Background-Pulmonary hypertension (PH) is associated with restricted physical capacity, limited quality of life, and a poor prognosis because of right heart failure. The present study is the first prospective randomized study to evaluate the effects of exercise and respiratory training in patients with severe symptomatic PH. Methods and Results-Thirty patients with PH (21 women; mean age, 50Ϯ13 years; mean pulmonary artery pressure, 50Ϯ15 mm Hg; mean World Health Organization [WHO] class, 2.9Ϯ0.5; pulmonary arterial hypertension, nϭ23; chronic thromboembolic PH, nϭ7) on stable disease-targeted medication were randomly assigned to a control (nϭ15) and a primary training (nϭ15) group. Medication remained unchanged during the study period. Primary end points were the changes from baseline to week 15 in the distance walked in 6 minutes and in scores of the Short Form Health Survey quality-of-life questionnaire. Changes in WHO functional class, Borg scale, and parameters of echocardiography and gas exchange also were assessed. At week 15, patients in the primary and secondary training groups had an improved 6-minute walking distance; the mean difference between the control and the primary training group was 111 m (95% confidence interval, 65 to 139 m; PϽ0.001). Exercise training was well tolerated and improved scores of quality of life, WHO functional class, peak oxygen consumption, oxygen consumption at the anaerobic threshold, and achieved workload. Systolic pulmonary artery pressure values at rest did not change significantly after 15 weeks of exercise and respiratory training (from 61Ϯ18 to 54Ϯ18 mm Hg) within the training group. Conclusions-This study indicates that respiratory and physical training could be a promising adjunct to medical treatment in severe PH. The effects add to the beneficial results of modern medical treatment.

After introduction of the new international guidelines on idiopathic pulmonary fibrosis (IPF) in 2011, we investigated clinical management practices for patients with IPF according to physicians' diagnoses.A prospective, multicenter, noninterventional study with comprehensive quality measures including on-site source data verification was performed in Germany.502 consecutive patients (171 newly diagnosed, 331 prevalent; mean±sd age 68.7±9.4 years, 77.9% males) with a mean disease duration of 2.3±3.5 years were enrolled. IPF diagnosis was based on clinical assessments and high-resolution computed tomography (HRCT) in 90.2%, and on surgical lung biopsy combined with histology in 34.1% (lavage in 61.8%). The median 6-min walk distance was 320 m (mean 268±200 m). The mean forced vital capacity was 72±20% pred and diffusing capacity of the lung for carbon monoxide was 35±15% pred. No drugs were administered in 17.9%, oral steroids in 23.7%, N-acetylcysteine in 33.7%, pirfenidone in 44.2% and other drugs in 4.6% of patients. Only 2.8% of the cohort was listed for lung transplantation.IPF patients were diagnosed in line with the new guidelines. They had more severe disease than those enrolled in recent randomised controlled trials. In addition to HRCT, the frequency of lung biopsies was surprisingly high. Treatment patterns varied substantially.

Background: Even though specific agents for the treatment of patients with pulmonary hypertension (PH) are available, in PH patients, physical capacity and quality of life (QoL) are often restricted and survival is reduced. Objectives: This study prospectively investigated the long-term effects of respiratory and exercise training in patients with severe chronic PH regarding safety, time to clinical worsening and survival. Methods: Fifty-eight consecutive patients with severe PH on stable disease-targeted medication received exercise and respiratory training in hospital for 3 weeks and continued at home. They were prospectively followed for 24 ± 12 months. Primary endpoints were time to clinical worsening and survival. Adverse events and changes in the 6-min walking test, QoL, WHO functional class and gas exchange were secondary endpoints and were evaluated at baseline and at weeks 3 and 15. Results: All patients tolerated the exercise training well without severe adverse events. In week 15, 6-min walking test results were significantly improved compared to baseline (by 84 ± 49 m, p < 0.001), as well as QoL scores, WHO functional class (from 2.9 ± 0.5 to 2.6 ± 0.6, p < 0.01), peak oxygen consumption (from 12.5 ± 3.0 to 14.6 ± 3.9 ml/min/kg, p < 0.001), heart rate at rest (from 75 ± 12 to 61 ± 18 beats/min, p < 0.001) and maximal workload (from 65 ± 21 to 80 ± 25 W, p < 0.001). Survival at 1 and 2 years was 100 and 95%, respectively. Fifteen events occurred during the follow-up. Conclusion: This study indicates that exercise and respiratory training as add-on to medical treatment may improve exercise capacity and QoL, and that they have a good long-term safety in the described setting.

Idiopathic pulmonary arterial hypertension (IPAH) is a pulmonary vasculopathy of unknown aetiology. Dyspnoea, peripheral airway obstruction and inefficient ventilation are common in IPAH. Data on respiratory muscle function are lacking.This prospective single-centre study included 26 female and 11 male patients with IPAH in World Health Organization functional classes II-IV. Mean¡SD pulmonary artery pressure was 48.6¡16.9 in females and 53.1¡22.9 mmHg in males; cardiac output was 3.7¡1.3 and 4.2¡1.7 L?min -1 .Maximal inspiratory pressure (PI,max) was lower in the female patients than in 20 controls (5.3¡2.0 versus 8.2¡2.0 kPa). In the male patients, PI,max was lower than in 25 controls (6.8¡2.2 versus 10.5¡3.7 kPa). Maximal expiratory pressure (PE,max) was lower in the female patients than in controls (6.2¡2.6 versus 9.5¡2.1 kPa), and in male patients as compared to controls (7.1¡1.6 versus 10.3¡3.9 kPa). There was no correlation between PI,max or PE,max and parameters of pulmonary haemodynamics or exercise testing. The ratio of mouth occlusion pressure within the first 0.1 s of inspiration and PI,max was higher in IPAH than in controls (females 0.067¡0.066 versus 0.021¡0.008; males 0.047¡0.061 versus 0.023¡0.016).In conclusion, this study provides the first evidence of inspiratory and expiratory muscle weakness in idiopathic pulmonary arterial hypertension. The pathomechanisms and the prognostic significance should be further investigated.KEYWORDS: Exercise test, pulmonary hypertension, respiratory function test, respiratory muscles I diopathic pulmonary arterial hypertension (IPAH) is defined as a pulmonary vasculopathy of unknown aetiology, predominantly affecting females [1]. Minute ventilation (V9E) is increased at rest and during exercise [2]. The ventilatory response to exercise in IPAH patients, i.e. the ratio of V9E to carbon dioxide production (V9CO 2 ) during exercise (V9E/V9CO 2 slope), is also increased and is a predictor of poor prognosis [2,3]. The increased V9E/V9CO 2 slope indicates inefficient ventilation in IPAH, which can partly be attributed to pulmonary-perfusion abnormalities [2]. It has been shown by the current authors' group and others that, in congestive left ventricular failure, inefficient ventilation is associated with reduced inspiratory muscle strength [4][5][6]. Moreover, inspiratory muscle function has recently been identified as a prognostic predictor in congestive left ventricular failure [7]. Risk stratification of patients with congestive left ventricular failure is improved by using the combination of maximal inspiratory pressure (PI,max) and peak oxygen consumption (V9O 2 ) [7].Inspiratory and expiratory muscle strength have not been studied in IPAH. Therefore, this prospective study investigated respiratory muscle function and the mouth occlusion pressures in 37 patients with IPAH. PI,max and maximal expiratory pressure (PE,max) were prospectively assessed in IPAH patients with varying degrees of disease and exercise limitation. Moreover, invasive (right heart c...

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In patients with CHF, inspiratory muscle strength is reduced and emerges as a novel, independent predictor of prognosis. Because testing for Pi(max) is simple in clinical practice, it might serve as an additional factor to improve risk stratification and patient selection for cardiac transplantation.

Background: As there is controversy about changes in lung function in primary pulmonary hypertension (PPH), lung mechanics were assessed with a focus on expiratory airflow in relation to pulmonary haemodynamics. Methods: A cross sectional study was performed in 64 controls and 171 patients with PPH (117 women) of mean (SD) age 45 (13) years, pulmonary artery pressure (PAPmean) 57 (15) mm Hg, and pulmonary vascular resistance 1371 (644) dyne.s/cm

Background: To evaluate the sensitivity, specificity and predictive values of spirometry for the diagnosis of chronic obstructive pulmonary disease (COPD) and asthma in patients suspected of suffering from an obstructive airway disease (OAD) in primary care.