The exercise in pulmonary arterial hypertension (ExPAH) study: A randomized controlled pilot of exercise training and multidisciplinary rehabilitation in pulmonary arterial hypertension.

Pulmonary hypertension (PH) is characterized by progressive dyspnea, fatigue, and reduced exercise capacity. Despite medical treatment, outcomes remain poor. While exercise training is well established in patients with heart failure, it is less established in patients with PH. This single-blind, randomized controlled pilot study examined the feasibility and effect of 12-week outpatient exercise (multidisciplinary rehabilitation or home walking program) on hemodynamics using cardiac magnetic resonance imaging (cMRI) and right heart catheterization (RHC) in patients with pulmonary arterial hypertension (PAH), a subset of PH. Sixteen participants were randomized to either multidisciplinary outpatient rehabilitation or a home walking program for 12 weeks. Primary outcome measures were changes in right ventricular ejection fraction and stroke volume index on cMRI. Secondary outcome measures included hemodynamics on RHC, quality of life (QOL), muscle strength (handgrip and vital capacity) and 6-min walk test. This preliminary, pilot study suggests that outpatient exercise interventions may be associated with improved hemodynamic function (mean pulmonary artery wedge pressure, stroke volume, and stroke volume index), QOL (PH symptoms, depression, and anxiety), and muscular strength (vital capacity and handgrip strength) for people with PAH, but was not adequately powered to make any formal conclusions. However, our outpatient programs were feasible, safe, and acceptable to participants. Future studies are required to further explore the potential hemodynamic benefits of exercise in PAH.

'Tired, afraid, breathless .' An international survey of the exercise experience for people living with pulmonary hypertension.

Patients with pulmonary hypertension are more sedentary than the general population, but attitudes and experiences that may influence their exercise behaviour remain poorly understood. This study identified patterns of behaviour, attitudes towards exercise, barriers and enablers of exercise for people living with pulmonary hypertension. Accessibility of rehabilitation services from a patient perspective was also explored. A voluntary, international survey of people living with pulmonary hypertension was conducted, with mixed quantitative and qualitative data collection. Data from 187 participants in 19 countries were included in the analyses. In total, 52% (95/183) of people with pulmonary hypertension reported that they attempted to engage in regular physical activity. This was less than the proportion who did so prior to diagnosis (61%, 112/184, p = 0.006) and was accompanied by uncertainty and anxiety about exercise. In total, 63% (113/180) of the cohort reported experiencing previous adverse events while exercising, which was associated with a greater likelihood of ongoing exercise concerns and anxiety. Fear, frustration and uncertainty about exercise were noted as common barriers to engaging in exercise with pulmonary hypertension. Other barriers to exercise included intrinsic factors such as debilitating breathlessness and fatigue, and external factors such as cost and access to appropriate services. Most respondents (76%, 128/169) did not have access to a multi-disciplinary rehabilitation service, although an overwhelming majority (92%, 159/172) reported that this would be helpful. Respondents rated education; a supervised, structured exercise programme; and psychology input as the most important components of a multi-disciplinary rehabilitation service for pulmonary hypertension. Health professionals must work together with consumers to co-design rehabilitation services that will facilitate exercise and increased activity for people living with pulmonary hypertension.

Attitudes towards exercise among medical specialists who manage patients with pulmonary hypertension.

Exercise training was not traditionally recommended for patients with pulmonary hypertension. However, recent work has demonstrated that exercise improves endurance and quality-of-life in patients with pulmonary hypertension. Unfortunately, patients with pulmonary hypertension are often sedentary. While some studies have examined patient attitudes to exercise, none have investigated physician perspectives on exercise in patients with pulmonary hypertension. This multinational survey of physicians involved in treating patients with pulmonary hypertension sought to ascertain physician attitudes to exercise and physician-identified barriers and enablers of exercise in this patient population. We collected cross-sectional survey data from a cohort of 280 physicians, including rehabilitation physicians, cardiologists, respiratory physicians and rheumatologists. We found that overall, 86% physicians recommended exercise, in line with current guidelines, although there were differences in the rationale for prescribing exercise and in the type of exercise prescription. Barriers to exercise included patient-related factors, such as patient ill health preventing exercise; poor patient motivation and lack of understanding regarding the benefits of exercise. Systemic barriers included cost/funding issues and limited availability of appropriate services. Perceived enablers of exercise included access to appropriate programmes, provision of education and supportive treating clinicians. Further research is required to identify and implement interventions to promote physical activity in patients with pulmonary hypertension.

Randomised controlled trial examining the effect of an outpatient exercise training programme on haemodynamics and cardiac MR parameters of right ventricular function in patients with pulmonary arterial hypertension: the ExPAH study protocol.

INTRODUCTION: Pulmonary hypertension (PH) is a potentially life-threatening condition characterised by elevated pulmonary artery pressure. Early stage PH patients are often asymptomatic. Disease progression is associated with impairment of right ventricular function and progressive dyspnoea. Current guidelines recommend exercise training (grade IIa, level B). However, many questions remain regarding the mechanisms of improvement, intensity of supervision and optimal frequency, duration and intensity of exercise. This study will assess the effect of an outpatient rehabilitation programme on haemodynamics and cardiac right ventricular function in patients with pulmonary arterial hypertension (PAH), a subgroup of PH. METHODS AND ANALYSIS: This randomised controlled trial involves both a major urban tertiary and smaller regional hospital in New South Wales, Australia. The intervention will compare an outpatient rehabilitation programme with a control group (home exercise programme). Participants will be stable on oral PAH-specific therapy. The primary outcome measure will be right ventricular ejection fraction measured by cardiac MRI. Secondary outcomes will include haemodynamics measured by right heart catheterisation, endurance, functional capacity, health-related quality of life questionnaires and biomarkers of cardiac function and inflammation. ETHICS APPROVAL AND DISSEMINATION: Ethical approval has been granted by St Vincent's Hospital, Sydney (HREC/14/SVH/341). Results of this study will be disseminated through presentation at scientific conferences and in scientific journals. TRIAL REGISTRATION NUMBER: ACTRN12615001041549; pre-results.

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

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 initial 18 months of the first multi-disciplinary regional Pulmonary Arterial Hypertension Clinic in Australia.

OBJECTIVE: To report the initial 18 months experience of the first multi-disciplinary regional Pulmonary Arterial Hypertension (PAH) Clinic in Australia. DESIGN: Prospective cohort study. SETTING: Community setting on the mid-north coast of New South Wales. PARTICIPANTS: A total of 47 patients (mean age±standard error of the mean: 71.8±1.8 years; male/female ratio 13/34). MAIN OUTCOME MEASURES: Diagnosis of PAH, exclusion of other causes of pulmonary hypertension, commencement of PAH-specific pharmacotherapy. RESULTS: Twenty-three (49%) patients were discharged back to their GP with pulmonary hypertension from a combination of ischaemic heart and/or lung disease. Three (6%) patients died from connective tissue disease (CTD)-related PAH with one death (2%) from ischaemic heart disease. Five (11%) patients remain on treatment (n=2, Bosentan for congenital heart disease-related PAH; n=1 Bosentan for CTD-related PAH; and n=1 Bosentan and n=1 Sildenafil for primary PAH). Fifteen (32%) patients have ongoing review for PAH related to CTD (n=11), carcinoid (n=1) and uncertain cause (n=3). CONCLUSION: Patients with CTD-related PAH have a poor prognosis. PAH should be considered in anyone with dyspnoea without obvious features of cardiac or pulmonary disease, especially in the setting of a CTD. Regional population centres are under-resourced with PAH specialty medical services. We have sought to address this by establishment of the first regional multi-disciplinary PAH Clinic in Australia.