Abstract
Purpose
Hypermobile Ehlers–Danlos syndrome (hEDS) is a connective tissue disorder with many different symptoms such as pain, fatigue, dysautonomia, or respiratory symptoms. Among the respiratory manifestations described, the most frequent are exertional dyspnea and breathing difficulties. Mechanical ventilatory constraints during exercise could participate in these respiratory manifestations. The objective of this study was to explore the response of pulmonary flow-volume loops to exercise in patients with hEDS and to look for dynamic hyperinflation and expiratory flow limitation during exercise.
Methods
For this purpose, breathing pattern and tidal exercise flow-volume loops were recorded at two workloads (30% and 80% of the peak power output) of a constant load exercise test.
Results
Twelve patients were included (11 women, mean age 41 ± 14 years). The results showed a decrease (p = 0.028) in the inspiratory capacity (from 3.12 ± 0.49 L to 2.97 ± 0.52 L), an increase (p = 0.025) in the end-expiratory lung volume (from 0.73 ± 0.68 L to 0.88 ± 0.66 L, i.e., from EELV comprising 17 ± 12% to 21 ± 12% of forced vital capacity) between the two workloads in favor of dynamic hyperinflation, and half of the patients had expiratory flow limitations.
Conclusion
This exploratory study provides evidence for mechanical ventilatory constraints during exercise in patients with hEDS, which may induce discomfort during exercise and could contribute to the respiratory symptomatology.
Trial registration number
This study is part of a larger clinical trial (ID: NCT04680793, December 2020).
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Data availability statement
The data that support the findings of this study are available from the corresponding author upon reasonable request.
Abbreviations
- CLET:
-
Constant load exercise test
- EDS:
-
Ehlers–Danlos syndrome
- EELV:
-
End-expiratory lung volume
- EFL:
-
Expiratory flow limitation
- EILV:
-
End-inspiratory lung volume
- fb :
-
Breathing frequency
- FEV1 :
-
Forced expiratory volume in one second
- FVC:
-
Forced vital capacity
- hEDS:
-
Hypermobile Ehlers–Danlos syndrome
- HR:
-
Heart rate
- IC:
-
Inspiratory capacity
- MVV:
-
Maximal voluntary ventilation
- PPO:
-
Peak power output
- RER:
-
Respiratory exchange ratio
- SpO2 :
-
Arterial oxygen saturation
- Ti :
-
Inspiratory time
- Ttot :
-
Total respiratory time
- \(\dot{V}\)CO2 :
-
Carbon dioxide output
- \(\dot{V}\) E :
-
Minute ventilation
- \(\dot{V}\)O2 :
-
Oxygen uptake
- VT :
-
Tidal volume
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Acknowledgements
The authors would like to thank the patients who agreed to participate in this study and the entire staff of the Clinique de la Mitterie.
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AH, CB, and PM contributed to the design and implementation of the research, to the analysis of the results, and to the writing of the manuscript.
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This study is part of a clinical trial that has been approved by a national ethics committee (CPP Ile de France IV, 2019/71). All patients were volunteers and gave their informed consent.
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Communicated by Susan Hopkins.
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Hakimi, A., Bergoin, C. & Mucci, P. Evidence of ventilatory constraints during exercise in hypermobile Ehlers–Danlos syndrome. Eur J Appl Physiol 122, 2367–2374 (2022). https://doi.org/10.1007/s00421-022-05017-y
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DOI: https://doi.org/10.1007/s00421-022-05017-y