Abstract
Purpose
Increased dyspnea and reduced exercise capacity in pulmonary arterial hypertension (PAH) can be partly attributed to impaired respiratory muscle function. This prospective study was designed to assess the impact of exercise and respiratory training on respiratory muscle strength and 6-min walking distance (6MWD) in PAH patients.
Methods
Patients with invasively confirmed PAH underwent 3 weeks of in-hospital exercise and respiratory training, which was continued at home for another 12 weeks. Medication remained constant during the study period. Blinded observers assessed efficacy parameters at baseline (I) and after 3 (II) and 15 weeks (III). Respiratory muscle function was assessed by twitch mouth pressure (TwPmo) during nonvolitional supramaximal magnetic phrenic nerve stimulation.
Results
Seven PAH patients (4 women; mean pulmonary artery pressure 45 ± 11 mmHg, median WHO functional class 3.1 ± 0.4, idiopathic/associated PAH n = 5/2) were included. The training program was feasible and well tolerated by all patients with excellent compliance. TwPmo was I: 0.86 ± 0.37 kPa, II: 1.04 ± 0.29 kPa, and III: 1.27 ± 0.44 kPa, respectively. 6MWD was I: 417 ± 51 m, II: 509 ± 39 m, and III: 498 ± 39 m, respectively. Both TwPmo (+0.41 ± 0.34 kPa, +56 ± 39 %) and 6MWD (+81 ± 30 m, +20 ± 9 %) increased significantly in the period between baseline and the final assessment (pairwise comparison: p = 0.012/<0.001; RM-ANOVA considering I, II, III: p = 0.037/<0.001).
Conclusions
Exercise and respiratory training as an adjunct to medical therapy may be effective in patients with PAH to improve respiratory muscle strength and exercise capacity. Future, randomized, controlled trials should be carried out to further investigate these findings.
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Acknowledgments
The authors thank all patients who participated in this demanding study. We are grateful to nSpire Health GmbH (Oberthulba, Germany) for technical support, Dr. Claudia Schmoor (Biometry and Data Management, Center of Clinical Trials, University Medical Center Freiburg, Germany) for biometric consulting, and Dr. Sandra Dieni for writing assistance. This work was supported by a research grant from the German Research Society DFG (Deutsche Forschungsgemeinschaft), Bonn, Germany [KA 2992/2-1]. Further support was provided by the German Pulmonary Hypertension self-help group (Pulmonale Hypertonie e.v.), Rheinstetten, Germany. The study was registered at the WHO Primary Register of Germany (German Clinical Trials Register DRKS00003567).
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The authors declare that they have no conflict of interest.
Ethical Standards
The Institutional Review Board for human studies at the Universities of Freiburg and Heidelberg, Germany approved the study protocol. All experiments comply with the current laws of Germany and the study was performed in agreement with the ethical standards laid down in the current version of the Declaration of Helsinki (59th World Medical Association General Assembly, Seoul, Korea, October 2008). Written informed consent was obtained from all patients.
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Hans-Joachim Kabitz and Hinrich-Cordt Bremer have contributed equally to this work.
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Kabitz, HJ., Bremer, HC., Schwoerer, A. et al. The Combination of Exercise and Respiratory Training Improves Respiratory Muscle Function in Pulmonary Hypertension. Lung 192, 321–328 (2014). https://doi.org/10.1007/s00408-013-9542-9
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DOI: https://doi.org/10.1007/s00408-013-9542-9