Pulmonary Arterial Hypertension Clinical Trial
Official title:
Home-based Exercise Training in Patients With Pulmonary Arterial Hypertension: Effect on Skeletal Muscular Function and Metabolism
Pulmonary Arterial Hypertension has gone from a disease that causes rapid death to a more
chronic condition. Yet, improved survival is associated with major challenges for clinicians
as most patients remain with poor quality of life and limited exercise capacity. The effects
of exercise training on exercise capacity have been largely evaluated and showed an
improvement in 6-minutes walking distance (6MWD), peak V'O2. It is also known that exercise
program improves quality of life. Maximal volitional and nonvolitional strength of the
quadriceps are reduced in patients with Pulmonary Arterial Hypertension and correlated to
exercise capacity. Moreover, on the cellular level, alterations are observed in both the
respiratory as well as the peripheral muscles. Muscle fiber size has been reported to be
decreased in some studies or conversely unaltered in human and animal models. Reduction in
type I fibers and a more anaerobic energy metabolism has also been reported, but not in all
studies. Likewise, a loss in capillary density in quadriceps of patients with Pulmonary
Arterial Hypertension and rats has been reported, but could not be confirmed in other
studies. While the impact of exercise training on clinical outcomes such as exercise capacity
or quality of life is well known, this data highlight the fact that the underlying causes of
peripheral muscle weakness as well as the mechanisms underlying the clinical improvements
observed with exercise programs are not completely understood. Improvement of muscle cell
metabolism in part via the enhancement of oxidative cellular metabolism and decrease in
intracellular lipid accumulation may play a role in improving muscle function and exercise
capacity.
In this study, we intend to evaluate the impact of a 12 weeks home-based rehabilitation
program on peripheral muscle function and metabolism, focusing on lipid infiltration,
oxidative metabolism and epigenetic factors that can be involved in metabolic syndrome, in
patients with Pulmonary Arterial Hypertension.
The 12 weeks home-based rehabilitation program is detailed as follows:
- 1st sessions at the hospital, in the presence of a physiotherapist/kinesiologist
- 3 weeks of supervised home-based rehabilitation (using a telemonitoring system) 3 times
a weeks
- 9 weeks of unsupervised home-based rehabilitation (one phone call a week)
Patients will be evaluated at baseline and at endpoint (12 weeks)
;
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