Upper Extremity Exercise Capacity in adolescent and young adult with pulmonary arterial hypertension: The Role of Muscle Function
Year 2025,
Volume: 3 Issue: 3, 124 - 133, 10.09.2025
Jan Dik
,
Ceyhun Topcuoğlu
,
Naciye Vardar Yaglı
,
Ebru Aypar
,
Dursun Alehan
,
Melda Saglam
Abstract
Background:
Pulmonary arterial hypertension (PAH) impairs aerobic capacity through combined cardiopulmonary and skeletal muscle dysfunction. However, upper extremity aerobic performance and muscle-related parameters correlates have not been widely explored in PAH population.
Objective: To investigate the relationship between maximal workload achieved during an arm ergometer test and peripheral muscle parameters in adolescents and young adults with PAH.
Methods:
Eighteen PAH patients were evaluated; two were excluded due to protocol deviations, resulting in a final sample of 16 participants (mean age: 20.4 ± 8.2 years; 9 females). Maximum workload (watts) was assessed via an arm ergometer test. Handgrip strength was measured using a handgrip dynamometer, and shoulder abduction strength with a digital dynamometer ; both were converted to percent predicted using age- and sex-adjusted normative equations. Fat-free mass (FFM) was calculated from body composition using bioelectrical impedance analysis. Surface electromyography (EMG) of the anterior deltoid was recorded during the first and end stage of the exercise and expressed as % maximal voluntary isometric contraction (MVIC), in accordance with Surface EMG for the Non-Invasive Assessment of Muscles (SENIAM) standards. Brain Natriuretic Peptide (BNP) levels were obtained from recent clinical records. Spearman correlation was used to evaluate associations.
Results:
Maximal workload was positively correlated with FFM (r = 0.615, p = 0.011), shoulder abduction strength (r = 0.535, p = 0.033), and handgrip strength (r = 0.648, p = 0.014). It was negatively correlated with BNP (r = –0.728, p = 0.005) and the maximum %MVIC value of anterior deltoid EMG obtained from the first 15 seconds of the initial arm ergometer test stage (r = –0.602, p = 0.014). A negative correlation was also observed with EMG from the final stage, though it did not reach statistical significance (r=–0.442, p=0.086).
Conclusion:
Upper extremity aerobic performance in PAH is significantly associated with muscle strength, mass, and activation, as well as cardiac stress. These results highlight the clinical relevance of specifically assessing upper extremity aerobic performance as a meaningful indicator of functional status in PAH.
Ethical Statement
This study was approved by the Hacettepe University Non-Interventional Ethics Committee (Ethics Committee No: GO 22/39). All participants provided informed consent prior to their inclusion in the study.
Supporting Institution
Hacettepe University, Faculty of Physical Therapy and Rehabilitation; Hacettepe University, Faculty of Medicine, Department of Pediatrics, Division of Pediatric Cardiology
Thanks
The authors would like to express their sincere gratitude to Prof. Dr. Melda Sağlam for her supervision and scientific contribution throughout the study. The authors also thank Prof. Dr. Dursun Alehan and Prof. Dr. Ebru Aypar from the Division of Pediatric Cardiology for their clinical support, and all the staff and participants who contributed to this study.
References
-
1. Vanhoof JM, Delcroix M, Vandevelde E, Denhaerynck K, Wuyts W, Belge C, et al. Emotional symptoms and quality of life in patients with pulmonary arterial hypertension. The Journal of Heart and Lung Transplantation. 2014;33(8):800-8.
-
2. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RM, Brida M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG). European heart journal. 2022;43(38):3618-731.
-
3. Peng Zhang DDSGB, Alexander Vang, Julia Feord, Danielle J. McCullough, Alexsandra Zimmer, Natalie D'Silva, Richard T. Clements, Gaurav Choudhary. Reduced exercise capacity occurs before intrinsic skeletal muscle dysfunction in experimental rat models of pulmonary hypertension. Pulmonary circulation. 2024;14(2 ).
-
4. Mainguy V, Maltais F, Saey D, Gagnon P, Martel S, Simon M, et al. Peripheral muscle dysfunction in idiopathic pulmonary arterial hypertension. Thorax. 2010;65(2):113-7.
-
5. Burcu Camcıoğlu Yılmaz MBG, Müşerrefe Nur Keleş, Gülten Aydoğdu Taçoy, Atiye Çengel,. Effects of upper extremity aerobic exercise training on oxygen consumption, exercise capacity, dyspnea and quality of life in patients with pulmonary arterial hypertension. Heart & Lung. 2020;49(5):564-71.
-
6. Hermens HJ, Freriks B, Disselhorst-Klug C, Rau G. Development of recommendations for SEMG sensors and sensor placement procedures. J Electromyogr Kinesiol. 2000;10(5):361-74.
-
7. Sturma A, Hruby LA, Prahm C, Mayer JA, Aszmann OC. Rehabilitation of Upper Extremity Nerve Injuries Using Surface EMG Biofeedback: Protocols for Clinical Application. Frontiers in Neuroscience. 2018;12.
-
8. Leuchte HH, Ten Freyhaus H, Gall H, Halank M, Hoeper MM, Kaemmerer H, et al. Risk stratification strategy and assessment of disease progression in patients with pulmonary arterial hypertension: updated recommendations from the Cologne Consensus Conference 2018. International journal of cardiology. 2018;272:20-9.
-
9. Ivy DD, Abman SH, Barst RJ, Berger RM, Bonnet D, Fleming TR, et al. Pediatric pulmonary hypertension. Journal of the American College of Cardiology. 2013;62(25S):D117-D26.
-
10. Hamilton GF, McDonald C, Chenier TC. Measurement of grip strength: validity and reliability of the sphygmomanometer and jamar grip dynamometer. Journal of Orthopaedic & Sports Physical Therapy. 1992;16(5):215-9.
-
11. Mijnarends DM, Meijers JM, Halfens RJ, ter Borg S, Luiking YC, Verlaan S, et al. Validity and reliability of tools to measure muscle mass, strength, and physical performance in community-dwelling older people: a systematic review. Journal of the American Medical Directors Association. 2013;14(3):170-8.
-
12. Bohannon RW, Peolsson A, Massy-Westropp N, Desrosiers J, Bear-Lehman J. Reference values for adult grip strength measured with a Jamar dynamometer: a descriptive meta-analysis. Physiotherapy. 2006;92(1):11-5.
-
13. McQuiddy VA, Scheerer CR, Lavalley R, McGrath T, Lin L. Normative values for grip and pinch strength for 6-to 19-year-olds. Archives of physical medicine and rehabilitation. 2015;96(9):1627-33.
-
14. Alidadi Y, Metanati M, Ataie-Jafari A. The validity of a bioelectrical impedance analyzer, Xiaomi MI scale 2, for measurement of body composition. 2019;2(2):36-8.
-
15. Kyle UG, Bosaeus I, De Lorenzo AD, Deurenberg P, Elia M, Gómez JM, et al. Bioelectrical impedance analysis—part I: review of principles and methods. Clinical nutrition. 2004;23(5):1226-43.
-
16. Sedgwick P. Spearman’s rank correlation coefficient. Bmj. 2014;349.
-
17. Pepplinkhuizen S, Eshuis G, Zijlstra WM, Timmer CY, Ploegstra MJ, Lelieveld OT, et al. Muscle strength is reduced in children with pulmonary arterial hypertension. Pulmonary circulation. 2023;13(2):e12246.
-
18. de Man Fd, Handoko M, Groepenhoff H, Van't Hul A, Abbink J, Koppers R, et al. Effects of exercise training in patients with idiopathic pulmonary arterial hypertension. European Respiratory Journal. 2009;34(3):669-75.
-
19. Laveneziana P, Albuquerque A, Aliverti A, Babb T, Barreiro E, Dres M, et al. ERS statement on respiratory muscle testing at rest and during exercise. European Respiratory Journal. 2019;53(6).
-
20. Gómez CA. Non-Invasive Monitoring of the Respiratory Muscles via Diffuse Optical Modalities: Boston University; 2024.
-
21. Calatayud J, Torres-Castro R, Vera-Uribe R, Olivares-Valenzuela Á, Guzmán-González B, Torres ME, et al. Neuromuscular and acute symptoms responses to progressive elastic resistance exercise in patients with chronic obstructive pulmonary disease: cross-sectional study. Frontiers in Medicine. 2022;9:934410.
-
22. Nagaya N, Nishikimi T, Okano Y, Uematsu M, Satoh T, Kyotani S, et al. Plasma brain natriuretic peptide levels increase in proportion to the extent of right ventricular dysfunction in pulmonary hypertension. Journal of the American College of Cardiology. 1998;31(1):202-8.
-
23. Lewis GD, Bossone E, Naeije R, Grünig E, Saggar R, Lancellotti P, et al. Pulmonary vascular hemodynamic response to exercise in cardiopulmonary diseases. Circulation. 2013;128(13):1470-9.
-
24. Mereles D, Ehlken N, Kreuscher S, Ghofrani S, Hoeper MM, Halank M, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation. 2006;114(14):1482-9.
-
25. González-Saiz L, Fiuza-Luces C, Sanchis-Gomar F, Santos-Lozano A, Quezada-Loaiza CA, Flox-Camacho A, et al. Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+ 12 trial. International journal of cardiology. 2017;231:277-83.
-
26. Saglam M, Vardar-Yagli N, Calik-Kutukcu E, Arikan H, Savci S, Inal-Ince D, et al. Functional exercise capacity, physical activity, and respiratory and peripheral muscle strength in pulmonary hypertension according to disease severity. Journal of physical therapy science. 2015;27(5):1309-12.