMID: 34781968) Pediatr Cardiol. 2025 Apr;46(4):966-975. (PMID: 38771376) Circ Cardiovasc Imaging. 2014 May;7(3):502-9. (PMID: 24619103) J Am Heart Assoc. 2019 May 7;8(9):e012008. (PMID: 31041880) J Cardiovasc Magn Reson. 2020 Dec 14;22(1):87. (PMID: 33308262) J Cardiovasc Magn Reson. 2020 Nov 30;22(1):77. (PMID: 33250055) Int J Cardiol. 2017 Aug 1;240:172-177. (PMID: 28461021) Pediatr Cardiol. 1999 Jan-Feb;20(1):57-9; discussion 60. (PMID: 9861079) Am J Hematol. 2020 Oct;95(10):E255-E257. (PMID: 32530536) J Cardiol. 2014 Nov;64(5):384-9. (PMID: 24698006) J Am Heart Assoc. 2022 Dec 20;11(24):e026181. (PMID: 36515232) Open Heart. 2020 Oct;7(2):. (PMID: 33109703) Radiology. 2019 Feb;290(2):317-326. (PMID: 30422092) Am J Clin Pathol. 2020 Aug 5;154(3):342-352. (PMID: 32525543) Clin Chem. 2015 Aug;61(8):1075-86. (PMID: 26044509) |
| Contributed Indexing: | Keywords: Exercise tolerance; Extracellular volume; Fontan; Myocardial fibrosis; Native T1; VO2 max |
| Entry Date(s): | Date Created: 20260603 Date Completed: 20260915 Latest Revision: 20261008 |
| Update Code: | 20261008 |
| DOI: | 10.1007/s00247-026-06675-2 |
| PMID: | 42233987 |
| Βάση Δεδομένων: | MEDLINE |
καταχωρήστε σχόλιο πρώτοι!