To describe the intra-rater and inter-rater reliability of the Brazilian Portuguese version of the fatigue severity scale (FSS) in patients with COPD and to identify the presence of its association with parameters of pulmonary function, dyspnea, and functional capacity. Methods: This was an observational cross-sectional study involving 50 patients with COPD, who completed the FSS in interviews with two researchers in two visits. The FSS scores were correlated with those of the Medical Research Council (MRC) scale, as well as with FEV1, FVC, and six-minute walk distance (6MWD). Results: The mean age of the patients was 69.4 ± 8.23 years, whereas the mean FEV1 was 46.5 ± 20.4% of the predicted value. The scale was reliable, with an intraclass correlation coefficient of 0.90 (95% CI, 0.81-0.94; p < 0.01). The FSS scores showed significant correlations with those of MRC scale (r = 0.70; p < 0.01), as well as with 6MWD (r = −0.77; p < 0.01), FEV1 (r = −0.38; p < 0.01), FVC (r = −0.35; p < 0.01), and stage of the disease in accordance with the Global Initiative for Chronic Obstructive Lung Disease criteria (r = 0.37; p < 0.01). Conclusions: The Brazilian Portuguese version of the FSS proved reliable for use in COPD patients in Brazil and showed significant correlations with sensation of dyspnea, functional capacity, pulmonary function, and stage of the disease.

Keywords: Fatigue; Pulmonary disease, chronic obstructive; Reproducibility of results; Validation studies.

Objective: To characterize a population of patients with bronchiectasis, correlating clinical, radiological, and functional aspects with the severity of dyspnea. Methods: This was a cross-sectional study involving adult patients with HRCT-confirmed bronchiectasis, categorized according to the severity of dyspnea (as being mildly or severely symptomatic, on the basis of the modified Medical Research Council scale). We correlated the severity of dyspnea with clinical parameters, functional parameters (spirometry values, lung volumes, and DLCO), and CT parameters. Results: We evaluated 114 patients, 47 (41%) of whom were men. The median age (interquartile range) was 42 years (30-55 years). The most common form was idiopathic bronchiectasis. Of the 114 patients, 20 (17.5%) were colonized with Pseudomonas aeruginosa and 59 (51.8%) were under continuous treatment with macrolides. When we applied the Exacerbation in the previous year, FEV1, Age, Colonization, Extension, and Dyspnea score, the severity of dyspnea was categorized as moderate in 54 patients (47.4%), whereas it was categorized as mild in 50 (43.9%) when we applied the Bronchiectasis Severity Index. The most common lung function pattern was one of obstruction, seen in 95 patients (83.3%), and air trapping was seen in 77 patients (68.7%). The prevalence of an obstructive pattern on spirometry was higher among the patients with dyspnea that was more severe, and most functional parameters showed reasonable accuracy in discriminating between levels of dyspnea severity. Conclusions: Patients with bronchiectasis and dyspnea that was more severe had greater functional impairment. The measurement of lung volumes complemented the spirometry data. Because bronchiectasis is a complex, heterogeneous condition, a single variable does not seem to be sufficient to provide an overall characterization of the clinical condition.

Keywords: Bronchiectasis; Dyspnea; Respiratory function tests; Multidetector computed tomography; Plethysmography.

Abstract Objective: To determine the validity and reproducibility of two subjective instruments to assess limitations in activities of daily living (ADLs) in patients with chronic obstructive pulmonary disease (COPD) in Brazil: the Pulmonary Functional Status and Dyspnea ­Questionnaire ‑ ­Modified version (PFSDQ-M) and the Medical Research Council (MRC) scale. Methods: Thirty patients with COPD (age, 67 ± 10 years; males, 17; forced expiratory volume in one second, 42% ± 13% of predicted) completed the Portuguese-language versions of the two instruments on two occasions, one week apart. The PFSDQ-M has three components: influence of dyspnea on ADLs, influence of fatigue on ADLs and change experienced by the patient with ADLs. The MRC scale is simple, with only five items, in which patients report the degree to which dyspnea limits their performance of ADLs. The traditional Saint George's Respiratory Questionnaire (SGRQ), previously validated for use in Brazil, was used as a validation criterion. Results: The test-retest reliability (intraclass correlation coefficient) of the PFSDQ-M was 0.93, 0.92 and 0.90 for the dyspnea, fatigue and change components, respectively, compared with 0.83 for the MRC scale. Bland-Altman plots showed good test-retest concordance for the PFSDQ-M. The components of the PFSDQ-M and the MRC scale correlated significantly with all of the domains and the total score on the SGRQ (0.49 < r < 0.80; p < 0.01 for all). Conclusions: The Portuguese-language versions of the PFSDQ-M and the MRC scale proved reproducible and valid for use in patients with COPD in Brazil.

Keywords: Pulmonary disease, chronic obstructive; Activities of daily living; Validation studies.

Objective: To translate the London Chest Activity of Daily Living (LCADL) scale into Portuguese and to determine whether this version is reproducible in Brazilian patients with severe chronic obstructive pulmonary disease (COPD). Methods: The LCADL scale was translated into Portuguese and then back-translated into English. This pilot Brazilian Portuguese version was administered to 8 patients with COPD, and possible text-related problems were investigated. The principal problems were discussed with the authors of the original scale, and a final translated version was arrived at. At the study outset, two observers administered this final version (twice in one day) to 31 patients with COPD. One of those observers again administered the scale to the same patients 15-20 days later. At baseline, the patients were submitted to pulmonary function testing and to the six-minute walk test (6MWT). Results: The Brazilian Portuguese version of the LCADL scale demonstrated excellent reproducibility in the total score and in most of the questions, with an inter-rater Cronbach's alpha coefficient of 0.97 (95% CI: 0.89-0.97; p < 0.01) and an intra-rater Cronbach's alpha coefficient of 0.96 (95% CI: 0.83-0.96; p < 0.01). The total score presented a negative correlation with forced expiratory volume in one second in liters (r = −0.49; p < 0.05) and with distance covered on the 6MWT (r = −0.56; p < 0.05). Conclusion: The Brazilian Portuguese version of the LCADL scale is a reliable, reproducible, and valid instrument for evaluating dyspnea during activities of daily living in patients with severe COPD.

Keywords: Activities of daily living; Dyspnea; Diagnostic techniques and procedures; Reproducibility of results.

Objective: To evaluate the properties of the Identity-Consequence Fatigue Scale (ICFS) in patients with lung cancer (LC), assessing the intensity of fatigue and associated factors. Methods: This was a cross-sectional study involving LC patients, treated at a teaching hospital in Brazil, who completed the ICFS. Patients with chronic heart disease (CHD) and healthy controls, matched for age and gender, also completed the scale. Initially, a Brazilian Portuguese-language version of the ICFS was administered to 50 LC patients by two independent interviewers; to test for reproducibility, it was readministered to those same patients. At baseline, the LC patients were submitted to spirometry and the six-minute walk test, as well as completing the Epworth Sleepiness Scale (ESS), Hospital Anxiety and Depression Scale (HADS), Medical Outcomes Study 36-item Short-Form Health Survey (SF-36), and Fatigue Severity Scale (FSS). Inflammatory status was assessed by blood C-reactive protein (CRP) levels. To validate the ICFS, we assessed the correlations of its scores with those variables. Results: The sample comprised 50 patients in each group (LC, CHD, and control). In the LC group, the intraclass correlation coefficients for intra-rater and inter-rater reliability regarding ICFS summary variables ranged from 0.94 to 0.76 and from 0.94 to 0.79, respectively. The ICFS presented excellent internal consistency, and Bland-Altman plots showed good test-retest reliability. The ICFS correlated significantly with FSS, HADS, and SF-36 scores, as well as with CRP levels. Mean ICFS scores in the LC group differed significantly from those in the CHD and control groups. Conclusions: The ICFS is a valid, reliable instrument for evaluating LC patients, in whom depression, quality of life, and CRP levels seem to be significantly associated with fatigue.

Keywords: Fatigue; Lung neoplasms; Symptom assessment.

Objective: To adapt the PROactive Physical Activity in COPD-clinical visit (C-PPAC) instrument to the cultural setting in Brazil and to determine the criterion validity, test-retest reliability agreement, and internal consistency of this version. Methods: A protocol for cultural adaptation and validation was provided by the authors of the original instrument and, together with another guideline, was applied in a Portuguese-language version developed by a partner research group from Portugal. The adapted Brazilian Portuguese version was then cross-sectionally administered twice within a seven-day interval to 30 individuals with COPD (57% were men; mean age was 69 ± 6 years; and mean FEV1 was 53 ± 18% of predicted) to evaluate internal consistency and test-retest reliability. Participants also completed the International Physical Activity Questionnaire (IPAQ), the modified Medical Research Council scale, the COPD Assessment Test, and Saint George's Respiratory Questionnaire to evaluate criterion validity. Results: The C-PPAC instrument showed good internal consistency and excellent test-retest reliability: "amount" domain = 0.87 (95% CI, 0.73-0.94) and "difficulty" domain = 0.90 (95% CI, 0.76-0.96). Bland & Altman plots, together with high Lin's concordance correlation coefficients, reinforced that agreement. Criterion validity showed moderate-to-strong correlations of the C-PPAC with all of the other instruments evaluated, especially with the IPAQ (rho = -0.63). Conclusions: The Brazilian Portuguese version of the C-PPAC is a reliable and valid instrument for evaluating the experience of Brazilian individuals with COPD with their physical activity in daily life.

Keywords: Pulmonary disease, chronic obstructive; Validation study; Activities of daily living; Psychometrics.

Objective: To test the construct validity, reliability, and measurement error of the Brazilian Portuguese-language version of the Manchester Respiratory Activities of Daily Living (MRADL) questionnaire in patients with COPD. Methods: We evaluated 50 patients with COPD, among whom 30 were men, the mean age was 64 ± 8 years, and the median FEV1 as a percentage of the predicted value (FEV1%predicted) was 38.4% (interquartile range, 29.1-57.4%). Pulmonary function and limitations in activities of daily living (ADLs) were assessed by spirometry and by face-to-face application of the MRADL, respectively. For the construct validity analysis, we tested the hypothesis that the total MRADL score would show moderate correlations with spirometric parameters. We analyzed inter-rater reliability, test-retest reliability, inter-rater measurement error, and test-retest measurement error. Results: The total MRADL score showed moderate correlations with the FEV1/FVC ratio, FEV1 in liters, FEV1%predicted, and FVC%predicted, all of the correlations being statistically significant (r = 0.34, r = 0.31, r = 0.42, and r = 0.38, respectively; p < 0.05 for all). For the reliability and measurement error of the total MRADL score, we obtained the following inter-rater and test-retest values, respectively: two-way mixed-effects model intraclass correlation coefficient for single measures, 0.92 (95% CI: 0.87-0.96) and 0.89 (95% CI: 0.81-0.93); agreement standard error of measurement, 1.03 and 0.97; smallest detectable change at the individual level, 2.86 and 2.69; smallest detectable change at the group level, 0.40 and 0.38; and limits of agreement, −2.24 to 1.96 and −2.65 to 2.69. Conclusions: In patients with COPD in Brazil, this version of the MRADL shows satisfactory construct validity, satisfactory inter-rater/test-retest reliability, and indeterminate inter-rater/test-retest measurement erro

Keywords: Pulmonary disease, chronic obstructive; Activities of daily living; Disability evaluation; Patient reported outcome measures; Validation studies.

Objective: To test the reliability, validity, and interpretability of the Brazilian version of the Clinical COPD Questionnaire (CCQ) in patients with COPD. Methods: Fifty patients with COPD completed the CCQ by interview on two occasions. At the first visit, the CCQ was administered twice, by two different raters, approximately 10 min apart; the patients also underwent spirometry and were administered the COPD Assessment Test, the modified Medical Research Council scale, and Saint George's Respiratory Questionnaire (SGRQ). At the second visit (1-2 weeks later), the CCQ was readministered. We tested the hypothesis that the CCQ total score would correlate positively with the total and domain SGRQ scores (r = 0.5). Results: Of the 50 patients, 30 (60%) were male. The mean age was 66 ± 8 years, and the mean FEV1 was 44.7 ± 17.9% of the predicted value. For all CCQ items, Cronbach's alpha coefficient (95% CI) was 0.93 (0.91-0.96). To analyze the interrater reliability and test-retest reliability of the CCQ, we calculated the two-way mixed effects model/single measure type intraclass correlation coefficient (0.97 [95% CI: 0.95-0.98] and 0.92 [95% CI: 0.86-0.95], respectively); the agreement standard error of measurement (0.65 for both); the smallest detectable change at the individual level (1.81 and 1.80, respectively) and group level (0.26 and 0.25, respectively); and the limits of agreement (-0.58 to 0.82 and -1.14 to 1.33, respectively). The CCQ total score correlated positively with all SGRQ scores (r = 0.70 for all). Conclusions: The Brazilian version of the CCQ showed an indeterminate measurement error, as well as satisfactory interrater/test-retest reliability and construct validity.

Keywords: Pulmonary disease, chronic obstructive; Health status; Patient reported outcome measures; Validation study.

Objective: To evaluate lung function and inspiratory muscle strength, correlating them with exercise tolerance, in obese individuals with obstructive sleep apnea syndrome (OSAS). Methods: The sample comprised 31 adult subjects with moderate-to-severe OSAS diagnosed by polysomnography. We used spirometry to measure FVC, FEV1, and FVC/FEV1 ratio, using pressure manometry to measure MIP and MEP. The incremental shuttle walk test (ISWT) and the six-minute walk test (6MWT) were used in order to determine functional exercise capacity. Results: In this sample, the mean values for FVC (% of predicted), FEV1 (% of predicted): MIP, and MEP were 76.4 ± 12.3%, 80.1 ± 6.3%, 60.0 ± 21.9 cmH2O, and 81.3 ± 22.2 cmH2O, respectively. The mean distances covered on the ISWT and 6MWT were 221 ± 97 m and 480.8 ± 67.3 m, respectively. The ISWT distance showed moderate positive correlations with FVC (r = 0.658; p = 0.001) and FEV1 (r = 0.522; p = 0.003). Conclusions: In this sample of obese subjects with untreated OSAS, lung function, inspiratory muscle strength, and exercise tolerance were all below normal. In addition, we found that a decline in lung function, but not in respiratory muscle strength, was associated with exercise tolerance in these patients.

Keywords: Sleep apnea syndromes; Exercise tolerance; Respiratory function tests; Respiratory muscles.

Objective: The assessment of the quality of life in patients with lung cancer has become one of the main goals in current clinical trials. To assess the quality of life of these patients, the most widely used instrument is the 30-item European Organization for Research and Treatment of Cancer Core Quality of Life Questionnaire (EORTC QLQ-C30) in conjunction with its supplemental 13-item lung cancer-specific module (QLQ-LC13). The objective of this study was to assess the reproducibility of the Brazilian Portuguese version of these questionnaires. Methods: A prospective study involving 30 stable outpatients with lung cancer who completed the instruments on the first day of the study and two weeks later. Results: The test-retest reproducibility using the intraclass correlation coefficient for the EORTC QLQ-C30 and the QLQ-LC13 ranged from 0.64 to 1.00 and from 0.64 to 0.95, respectively. No correlations were found between the domains of the instruments and clinical parameters. Conclusions: Our findings show that these instruments were reproducible in this sample of patients with lung cancer in Brazil.

Keywords: Lung neoplasms; Reproducibility of results; Quality of life.

It has been well established that, in addition to the pulmonary involvement, COPD has systemic consequences that can lead to peripheral muscle dysfunction, with greater muscle fatigue, lower exercise tolerance and lower survival in these patients. In view of the negative repercussions of early muscle fatigue in COPD, the objective of this review was to discuss the principal findings in the literature on the metabolic and bioenergy determinants of muscle fatigue, its functional repercussions, as well as the methods for its identification and quantification. The anatomical and functional substrate of higher muscle fatigue in COPD appears to include lower levels of high-energy phosphates, lower mitochondrial density, early lactacidemia, higher serum ammonia and reduced muscle perfusion. These alterations can be revealed by contraction failure, decreased firing rates of motor units and increased recruitment of motor units in a given activity, which can be functionally detected by a reduction in muscle strength, power and endurance. This review article also shows that various types of muscle contraction regimens and protocols have been used in order to detect muscle fatigue in this population. With this understanding, rehabilitation strategies can be developed in order to improve the resistance to muscle fatigue in this population.

Keywords: Pulmonary disease, chronic obstructive; Neuromuscular manifestations; Muscle fatigue; Exercise tolerance; Energy metabolism; Evaluation.

Introduction: Spirometry and analysis of blood gases data have been extensively used to assess ventilatory limitation and prognosis of COPD patients. However, functional physical tests, such as the six-minute walk test (6wt) have been used for the dynamic assessment of COPD patients. Objective: To determine correlations between respiratory function test data and the six-minute walk test in COPD patients. Patients and methods: 45 patients were submitted to clinical history survey and complete physical examination performed by the medical staff of the Pneumology Department of the Brasilia University Hospital, followed by spirometry, analysis of arterial blood gases, maximal respiratory pressures data, and the six-minute walk test. Results: Concerning the spirometric, analysis of blood gases and maximal respiratory pressures data, statistically significant positive correlations (p < 0.05) between FEV1, PaO2, SpO2, MEP and the six-minute walk test were obtained. Conclusions: For the studied group, the six minute walk test has significant correlation with FEV1, PaO2, SpO2 and MEP, and can be used as an alternative functional assessment of COPD patients.

Objective: To establish a cut-off point for clinical and functional variables to determinate sarcopenia and dynapenia in COPD patients, and to analyze the impact of skeletal muscle dysfunction (SMD) on these variables. Methods: Cross-sectional study, screened COPD patients for sarcopenia or dynapenia through low muscle mass and hand grip strength (HGS). Clinical variables: pulmonary function, respiratory muscle strength and functional capacity (FC). The precision of the variables in determining points of predictive cut-off for sarcopenia or dynapenia were performed using the Receiver Operating Characteristic curve and two-way analysis of variance. Results: 20 COPD patients stratified for sarcopenia (n = 11) and dynapenia (n = 07). Sarcopenia group presented lower lean mass and lower maximal inspiratory pressure (MIP), decreased HGS, reduced FC (p<0.050). Dynapenia group presented reduced MIP, lower HGS and walked a shorter distance at Incremental shuttle walk test (ISWT) (p<0.050). We found cut-off points of forced expiratory volume in one second (FEV1), MIP and maximal expiratory pressure (MEP) and ISWT. It is possible to identify sarcopenia or dynapenia in these patients. We found the coexistence of the conditions (SMD effect) in COPD - reduction in the distance in the ISWT (p = 0.002) and %ISWT (p = 0.017). Conclusion: In moderate to very severe COPD patients the sarcopenia could be predicted by FEV1 (%predicted) < 52, MIP < 73 cmH2O, MEP < 126 cmH2O and distance traveled of < 295 m in ISWT. Whereas dynapenia could be predicted by FEV1 < 40%, MIP < 71 cmH2O, MEP < 110 cmH2O and distance of < 230 m traveled in ISWT.

Keywords: Sarcopenia; Chronc obstructive pulmonary disease; Musculoskeletal system.

Objective: To evaluate the magnitude of diurnal changes in the parameters of pulmonary function and respiratory muscle strength/endurance in a sample of patients with COPD. Methods: A group of 7 patients underwent spirometry, together with determination of MIP and MEP, at two distinct times (between 8:00 and 8:30 a.m. and between 4:30 and 5:00 p.m.) on a single day. Between assessments, the patients remained at rest in the laboratory. Results: In accordance with the Global Initiative for Chronic Obstructive Pulmonary Disease staging system, COPD was classified as moderate, severe, and very severe in 1, 3, and 3 of the patients, respectively. From the first to the second assessment, there were significant decreases in FVC, FEV1, and MEP (of 13%, 15%, and 10%, respectively), as well as (less than significant) decreases in PEF, MIP, and maximal voluntary ventilation (of 9%, 3%, and 11%, respectively). Conclusions: In this sample of COPD patients, there were diurnal variations in the parameters of pulmonary function and respiratory muscle strength. The values of FEV1, FVC, and MEP were significantly lower in the afternoon than in the morning.

Keywords: Pulmonary disease, chronic obstructive; Respiratory function tests; Respiratory muscles.

Objective: To investigate the reliability, internal consistency and validity of the Brazilian Portuguese version of the University of California San Diego Shortness of Breath Questionnaire (UCSD SOBQ) in patients with interstitial lung disease (ILD). Methods: Patients with ILD completed the questionnaire at three different time points, one week apart, with the assistance of two independent assessors. Intra- and inter-rater reliability were analysed via the intraclass correlation coefficient (ICC). Internal consistency was assessed with the Cronbach's alpha coefficient. For the validity analysis, associations between variables were assessed with Spearman's or Pearson's correlation coefficient. Results: Thirty patients with ILD (idiopathic pulmonary fibrosis, connective tissue disease-associated pulmonary fibrosis, sarcoidosis, asbestosis or non-specific interstitial pneumonia) were included (15 men; mean age, 59 ± 10 years; DLCO: 46 [33-64] % predicted). UCSD SOBQ scores showed excellent agreement and internal consistency in the intra-rater analysis (ICC: 0.93 [0.85-0.97]; Cronbach alpha: 0.95) and in the inter-rater analysis (ICC: 0.95 [0.89-0.97]; Cronbach alpha: 0.95), as well as correlating significantly with dyspnoea (as assessed by the Medical Research Council scale; r = 0.56); Medical Outcomes Study 36-item Short-Form Health Survey domains bodily pain, general health, vitality and physical functioning (-0.40 = r = -0.74); six-minute walk distance (r = -0.38); and quadriceps muscle strength (r = -0.41). Conclusions: The Brazilian Portuguese version of the UCSD SOBQ is valid, is reliable and has internal consistency in patients with ILD in Brazil.

Keywords: Lung diseases, interstitial; Dyspnea; Surveys and questionnaires.

Objective: To cross-culturally adapt the Wisconsin Smoking Withdrawal Scale (WSWS) for use in Brazil and evaluate the reproducibility of the new (Brazilian Portuguese-language) version. Methods: The original English version of the WSWS was translated into Brazilian Portuguese. For cross-cultural adaptation, the Brazilian Portuguese-language version of the WSWS was administered to eight volunteers, all of whom were smokers. After adjustments had been made, the WSWS version was back-translated into English. The Brazilian Portuguese-language version was thereby found to be accurate. The final Brazilian Portuguese-language version of the WSWS was applied to 75 smokers at three distinct times. For the assessment of interobserver reproducibility, it was applied twice within a 30-min interval by two different interviewers. For the assessment of intraobserver reproducibility, it was applied again 15 days later by one of the interviewers. Intraclass correlation coefficients (ICCs) were used in order to test the concordance of the answers. The significance level was set at p < 0.05. Results: Of the 75 volunteers, 43 (57.3%) were female. The overall mean age was 46.3 years. Interobserver and intraobserver reproducibility was determined for each of the WSWS seven domains, the ICCs for which ranged from 0.87 to 0.94 and from 0.76 to 0.92, respectively. The mean time to completion of the WSWS was 6 min and 44 s, and the response time per question ranged from 4.2 to 12.6 s. Conclusions: The Brazilian Portuguese-language version of the WSWS is reproducible, fast, and simple. It can therefore be used as a tool for assessing the severity of the symptoms of nicotine withdrawal syndrome.

Keywords: Nicotine; Tobacco use disorder; Substance withdrawal syndrome; Reproducibility of results.

Objective: Barriers to physical activity can affect the functional capacity and quality of life of patients with pulmonary hypertension (PH). This study aimed to identify the main barriers to physical activity in patients with PH and to examine whether those barriers are related to functional capacity, echocardiographic variables, or quality of life. Methods: This was a cross-sectional observational study involving 70 patients. Participants scored seven potential barriers to their activities, with a score = 5 indicating a significant barrier. Participants completed the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36) and Manchester Respiratory Activity of Daily Living questionnaire, as well as the six-minute walk test. Correlation analysis, univariate analysis and multiple logistic regression were performed. Results: As a perceived barrier to physical activity, 'lack of will' or 'lack of energy' was cited by 67% of the patients. The 'lack of will' barrier was found to correlate with all SF-36 domains except bodily pain. We also identified a correlation between the SF-36 vitality domain and the barriers 'lack of energy', 'lack of will' and 'lack of structure'. The logistic regression analysis indicated that the vitality domain correlated significantly with the barriers 'social influence', 'lack of energy', 'lack of will', and 'lack of structure'. For each unit decrease in the vitality score, there was a 10% increase in the probability of citing the barrier 'lack of will'. No significant correlations were identified between any of the perceived barriers and echocardiographic parameters. Conclusions: The perceived barrier most commonly reported was 'lack of will/energy', which correlated with almost all SF-36 domains, especially vitality. The 'lack of will' barrier also correlated with functional capacity.

Keywords: Pulmonary arterial hypertension; Exercise; Motivation; Quality of life; Surveys and questionnaires; Walk test.