Effects of a Rehabilitation Program on Skeletal Muscle Function in Idiopathic Pulmonary Arterial Hypertension

Mainguy, Vincent; Maltais, François; Saey, Didier; Gagnon, Philippe; Martel, Simon; Simon, Mathieu; Provencher, Steeve

doi:10.1097/hcr.0b013e3181d6f962

Cited by 93 publications

(114 citation statements)

References 15 publications

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“…These improvements were in line with the increase in quadriceps muscle endurance [72]. Others found a reduction in fast-twitch muscle fibres and a potential increase in capillary density in the quadriceps of PAH patients after ExT [73].…”

Section: Improving Endurance Capacity Exercise Trainingsupporting

confidence: 59%

The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle

et al. 2015

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Pulmonary arterial hypertension (PAH) is a fatal lung disease characterised by progressive remodelling of the small pulmonary vessels. The daily-life activities of patients with PAH are severely limited by exertional fatigue and dyspnoea. Typically, these symptoms have been explained by right heart failure. However, an increasing number of studies reveal that the impact of the PAH reaches further than the pulmonary circulation. Striated muscles other than the right ventricle are affected in PAH, such as the left ventricle, the diaphragm and peripheral skeletal muscles. Alterations in these striated muscles are associated with exercise intolerance and reduced quality of life. In this Back to Basics article on striated muscle function in PAH, we provide insight into the pathophysiological mechanisms causing muscle dysfunction in PAH and discuss potential new therapeutic strategies to restore muscle dysfunction. @ERSpublications RV, LV, diaphragm and peripheral muscle dysfunction contributes to reduced quality of life in PAH patients

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Section: Improving Endurance Capacity Exercise Trainingsupporting

confidence: 59%

The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle

et al. 2015

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“…a 100-s or 33% improvement was reached in 30 (91%) out of 33 and 27 (82%) out of 33 investigations, respectively) (figure 1). tLIM is also responsive to rehabilitation in idiopathic PAH [121,122] and IPF [123,124]. In one observational study of 53 IPF patients, the effect size for tLIM after rehabilitation was larger than for V′O 2 peak, WRpeak, 6MWD and ISWT [123] (table 3).…”

Section: Discussionmentioning

confidence: 99%

“…Isotime V′O 2 , V′CO 2 and V′E, as well as cardiac output and [La − ] a and other variables are responsive to a number of interventions in COPD [25,62,98,99,120,[128][129][130][131] and in some other conditions such as cystic fibrosis [132] and PAH [122,133]. Isotime comparisons are also responsive outcomes of interventions directed to train arm muscles [134][135][136].…”

Section: Evaluativementioning

confidence: 98%

Use of exercise testing in the evaluation of interventional efficacy: an official ERS statement

et al. 2016

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This document reviews 1) the measurement properties of commonly used exercise tests in patients with chronic respiratory diseases and 2) published studies on their utilty and/or evaluation obtained from MEDLINE and Cochrane Library searches between 1990 and March 2015.Exercise tests are reliable and consistently responsive to rehabilitative and pharmacological interventions. Thresholds for clinically important changes in performance are available for several tests. In pulmonary arterial hypertension, the 6-min walk test (6MWT), peak oxygen uptake and ventilation/carbon dioxide output indices appear to be the variables most responsive to vasodilators. While bronchodilators do not always show clinically relevant effects in chronic obstructive pulmonary disease, high-intensity constant work-rate (endurance) tests (CWRET) are considerably more responsive than incremental exercise tests and 6MWTs. High-intensity CWRETs need to be standardised to reduce interindividual variability. Additional physiological information and responsiveness can be obtained from isotime measurements, particularly of inspiratory capacity and dyspnoea. Less evidence is available for the endurance shuttle walk test. Although the incremental shuttle walk test and 6MWT are reliable and less expensive than cardiopulmonary exercise testing, two repetitions are needed at baseline. All exercise tests are safe when recommended precautions are followed, with evidence suggesting that no test is safer than others. @ERSpublications A review of exercise testing to evaluate interventions aimed to improve exercise tolerance in respiratory patients

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“…1 Previously, expert recommendations included avoidance of any exertion or activity, including pulmonary rehabilitation (PR), but there are now sufficient studies demonstrating the benefits of PR in this population. [2][3][4][5][6][7][8] These studies have demonstrated the presence of myopathic changes in skeletal musculature that are responsive to training. 3,9 They have also demonstrated meaningful outcomes in physical activity as measured by the sixminute walk test (6MWT) 4,6 as well as improvement in QOL scores postpulmonary rehabilitation.…”

Section: Introductionmentioning

confidence: 99%

“…[2][3][4][5][6][7][8] These studies have demonstrated the presence of myopathic changes in skeletal musculature that are responsive to training. 3,9 They have also demonstrated meaningful outcomes in physical activity as measured by the sixminute walk test (6MWT) 4,6 as well as improvement in QOL scores postpulmonary rehabilitation. 2,5,10 The 6MWT is a part of the evaluation of patients with chronic respiratory disease in general and has been used as a part of the evaluation of pharmacotherapy in PH.…”

Section: Introductionmentioning

confidence: 99%

A retrospective study on the effects of pulmonary rehabilitation in patients with pulmonary hypertension

et al. 2014

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A retrospective analysis of 23 patients was undertaken to evaluate the outcome of pulmonary rehabilitation (PR) in patients with pulmonary hypertension (PH) over a preceding 6-year time frame. Chart review and data analyses were undertaken evaluating pulmonary arterial hypertension pharmacotherapy versus the same therapy with the addition of PR. Analysis included 23 patients who had a mean pulmonary artery pressure of 36.6 while on therapy and who had initial six-minute walk tests (6MWTs) ranging between 54 and 396 meters. Outcomes included 6MWTs and Saint George's Respiratory Questionnaire. There was no improvement in 6MWT postpharmacotherapy. Patients with low 6MWT <250 meters had substantial gains in 6MWT postrehabilitation (average of 86 meters). As well, those with initial 6MWT >250 meters had a significant improvement of 52.55 meters, documenting the utility of PR in patients with PH. PH patients are increasingly seen in PR clinics as they share many characteristics present in chronic respiratory disease states. There is increasing literature supporting the utility of PR in this population. We have found that patients with 6MWTs as low as <250 meters are also candidates for PR and can demonstrate substantial benefits as measured using the 6MWTs.

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Effects of a Rehabilitation Program on Skeletal Muscle Function in Idiopathic Pulmonary Arterial Hypertension

Abstract: Peripheral muscle characteristic improvements may contribute to the clinical benefit observed following a rehabilitation program in IPAH.

Cited by 93 publications

References 15 publications

The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle

The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle

Use of exercise testing in the evaluation of interventional efficacy: an official ERS statement

A retrospective study on the effects of pulmonary rehabilitation in patients with pulmonary hypertension

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