Respiratory and peripheral muscle involvement in patients with pulmonary arterial hypertension due to congenital heart diseases
Skeletal and respiratory muscle dysfunction has been previously described in patients with other etiologic subgroups of pulmonary arterial hypertension (PAH) but has never been investigated in patients with PAH due to congenital heart diseases (CHD). This study aims to show the involvement of skeletal and respiratory muscles in these patients. This cross-sectional study included patients with PAH due to CHD and healthy controls. Patients’ demographic properties, six-minute walk tests; shoulder abduction, handgrip, knee extension, and ankle dorsiflexion muscle strength, maximum inspiratory (MIP) and expiratory pressures (MEP) were measured. Deltoid, flexor digitorum superficialis, and profundus, tibialis anterior and rectus femoris muscles were visualized with ultrasonography and their cross-sectional areas (CSA) were also measured in both groups. 12 patients and 12 controls were included. Mean MIP was 104.22±32.57 cm H2O for healthy participants while 61.33±29.74 cm H2O for patients (p<0.001). For mean MEP, it was 100.08±26.05 cm H2O in healthy participants and 69.75±39.79 cmH2O in controls (p=0.004). When the strength of skeletal muscles was compared, there were significant differences between the groups in all measurements except for bilateral grip strength. In the correlation analysis, MIP and MEP values showed no significant correlations with clinical parameters. They showed significant moderate correlations with skeletal muscle strength. When CSAs of the muscles were compared, there were significant differences in all measurements except for left FDS and FDP and bilateral rectus femoris. This study showed that in patients with pulmonary arterial hypertension due to CHD, respiratory muscle strength is significantly worse than healthy participants. Patients had also significantly worse skeletal muscle strength except for grip strength.
Galiè N, Humbert M, Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint 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: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016;37:67-119. DOI: https://doi.org/10.1093/eurheartj/ehv317
Spiesshoefer J, Orwat S, Henke C, et al. Inspiratory muscle dysfunction and restrictive lung function impairment in congenital heart disease: Association with immune inflammatory response and exercise intolerance. Int J Cardiol 2020;318:45-51. DOI: https://doi.org/10.1016/j.ijcard.2020.06.055
Meyer FJ, Lossnitzer D, Kristen AV, et al. Respiratory muscle dysfunction in idiopathic pulmonary arterial hypertension. Eur Respir J 2005;25:125-30. DOI: https://doi.org/10.1183/09031936.04.00095804
Aslan GK, Akinci B, Yeldan I, Okumus G. Respiratory muscle strength in patients with pulmonary hypertension: The relationship with exercise capacity, physical activity level, and quality of life. Clin Respir J 2018;12:699-705.
Riou M, Pizzimenti M, Enache I, et al. Skeletal and respiratory muscle dysfunctions in pulmonary arterial hypertension. J Clin Med 2020;9:410. DOI: https://doi.org/10.3390/jcm9020410
Laboratories ATSCoPSfCPF. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 2002;166:111-7. DOI: https://doi.org/10.1164/ajrccm.166.1.at1102
Andrews AW, Thomas MW, Bohannon RW. Normative values for isometric muscle force measurements obtained with hand-held dynamometers. Phys Ther 1996;76:248-59. DOI: https://doi.org/10.1093/ptj/76.3.248
Maayah MF, Al-Jarrah MD, El Zahrani SS, et al. Test-retest strength reliability of the Electronic Push/Pull Dynamometer (EPPD) in the measurement of the quadriceps and hamstring muscles on a new chair. Open J Int Med 2012;2:123-8. DOI: https://doi.org/10.4236/ojim.2012.22022
Ashford RF, Nagelburg S, Adkins R. Sensitivity of the Jamar Dynamometer in detecting submaximal grip effort. J Hand Surg Am 1996;21:402-5. DOI: https://doi.org/10.1016/S0363-5023(96)80352-2
American Thoracic Society/European Respiratory S. ATS/ERS Statement on respiratory muscle testing. Am J Respir Crit Care Med 2002;166:518-624. DOI: https://doi.org/10.1164/rccm.166.4.518
Bhansing KJ, Van Rosmalen MH, Van Engelen BG, Vonk MC, Van Riel PL, Pillen S. Increased fascial thickness of the deltoid muscle in dermatomyositis and polymyositis: An ultrasound study. Muscle Nerve 2015;52:534-9. DOI: https://doi.org/10.1002/mus.24595
Yaşar E, Adigüzel E, Kesikburun S, et al. Assessment of forearm muscle spasticity with sonoelastography in patients with stroke. Br J Radiol 2016;89:20160603. DOI: https://doi.org/10.1259/bjr.20160603
Seymour JM, Ward K, Sidhu PS, et al. Ultrasound measurement of rectus femoris cross-sectional area and the relationship with quadriceps strength in COPD. Thorax 2009;64:418-23. DOI: https://doi.org/10.1136/thx.2008.103986
Sandberg C, Thilén U, Wadell K, Johansson B. Adults with complex congenital heart disease have impaired skeletal muscle function and reduced confidence in performing exercise training. Eur J Prev Cardiol 2015;22:1523-30. DOI: https://doi.org/10.1177/2047487314543076
Gil Obando LM, López López A, Avila CL. Normal values of the maximal respiratory pressures in healthy people older than 20 years old in the City of Manizales - Colombia. Colomb Med (Cali) 2012;43:119-25. DOI: https://doi.org/10.25100/cm.v43i2.1141
Sclauser Pessoa IM, Franco Parreira V, Fregonezi GA, et al. Reference values for maximal inspiratory pressure: a systematic review. Can Respir J 2014;21:43-50. DOI: https://doi.org/10.1155/2014/982374
Aslan GK, Akinci B, Yeldan I, Okumus G. Respiratory muscle strength in patients with pulmonary hypertension: The relationship with exercise capacity, physical activity level, and quality of life. Clin Respir J 2018;12:699-705. DOI: https://doi.org/10.1111/crj.12582
Marra AM, Arcopinto M, Bossone E, et al. Pulmonary arterial hypertension-related myopathy: an overview of current data and future perspectives. Nutr Metab Cardiovasc Dis 2015;25:131-9. DOI: https://doi.org/10.1016/j.numecd.2014.10.005
Velez-Roa S, Ciarka A, Najem B, et al. Increased sympathetic nerve activity in pulmonary artery hypertension. Circulation 2004;110:1308-12. DOI: https://doi.org/10.1161/01.CIR.0000140724.90898.D3
Bauer R, Dehnert C, Schoene P, et al. Skeletal muscle dysfunction in patients with idiopathic pulmonary arterial hypertension. Respir Med 2007;101:2366-9. DOI: https://doi.org/10.1016/j.rmed.2007.06.014
Greutmann M, Le TL, Tobler D, et al. Generalised muscle weakness in young adults with congenital heart disease. Heart 2011;97:1164-8. DOI: https://doi.org/10.1136/hrt.2010.213579
Kröönström LA, Johansson L, Zetterström A-K, et al. Muscle function in adults with congenital heart disease. Int J Cardiol 2014;170:358-63. DOI: https://doi.org/10.1016/j.ijcard.2013.11.014
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