Exercise capacity in patients with cystic fibrosis vs. non-cystic fibrosis bronchiectasis

Ronen Bar-Yoseph, Anat Ilivitzki, Dan M Cooper, Michal Gur, Gur Mainzer, Fahed Hakim, Galit Livnat, Zeev Schnapp, George Shalloufeh, Merav Zucker-Toledano, Yael Subar, Lea Bentur, Ronen Bar-Yoseph, Anat Ilivitzki, Dan M Cooper, Michal Gur, Gur Mainzer, Fahed Hakim, Galit Livnat, Zeev Schnapp, George Shalloufeh, Merav Zucker-Toledano, Yael Subar, Lea Bentur

Abstract

Background: Bronchiectasis is associated with morbidity, low exercise capacity and poor quality of life. There is a paucity of data on exercise capacity using cardiopulmonary exercise test (CPET) in non-cystic fibrosis (CF) bronchiectasis. Our aim was to compare exercise capacity using CPET in CF and non-CF bronchiectasis patients.

Methods: Cross-sectional retrospective/prospective controlled study assessing CPET using cycle ergometer. Exercise parameters and computed tomography (CT) findings were compared. Results: Hundred two patients with bronchiectasis and 88 controls were evaluated; 49 CF (age 19.7 ± 9.7 y/o, FEV1%predicted 70.9 ± 20.5%) and 53 non-CF (18.6 ± 10.6 y/o, FEV1%predicted 68.7 ± 21.5%). Peak oxygen uptake (peak [Formula: see text]) was similar and relatively preserved in both groups (CF 1915.5±702.0; non-CF 1740±568; control 2111.0±748.3 mL/min). Breathing limitation was found in the two groups vs. control; low breathing reserve (49% in CF; 43% non-CF; 5% control) and increased [Formula: see text] (CF 31.4±4.1, non-CF 31.7±4.1 and control 27.2 ± 2.8). Oxygen pulse was lower in the non-CF; whereas a linear relationship between peak [Formula: see text] vs. FEV1 and vs. FVC was found only for CF. CT score correlated with [Formula: see text] and negatively correlated with [Formula: see text] and post exercise oxygen saturation (SpO2).

Conclusions: CPET parameters may differ between CF and non-CF bronchiectasis. However, normal exercise capacity may be found unrelated to the etiology of the bronchiectasis. Anatomical changes in CT are associated with functional finding of increased [Formula: see text] and decreased SpO2. Larger longitudinal studies including cardiac assessment are needed to better study exercise capacity in different etiologies of non-CF bronchiectasis.

Trial registration: ClinicalTrials.gov, registration number: NCT03147651.

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Fig 1. Baseline FEV1 (%pred) and peak…
Fig 1. Baseline FEV1 (%pred) and peak V˙O2 (%pred) relationship.
Moderate linear relationship (r = 0.63, p

References

    1. Gao Y, Guan W, Xu G, Lin Z, Tang Y, Lin Z, et al. Sleep Disturbances and Health-Related Quality of Life in Adults with Steady-State Bronchiectasis. PLoS One. 2014;9: e102970 10.1371/journal.pone.0102970
    1. Aliberti S, Lonni S, Dore S, McDonnell MJ, Goeminne PC, Dimakou K, et al. Clinical phenotypes in adult patients with bronchiectasis. Eur Respir J. 2016; 10.1183/13993003.01899-2015
    1. Brower KS, Del Vecchio MT, Aronoff SC. The etiologies of non-CF bronchiectasis in childhood: a systematic review of 989 subjects. BMC Pediatr. 2014;14: 4 10.1186/s12887-014-0299-y
    1. Nixon PA, Orenstein DM, Kelsey SF, Doershuk CF. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med. 1992;327: 1785–8. 10.1056/NEJM199212173272504
    1. Pianosi P, Leblanc J, Almudevar A. Peak oxygen uptake and mortality in children with cystic fibrosis. Thorax. 2005;60: 50–4. 10.1136/thx.2003.008102
    1. Swaminathan S, Kuppurao K V, Somu N, Vijayan VK. Reduced exercise capacity in non-cystic fibrosis bronchiectasis. Indian J Pediatr. 2003;70: 553–556. 10.1007/BF02723157
    1. Rosenthal M, Narang I, Edwards L, Bush A. Non-Invasive assessment of exercise performance in children with cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis: Is there a CF specific muscle defect? Pediatr Pulmonol. 2009;44: 222–230. 10.1002/ppul.20899
    1. Fielding J, Brantley L, Seigler N, McKie KT, Davison GW, Harris RA. Oxygen uptake kinetics and exercise capacity in children with cystic fibrosis. Pediatr Pulmonol. 2015;50: 647–654. 10.1002/ppul.23189
    1. Pastré J, Prévotat A, Tardif C, Langlois C, Duhamel A, Wallaert B. Determinants of exercise capacity in cystic fibrosis patients with mild-to-moderate lung disease. BMC Pulm Med. 2014;14: 74 10.1186/1471-2466-14-74
    1. Wells GD, Wilkes DL, Schneiderman JE, Rayner T, Elmi M, Selvadurai H, et al. Skeletal Muscle Metabolism in Cystic Fibrosis and Primary Ciliary Dyskinesia. Pediatr Res. 2011;69: 40–45. 10.1203/PDR.0b013e3181fff35f
    1. Giacchi V, Rotolo N, Amato B, Di Dio G, Betta P, La Rosa M, et al. Heart Involvement in Children and Adults with Cystic Fibrosis: Correlation with Pulmonary Indexes and Inflammation Markers. Hear Lung Circ. 2015;24 10.1016/j.hlc.2015.03.006
    1. Cooper DM, Bar-Yoseph R, Olin JT, Radom-Aizik S. Exercise and Lung Function in Child Health and Disease. Kendig and Chernick’s Disorders of the Respiratory Tract in Children. 9th ed. Philadelphia, PA: Elsevier; 2018. pp. 2017–223.
    1. Edgeworth D, Keating D, Ellis M, Button B, Williams E, Clark D, et al. Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: a double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment. Clin Sci. 2017;131: 2037–2045. 10.1042/CS20170995
    1. Linda S Pescatello Ross Arena, Deborah Riebe PDT, editor. ACSM’s Guidelines for Exercise Testing and Prescription. 9th ed. Philadelphia, PA: Paul D Thompson Wolters Kluwer/Lippincott Williams & Wilkins; 2014. pp. 52–53.
    1. American Thoracic Society, American College of Chest Physicians. Statement on cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2003;167: 211–277. 10.1164/rccm.167.2.211
    1. Tanaka H, Monahan KD, Seals DR. Age-predicted maximal heart rate revisited. J Am Coll Cardiol. 2001;37: 153–6.
    1. Wasserman K, Hansen JE, Sue DY, Stringer WW, Sietsema KE, Sun XG WB. Principles of exercise testing and interpretation 5th ed. Philadelphia, USA: Lippincott Williams & Wilkins; 2012.
    1. Quon BS, Wilkie SS, Molgat-Seon Y, Schaeffer MR, Ramsook AH, Wilcox PG, et al. Cardiorespiratory and sensory responses to exercise in adults with mild cystic fibrosis. J Appl Physiol. 2015;119: 1289–96. 10.1152/japplphysiol.00692.2015
    1. Park J, Kim S, Lee YJ, Park JS, Cho Y-J, Yoon H Il, et al. Factors associated with radiologic progression of non-cystic fibrosis bronchiectasis during long-term follow-up. Respirology. 2016;21: 1049–1054. 10.1111/resp.12768
    1. Bhalla M, Turcios N, Aponte V, Jenkins M, Leitman BS, McCauley DI, et al. Cystic fibrosis: scoring system with thin-section CT. Radiology. 1991;179: 783–788. 10.1148/radiology.179.3.2027992
    1. Edwards EA, Narang I, Li I, Hansell DM, Rosenthal M, Bush A. HRCT lung abnormalities are not a surrogate for exercise limitation in bronchiectasis. Eur Respir J. 2004;24: 538–544. 10.1183/09031936.04.00142903
    1. Magnin ML, Cros P, Beydon N, Mahloul M, Tamalet A, Escudier E, et al. Longitudinal lung function and structural changes in children with primary ciliary dyskinesia. Pediatr Pulmonol. 2012;47: 816–825. 10.1002/ppul.22577
    1. Gur M, Yaacoby-Bianu K, Ilivitzki A, Bar-Yoseph R, Nir V, Hakim F, et al. Lung Clearance Index (LCI) in Patients with Bronchiolitis Obliterans: A Preliminary Report and Comparison to Cystic Fibrosis Patients. Lung. 2016;194 10.1007/s00408-016-9934-8
    1. Dodd JD, Barry SC, Barry RBM, Gallagher CG, Skehan SJ, Masterson JB. Thin-Section CT in Patients with Cystic Fibrosis: Correlation with Peak Exercise Capacity and Body Mass Index. Radiology. 2006;240: 236–245. 10.1148/radiol.2401050502
    1. Lamhonwah A-M, Bear CE, Huan LJ, Kim Chiaw P, Ackerley CA, Tein I. Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise. Ann Neurol. 2010;67: 802–8. 10.1002/ana.21982
    1. Labombarda F, Saloux E, Brouard J, Bergot E, Milliez P. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes? Respir Med. 2016;118: 31–38. 10.1016/j.rmed.2016.07.011
    1. Stevens D, Oades PJ, Armstrong N, Williams CA. Early oxygen uptake recovery following exercise testing in children with chronic chest diseases. Pediatr Pulmonol. 2009;44: 480–488. 10.1002/ppul.21024
    1. Hulzebos EHJ, Bomhof-Roordink H, Van De Weert-Van Leeuwen PB, Twisk JWR, Arets HGM, Van Der Ent CK, et al. Prediction of mortality in adolescents with cystic fibrosis. Med Sci Sports Exerc. 2014;46: 2047–2052. 10.1249/MSS.0000000000000344
    1. Weir E, Burns PD, Devenny A, Young D, Paton JY. Cardiopulmonary exercise testing in children with cystic fibrosis: one centre’s experience. Arch Dis Child. 2017;102: 440–444. 10.1136/archdischild-2016-310651
    1. Banjar H. A review of 151 cases of pediatric noncystic fibrosis bronchiectasis in a tertiary care center. Ann Thorac Med. 2007;2: 3 10.4103/1817-1737.30354
    1. Valerio G, Giallauria F, Montella S, Vaino N, Vigorito C, Mirra V, et al. Cardiopulmonary assessment in primary ciliary dyskinesia. Eur J Clin Invest. 2012;42: 617–622. 10.1111/j.1365-2362.2011.02626.x
    1. Madsen A, Green K, Buchvald F, Hanel B, Nielsen KG. Aerobic fitness in children and young adults with primary ciliary dyskinesia. PLoS One. 2013;8: 1–8. 10.1371/journal.pone.0071409
    1. Pianosi P, LeBlanc J, Almudevar A. Relationship between FEV1 and peak oxygen uptake in children with cystic fibrosis. Pediatr Pulmonol. 2005;40: 324–329. 10.1002/ppul.20277
    1. Johnson BD, Weisman IM, Zeballos RJ, Beck KC. Emerging concepts in the evaluation of ventilatory limitation during exercise: the exercise tidal flow-volume loop. Chest. 1999;116: 488–503. 10.1378/chest.116.2.488
    1. Radtke T, Hebestreit H, Gallati S, Schneiderman JE, Braun J, Stevens D, et al. CFTR Genotype and Maximal Exercise Capacity in Cystic Fibrosis: A Cross-sectional Study. Ann Am Thorac Soc. 2018;15: 209–216. 10.1513/AnnalsATS.201707-570OC
    1. Kongstad T, Green K, Buchvald F, Skov M, Pressler T, Nielsen KG. Association between spirometry controlled chest CT scores using computer-animated biofeedback and clinical markers of lung disease in children with cystic fibrosis. Eur Clin Respir J. 2017;4: 1318027 10.1080/20018525.2017.1318027
    1. Gustafsson PM, De Jong PA, Tiddens HAWM, Lindblad A. Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax. 2007;63: 129–134. 10.1136/thx.2007.077784
    1. Cohen-Cymberknoh M, Simanovsky N, Hiller N, Gileles Hillel A, Shoseyov D, Kerem E. Differences in disease expression between primary ciliary dyskinesia and cystic fibrosis with and without pancreatic insufficiency. Chest. 2014;145: 738–744. 10.1378/chest.13-1162
    1. Rowan SA, Bradley JM, Bradbury I, Lawson J, Lynch T, Gustafsson P, et al. Lung Clearance Index Is a Repeatable and Sensitive Indicator of Radiological Changes in Bronchiectasis. Am J Respir Crit Care Med. 2014;189: 586–592. 10.1164/rccm.201310-1747OC

Source: PubMed

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