Rehabilitation in patients with pulmonary arterial hypertension

Abstract

Exertional dyspnoea is a leading symptom in patients with pulmonary arterial hypertension (PAH). Patients suffering from PAH report poor quality of life, have skeletal muscle dysfunction and in the absence of advanced medical therapy deteriorate progressively due to right heart failure which can lead to death. For decades, patients with PAH were advised to avoid exercise in fear of exacerbated right heart failure. Recently, it has been shown that a highly supervised rehabilitation programme in expert centres leads to significant improvements in symptoms, quality of life, exercise capacity and may even enhance haemodynamics in selected stable patients treated with advanced regimens of PAH-targeted drugs. As a consequence of these promising results, pulmonary rehabilitation performed in an expert centre has been included in recent guidelines. The underlying mechanisms are not completely understood, but positive effects can be measured in different organ systems such as skeletal muscles, the cardiopulmonary system and immune system (inflammation), and also on the psychological level. Thus, improvements in 6-minute walking distance (6MWD), peak oxygen uptake (VO2 peak), muscle strength and muscle endurance, as well as physical and mental quality of life scores (SF-36 questionnaire) have been shown. Different training protocols have been used. Essential are qualified patient selection in expert centres, a low workload endurance and dumbbell (weight lifting) training avoiding strenuous exercise and exhaustion, thorough patient education and close supervision by experts especially during the first weeks. Adverse events may occur (e.g., pre-/syncope, arrhythmia, respiratory infections). PAH patients tend to overestimate their physical capacity, not perceiving their own limits properly, which makes education and expert advice even more important as exercise training can also worsen the right heart failure. Therefore, a core issue of the multidisciplinary rehabilitation is the close cooperation between the experienced rehabilitation clinic offering a specialised programme for PAH patients and the PAH expert centre, which takes care of the patient and is thoroughly involved in the training programme. Further multicentre international randomised trials are needed to evaluate whether this specialised programme is feasible within different healthcare systems and to assess long term effects and survival.

Risk stratification and medical therapy of pulmonary arterial hypertension

“…Physically deconditioned patients who are stable on targeted medication are also advised to undertake supervised exercise training. Recently, two further RCTs and meta-analyses have been published in this regard, confirming the positive effect of training in PAH [77, 78].…”

The burden of comorbidities in pulmonary arterial hypertension

“…A multidisciplinary team and network are therefore important when patients with PAH are also being treated for comorbidities. Patients with PAH and comorbidities may require different aspects of care including cardiac exercise rehabilitation (for peripheral artery disease, heart failure, myocardial infarction, and atrial fibrillation) and surgical interventions, in addition to multiple pharmacological therapies 32–37 . Patients may also require additional support in their daily lives from social workers, occupational therapists, and psychotherapists.…”

Muscle training in patients with pulmonary hypertension. a narrative review

“…For the upper extremities, dumbbell workouts with low weight (500-1000 g) have been reported, focused on muscles independently 51 , 52 ; for 30 minutes, five times a week during 12 weeks; this could be due to the intention of carrying out safe activities that avoid as much as possible the appearance of complications related to musculoskeletal alterations and also to the heart-lung interaction 53 .…”

“… Upper Limbs Dumbbell workouts Low weight (500 - 1000 gr) have been reported, focused on muscles independently for 30 minutes, five times a week during 12 weeks. Making increments according to the patient's response 4 , 51 , 53 …”