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.

Aims: This prospective, randomized, controlled, multicentre study aimed to evaluate efficacy and safety of exercise training in patients with pulmonary arterial (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods and results: For the first time a specialized PAH/CTEPH rehabilitation programme was implemented in 11 centres across 10 European countries. Out of 129 enrolled patients, 116 patients (58 vs. 58 randomized into a training or usual care control group) on disease-targeted medication completed the study [85 female; mean age 53.6 ± 12.5 years; mean pulmonary arterial pressure 46.6 ± 15.1 mmHg; World Health Organization (WHO) functional class II 53%, III 46%; PAH n = 98; CTEPH n = 18]. Patients of the training group performed a standardized in-hospital rehabilitation with mean duration of 25 days [95% confidence interval (CI) 17–33 days], which was continued at home. The primary endpoint, change of 6-min walking distance, significantly improved by 34.1 ± 8.3 m in the training compared with the control group (95% CI, 18–51 m; P < 0.0001). Exercise training was feasible, safe, and well-tolerated. Secondary endpoints showed improvements in quality of life (short-form health survey 36 mental health 7.3 ± 2.5, P = 0.004), WHO-functional class (training vs. control: improvement 9:1, worsening 4:3; χ2 P = 0.027) and peak oxygen consumption (0.9 ± 0.5 mL/min/kg, P = 0.048) compared with the control group. Conclusion: This is the first multicentre and so far the largest randomized, controlled study on feasibility, safety, and efficacy of exercise training as add-on to medical therapy in PAH and CTEPH. Within this study, a standardized specialized training programme with in-hospital start was successfully established in 10 European countries.

The purpose of the current trial was to test the hypothesis that breathing oxygen-enriched air increases exercise performance of patients with pulmonary arterial or chronic thrombo-embolic pulmonary hypertension (PAH/CTEPH) and to investigate involved mechanisms. Twenty-two patients with PAH/CTEPH, eight women, means ± SD 61 ± 14 years, resting mPAP 35 ± 9mmHg, PaO₂ ambient air >7.3 kPa, underwent four bicycle ergospirometries to exhaustion on different days, while breathing oxygen-enriched (FiO₂ 0.50, hyperoxia) or ambient air (FiO₂ 0.21, normoxia) using progressively increased or constant load protocols (with 75% maximal work rate under FiO₂ 0.21), according to a randomized, sham-controlled, single-blind, cross-over design. ECG, pulmonary gas-exchange, arterial blood gases, cerebral and quadriceps muscle tissue oxygenation (CTO and QMTO) by near-infrared spectroscopy were measured. In ramp exercise, maximal work rate increased from 113 ± 38 W with normoxia to 132 ± 48 W with hyperoxia, mean difference 19.7 (95% CI 10.5–28.9) W, P < 0.001. Constant load exercise endurance increased from 571 ± 443 to 1242 ± 514 s, mean difference 671 (95% CI 392–951) s, P < 0.001. At end-exercise with hyperoxia PaO₂, CTO, QMTO, and PaCO₂ were increased, and ventilatory equivalents for CO₂ were reduced while the physiological dead space/tidal volume ratio remained unchanged. In patients with PAH/CTEPH, breathing oxygen-enriched air provides major increases in exercise performance. This is related to an improved arterial oxygenation that promotes oxygen availability in muscles and brain and to a reduction of the excessive ventilatory response to exercise thereby enhancing ventilatory efficiency. Patients with PAH/CTEPH may therefore benefit from oxygen therapy during daily physical activities and training. clinicaltrials.gov Identifier: NCT01748474.

The aim of the present study was to investigate the prognostic value of exercise haemodynamics measured during right heart catheterisation (RHC) in patients with systemic sclerosis (SSc) referred for evaluation of pulmonary hypertension.SSc patients undergoing RHC at rest and during maximal supine incremental cycle exercise were grouped into resting precapillary pulmonary hypertension (PH_rest) (mean pulmonary artery pressure (mPAP) ≥25 mmHg, pulmonary artery wedge pressure 3 mmHg·L⁻¹·min⁻¹at maximal exercise), and without pulmonary hypertension (PH_none). Patients' characteristics, haemodynamics and follow up data were compared between groups.72 SSc patients were followed for median (interquartile range) 33 (15–55) months. Mean (95% CI) survival without transplantation estimated by Kaplan-Meyer analysis was 4.4 (0.8–2.9) years in PH_rest(n=17), 5.2 (4.4–6.1) years in PH_ex(n=28) and 9.5(8.4–10.6) years in PH_none(n=27; p<0.05versusothers). In Cox regression models, the exercise-induced increase in mPAP (hazard ratio (HR) 1.097, 95% CI 1.002–1.200) and the coefficient of pulmonary vascular distensibility alpha (HR 0.100, 95% CI 0.012–0.871) controlled for age, but not resting haemodynamics predicted transplant-free survival.Among SSc patients with normal mPAP at rest, an excessive increase in mPAP during exercise and an impaired vascular distensibility may indicate an early stage of pulmonary vasculopathy, associated with reduced survival similar to resting pulmonary hypertension patients.

The question addressed by the study: Chronic exposure to hypoxia increases pulmonary artery pressure (PAP) in highlanders, but the criteria for diagnosis of high-altitude pulmonary hypertension (HAPH) are debated. We assessed cardiac function and PAP in highlanders at 3250 m and explored HAPH prevalence using different definitions.Patients and methods: Central Asian highlanders free of overt cardiorespiratory disease, permanently living at 2500–3500 m compared to age-matched lowlanders living <800 m. Participants underwent echocardiography close to their altitude of residence (at 3250 mversus760 m).Results: 173 participants (97 highlanders, 76 lowlanders), mean±sdage 49±9 years (49% females) completed the study. Results in lowlandersversushighlanders were systolic PAP (23±5versus30±10 mmHg), right ventricular fractional area change (42±6%versus39±8%), tricuspid annular plane systolic excursion (2.1±0.3versus2.0±0.3 cm), right atrial volume index (20±6versus23±8 mL·m⁻²), left ventricular ejection fraction (62±4%versus57±5%) and stroke volume (64±10versus57±11 mL); all between-group comparisons p<0.05. Depending on criteria, HAPH prevalence varied between 6% and 35%.The answer to the question: Chronic exposure to hypoxia in highlanders is associated with higher PAP and slight alterations in right and left heart function compared to lowlanders. The prevalence of HAPH in this large highlander cohort varies between 6% according to expert consensus definition of chronic high-altitude disease to 35% according to the most recent definition of pulmonary hypertension proposed for lowlanders.