Objectives of this European Respiratory Society task force were to summarise current studies, to develop strategies for future research and to increase availability and awareness of exercise training for pulmonary hypertension (PH) patients.An evidence-based approach with clinical expertise of the task force members, based on both literature search and face-to-face meetings was conducted. The statement summarises current knowledge and open questions regarding clinical effects of exercise training in PH, training modalities, implementation strategies and pathophysiological mechanisms.In studies (784 PH patients in total, including six randomised controlled trials, three controlled trials, 10 prospective cohort studies and four meta-analyses), exercise training has been shown to improve exercise capacity, muscular function, quality of life and possibly right ventricular function and pulmonary haemodynamics. Nevertheless, further studies are needed to confirm these data, to investigate the impact on risk profiles and to identify the most advantageous training methodology and underlying pathophysiological mechanisms.As exercise training appears to be effective, cost-efficient and safe, but is scarcely reimbursed, support from healthcare institutions, commissioners of healthcare and research funding institutions is greatly needed. There is a strong need to establish specialised rehabilitation programmes for PH patients to enhance patient access to this treatment intervention.

https://erj.ersjournals.com/content/erj/53/2/1800332.full.pdf

ERS statement on exercise training and rehabilitation in patients with severe chronic pulmonary hypertension.

Background —Exercise training has been shown to improve cardiorespiratory fitness, physical capacity and quality of life in patients with cardiopulmonary conditions such as heart failure and COPD. However, its role in management of pulmonary hypertension is not well defined. In this study, we aim to evaluate the efficacy and safety of exercise training in patients with pulmonary hypertension. 

Methods and Results —We included all prospective intervention studies that evaluated the efficacy and safety of exercise training in patients with pulmonary hypertension. Primary outcome of this meta-analysis was a change in six-minute walk distance (6MWD). We also assessed the effect of exercise on peak oxygen uptake (VO2peak), resting pulmonary arterial systolic pressure (PASP), peak exercise heart rate (HRpeak), and quality of life. A total of 16 studies with 434 exercise-training participants were included. In the pooled analysis, exercise training was associated with significant improvement in 6MWD [Weighted mean difference (WMD): 57.7 meters (95% CI: 42.5 to 72.8)], VO2peak [WMD = 1.7 ml/kg/min (95% CI: 1.3 to 2.0)], PASP [WMD = -3.6 mmHg (95% CI = -5.8 to -1.4)], HRpeak [WMD = 10.4 beats per min (95% CI: 5.5 to 15.3)], and quality of life as measured on SF-36 questionnaire subscale scores. Furthermore, exercise training was well tolerated with a low dropout rate and no major adverse events related to exercise training. 

Conclusions —Exercise training in patients with pulmonary hypertension appears safe and is associated with a significant improvement in exercise capacity, pulmonary arterial pressure and quality of life.

Efficacy and Safety of Exercise Training in Chronic Pulmonary Hypertension Systematic Review and Meta-Analysis

Pulmonary hypertension (PH) is characterized by severe exercise limitation mainly attributed to the impairment of right ventricular function resulting from a concomitant elevation of pulmonary vascular resistance and pressure. The unquestioned cornerstone in the management of patients with pulmonary arterial hypertension (PAH) is specific vasoactive medical therapy to improve pulmonary hemodynamics and strengthen right ventricular function. Nevertheless, evidence for a beneficial effect of exercise training (ET) on pulmonary hemodynamics and functional capacity in patients with PH has been growing during the past decade. Beneficial effects of ET on regulating factors, inflammation, and metabolism have also been described. Small case-control studies and randomized clinical trials in larger populations of patients with PH demonstrated substantial improvements in functional capacity after ET. These findings were accompanied by several studies that suggested an effect of ET on inflammation, although a direct link between this effect and the therapeutic benefit of ET in PH has not yet been demonstrated. On this background, the aim of the present review is to describe current concepts regarding the effects of exercise on the pulmonary circulation and pathophysiological limitations, as well as the clinical and mechanistic effects of exercise in patients with PH.

https://journals.sagepub.com/doi/pdf/10.1086/690553

Effects of exercise training on pulmonary hemodynamics, functional capacity and inflammation in pulmonary hypertension.

Pulmonary hypertension (PH) is a clinical condition characterised by raised pulmonary artery pressure, which results in increased right ventricular afterload and dyspnoea. This is accompanied by reduced exercise capacity, quality of life and, eventually, death. An increasing range of targeted medications has transformed the treatment of pulmonary arterial hypertension, a specific type of PH. Supervised exercise training is recommended as part of a multifaceted management plan for PH. However, many questions remain regarding how exercise training improves exercise capacity and quality of life. The optimal exercise regimen (frequency, timing, duration and intensity) also remains unclear. This review provides an update on the pathophysiology of exercise impairment in PH, suggests mechanisms by which exercise may improve symptoms and function and offers evidence-based recommendations regarding the frequency and intensity of an exercise programme for patients with PH.

The benefit of exercise training in pulmonary hypertension: a clinical review.

Exercise training in patients with pulmonary and systemic hypertension: A unique therapy for two different diseases

Background: The purpose of this study was to examine the effect of an integrated outpatient training program on exercise capacity and health-related quality of life (HRQOL) in patients with pulmonary hypertension (PH). Methods: 17 patients of our pulmonary hypertension program (World Health Organisation clinical classification system “Dana Point 2008” group 1 n=14, group 4 n=3, age 61±13 years, 11 female [65%], body mass index [BMI] 26.7±5.9) on stable disease-targeted medication attended a supervised integrated outpatient training program including breathing exercises, education, strength and endurance training for 1.5 hours once a month for 10 consecutive months. Patients` exercise capacity was evaluated performing a 6 minute walk test (6MWT), and their quality of life was assessed by the standardized “Short Form 36 Health Survey” and the “Cambridge Pulmonary Hypertension Outcome Review” (CAMPHOR), before and after the program. Data analysis was performed using the paired T-test and Spearman`s rank order correlation. Results: All patients completed the outpatient training while medication remained unchanged during the observational period. At baseline 6-minute walk distance was 383 ± 91 m and after 10 months 391 ± 85 m (p=0.157). Both, HRQOL questionnaires, SF-36 and CAMPHOR, tended to improve with a significant signal only in the CAMPHOR activity core (p < 0.023), indicating improved physical capabilities. During the 10 months observation period no adverse events occurred in the study group. Conclusion: Our data suggest that an integrated outpatient training is safe and may be beneficial as an adjuvant therapeutic option. The correlation between 6MWD and physical domains of HRQOL questionnaires demonstrate the consistency of our data. The best approach for a structured outpatient training for PH patients merits further investigation.

https://www.longdom.org/open-access/an-integrated-outpatient-training-program-for-patients-with-pulmonary-hypertension-the-munich-pilot-project-2329-9096.1000204.pdf

An Integrated Outpatient Training Program for Patients with Pulmonary Hypertension - the Munich Pilot Project

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An Integrated Outpatient Training Program for Patients with Pulmonary Hypertension - the Munich Pilot Project