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Pulmonary rehabilitation (PR) is a mandatory component of a comprehensive treatment of patients with chronic respiratory disease. However, there is no officially published data about PR Centers in Latin America. The objetive is to identify Latin American Pulmonary Rehabilitation Centers and evaluate their characteristics and organizational aspects. A cross-sectional study with the Pulmonary Rehabilitation Centers indicated by the Latin American Respiratory and Physiotherapy Societies and by our own personal survey among physicians and physiotherapists. An eletronic transmission questionnaire with 20 questions was sent to the Coordinator of each one of the Centers. 217 Pulmonary Rehabilitation Centers were found throughout Latin America and a total of 160 (73.7%) Centers answered the questionnaire. Of these, 65.8% had private administration; 68.8% had an associated program for patients with heart disease; programs lasted an average of 24 sessions; the rehabilitation team consisted mainly of physiotherapists, physicians and dietitians; 90.6% of the centers evaluated the patients with different questionnaires; 91.9% used treadmill and 90.6% bicycle for exercises of lower limbs and 80.1% proprioceptive neuromuscular facilitation technique with weights to train the upper limbs of their patients; 55.6% had an educational program and 36.9% presented a home-based program. There has been a great increase in the number of Latin American Pulmonary Rehabilitation Centers, presenting diversity in organizational aspects.

Introduction: Chronic thromboembolic hypertension (CTEPH) patients present decreased aerobic capacity during cardiopulmonary exercise test (CPET) with lower limbs (LL). It is not known if CTEPH patients present the same decreased aerobic capacity during upper limbs (UL) test. Objective: To investigate the response of a UL maximal test on cycloergometer in CTEPH patients and control group and compare to a CPET. Methods: The cross-sectional study including 33 CTEPH patients (54.5% male, 46.5 ± 14.2 years, 33% NYHA FC III and IV) and 22 controls (40.9% male, 39.8 ± 10.9 years). All patients underwent a UL and LL CPET cycloergometer measuring ventilation and gas exchange at peak exercise. Results: The oxygen consuption (VO2) of a UL maximal test of CTEPH patients (815.9 ± 212.7 mL/min) was lower than controls (1066.8 ± 340.2 mL/min) (p=0.004) and corresponded to 76% of the control group. The UL VO2 of CTEPH patients corresponded to 86% of their VO2max in a CPET and to 68% in the control group (p Conclusion: The aerobic capacity of UL in CTEPH patients is lower than a matched sex and age control group and worse physiological responses were observed in the CTEPH patients.

Cardiopulmonary exercise testing (CPET) on a treadmill or cycle ergometer provides an integrated assessment of the cardiorespiratory system during exertion and is widely used in clinical practice. An incremental step test (IST) can be an alternative for eliciting maximal exercise responses. Therefore, 20 patients with pre-capillary PH (65% female, 41 ± 15 yrs) randomly performed a symptom-limited CPET on a cycle ergometer and IST. Metabolic, cardiovascular, ventilatory and gas exchange variables were recorded during both tests. There was a greater desaturation and higher VO2PEAK in IST compared to CPET. The VO2GET, HR PEAK (% pred), ΔVE/ΔVCO2 and ΔHR/ΔVO2 were similar in both IST and CPET. By linear regression analyses, the work performed on IST [W = (mass × 9,8 m/s2 x vertical distance)] was a predictor of peak VO2 independent of the gender and age (r2 = 077, p = 0001). In conclusion, IST elicited higher peak cardiopulmonary responses and has a good agreement with known severity markers in patients with pre-capillary PH.

Six-minute walk distance (6MWD) assessment is recommended for pulmonary arterial hypertension multidimensional risk stratification. However, current 6MWD cut-off values were mainly derived from North American and European pulmonary arterial hypertension registries. Therefore, it is unknown if such cut-off values broadly apply to other geographical populations. In this study, we aimed to identify 6MWD cut-off values for Brazilian pulmonary arterial hypertension patients and to contrast our findings to current international Pulmonary Hypertension guidelines recommendations. One-hundred four consecutive pulmonary arterial hypertension patients were allocated in groups according to their 6MWD, considering 50 m as a clinically relevant 6MWD difference. Next, patients were categorized into different 6MWD ranges based on similar survival rates in each group: 400 m. The study outcome was all-cause mortality and transplantation according to the 6MWD range. Survival was truncated at five years. Median follow-up period was 4.35 years (0.48–5.00). Survival rates at 1, 2, 3, and 5 years were 96%, 89%, 81%, and 73%, respectively. Cox analyses adjusted for age, sex, and pulmonary arterial hypertension etiology showed that 6MWD 400 m were associated with higher and lower risk of all-cause mortality and transplantation. According to Harrell's c-statistic, the prognostic discrimination of the 6MWD cut-off value identified by the current study was 0.70 while international Pulmonary Hypertension guidelines 6MWD cut-offs value was 0.61. In conclusion, our findings suggest that 6MWD geographical variations should be considered when assessing risk stratification in pulmonary arterial hypertension.

Introduction: Patients with CTEPH have more proximal and heterogenous obstructive vascular lesions that may lead to more pronounced gas exchange abnormalities during walking compared to patients with IPAH. Objective: To analyze the physiological responses during the 6MWT in patients with CTEPH and IPAH. Methods: 15 patients with IPAH (29.5±19.6 yrs; 53% female; 80% NYHA functional class II and III) and 27 with CTEPH (50.4±16.3 yrs; 60% female; 96% NYHA FC II and III), with similar resting hemodynamics (cardiac index: 2.21±0.43 vs 2.32±0.65 and pulmonary vascular resistance 1132±416 vs 883±352 dynes.s.cm -3 ) were included. Patients underwent the 6MWT using a portable metabolic cart (Oxycon Mobile). Results: There was no difference in the walking distance between the two groups, expressed in absolute or %predicted [419,7±61,7m (79±11%pred) vs 429,2±82,4m (78±14 %pred), p>0.05, VE/VCO 2 53±13 vs 57±11, p>0.05. Despite similar cardiovascular performance at the end of the test (heart rate 68±6 vs 67±17 %pred and O 2 pulse 70±16 vs 64±16%pred, p>0.05), patients with CTEPH presented a higher oxyhemoglobin desaturation by pulse oximetry (SpO 2 89±7 vs 85±4% p=0.03) and greater sensation of dyspnea [Borg score 3(0;6) vs 5(2;8) p=0,04]. Conclusion: Patients with CTEPH present more severe gas exchange abnormalities and greater sensation of dyspnea during 6MWT.

Introduction: Despite therapeutic advances, exercise capacity of patients with CTEPH is often compromised. In addition to central haemodynamic impairment, systemic inflammation and low oxygen delivery may lead to abnormalities in peripheral striated muscles with important repercussion in muscular groups placed under high demand conditions. Since excessive ventilation is a key feature of patients with CTEPH, we hypothesize that inspiratory muscle weakness (IMW) is a frequent finding and may be associated with exercise impairment. Objective: To evaluate the frequency of IMW [maximal inspiratory pressure (Pi MAX ) pred ] in patients with CTEPH and its association with peak VO 2 obtained at cardiopulmonary exercise test (CPET) and distance of 6 minute walk test (6MWT). Methods: 24 patients with CTEPH [54±11 yrs; 38% male; 46% in NYHA functional class (FC) III/IV], confirmed by right-heart catheterization, underwent a CPET, 6MWT and PI MAX measurements. Results: Pi max pred was found in 41% of patients. Despite similar haemodynamics [cardiac index 2.4±0.6 vs 2.2±0.4L/min/m 2 and pulmonary vascular resistance (PVR) 854 ± 317 vs 823 ± 374 dynes.s.cm -3 ], patients with IMW showed lower 6MWD (342 ± 99 vs 442 ± 67m), lower peak VO 2 (50.8 ± 9.4 vs 63.8 ± 17.2% predicted) and increased ΔVE/ΔVCO 2 , compared to patients with Pi MAX >70% pred (p MAX was the only independent predictor of Peak VO 2 (partial r 0.445, p 0.04). Similar results were found for 6MWD as outcome variable. Conclusions: Pi MAX was independently associated with exercise capacity, when adjusted for resting heamodynamics and FC.

Introduction: Compared to IPAH, patients with CTEPH have greater abnormalities in gas exchange and ventilatory responses during maximum exercise test. Thus, it is possible that these patients have a higher limitation to perform activities of daily living (ADL). Objective: To compare energy expenditure and pathophysiological responses during the simulation of activities of daily living in patients with CTEPH and IPAH. Methods: Cross-sectional study including 9 patients with CTEPH (49±11.8yrs, 66.7% male, 55.6% NYHA FC II and III) and 12 with IPAH (37.4±9.4yrs, 38.5% male, 46.2% NYHA FC II and III). All patients underwent simulation of 10 ADLs with use of Oxycom Mobile device. The oxygen consumption (VO 2 ) during ADL was analyzed in absolute value and as a percentage of Peak VO 2 in incremental exercise test. Results: Patients with CTEPH presented higher VO 2 for walking with 2.5 kg in both hands for 5 minutes (100 ± 16.7% vs 83.2 ± 16.8%, p = 0.03) and walking with 5kg in one hand for another 5 minutes (105.9 ± 18.2% vs 89.8 ± 13.9%, p = 0.042). In both groups, many of ADLs were performed above the anaerobic threshold and very close to their peak aerobic capacity. There was a higher degree of dessaturation and increased VE/VCO 2 in patients with CTEPH. Conclusion: Patients with CTEPH have higher desaturation and excessive ventilatory responses while performing ADL, compared to patients with IPAH.

Introduction: Despite controversy, Maximal Voluntary Ventilation (MVV) is still considerate a key physiological variable to assess ventilatory reserve in maximum cardiopulmonary exercise test. It can be measured or calculated using forced expiratory volume in 1 second (FEV1) by equations derived from healthy individuals. Patients with pulmonary hypertension (PH) may have abnormalities in pulmonary function tests that may change the accuracy of measured MVV. Objective: Determine whether calculated MVV is an adequate method in patients with PH. Methods: We evaluated 119 patients with PH (60% of Group 1 and 40% of Group 4 of Nice Classification; 66% female; 47±15yrs). Patients performed three acceptable and reproducible maximal maneuvers (difference of 10% or less between values) and recorded value was the highest. Estimated MVV was defined by an equation validated for Brazilian healthy population (MVV=FEV1x37.5+15.8). Results: The average of measured and estimated MVV was 98.1±21.2 and 100.7±27.2L/min, respectively. The agreement interval between them was wide and there was a great variability between values (mean difference: -2.6L/min; 95% limits of agreement range from −25.7 to 26.7L/min). There was an overestimation of estimated values at lower measured MVV and underestimation at higher values. Conclusion: There is a poor agreement between estimated and measured MVV in patients with PH.

Objective: To determine the prevalence of alpha 1-antitrypsin (AAT) deficiency (AATD), as well as allele frequency, in COPD patients in Brazil. Methods: This was a cross-sectional study involving 926 COPD patients 40 years of age or older, from five Brazilian states. All patients underwent determination of AAT levels in dried blood spot (DBS) samples by nephelometry. Those with DBS AAT levels ≤ 2.64 mg/dL underwent determination of serum AAT levels. Those with serum AAT levels of < 113 mg/dL underwent genotyping. In case of conflicting results, SERPINA1 gene sequencing was performed. Results: Of the 926 COPD patients studied, 85 had DBS AAT levels ≤ 2.64 mg/dL, and 24 (2.6% of the study sample) had serum AAT levels of < 113 mg/dL. Genotype distribution in this subset of 24 patients was as follows: PI*MS, in 3 (12.5%); PI*MZ, in 13 (54.2%); PI*SZ, in 1 (4.2%); PI*SS, in 1 (4.2%); and PI*ZZ, in 6 (25.0%). In the sample as a whole, the overall prevalence of AATD was 2.8% and the prevalence of the PI*ZZ genotype (severe AATD) was 0.8% Conclusions: The prevalence of AATD in COPD patients in Brazil is similar to that found in most countries and reinforces the recommendation that AAT levels be measured in all COPD patients. RESUMO Objetivo: Determinar a prevalência da deficiência de alfa 1-antitripsina (AAT), bem como a frequência alélica, em pacientes com DPOC no Brasil. Métodos: Estudo transversal com 926 pacientes com DPOC, com 40 anos ou mais, oriundos de cinco estados brasileiros. Todos os pacientes foram submetidos a dosagem de AAT em amostras de sangue seco por meio de nefelometria. Aqueles em que a concentração de AAT no sangue seco foi ≤ 2,64 mg/dl foram submetidos a dosagem sérica de AAT. Aqueles em que a concentração sérica de AAT foi < 113 mg/dl foram submetidos a genotipagem. Quando os resultados foram discrepantes, foi realizado o sequenciamento do gene SERPINA1. Dos 926 pacientes com DPOC estudados, 85 apresentaram concentração de AAT em sangue seco ≤ 2,64 mg/dl, e 24 (2,6% da amostra) apresentaram concentração sérica de AAT < 113 mg/dl. A distribuição genotípica nesse subgrupo de 24 pacientes foi a seguinte: PI*MS, em 3 (12,5%); PI*MZ, em 13 (54,2%); PI*SZ, em 1 (4,2%); PI*SS, em 1 (4,2%); e PI*ZZ, em 6 (25,0%). Na amostra estudada, a prevalência global da deficiência de AAT foi de 2,8% e a prevalência do genótipo PI*ZZ (deficiência grave de AAT) foi de 0,8%. Conclusões: A prevalência da deficiência de AAT em pacientes com DPOC no Brasil é semelhante àquela encontrada na maioria dos países e reforça a recomendação de que se deve medir a concentração de AAT em todos pacientes com DPOC.

Background: Patients with idiopathic pulmonary arterial hypertension (iPAH) have a poor exercise tolerance and frequently report limitations during domestic activities of daily life (ADLs). We hypothesize that patients with iPAH have a relatively higher task-related oxygen uptake, VE/VCO2 and dyspnoea perception during domestic ADLs than healthy subjects. Methods: Metabolic, ventilatory and cardiovascular responses were evaluated in 15 patients with iPAH and 9 sex and age-matched healthy subjects during 11 ADL. These data were assessed using a Oxycon Mobile device and compared with values obtained by cycle-ergometer incremental cardiopulmonary exercise test of upper and lower limbs and distance walked in six minute walk test. Borg scores were used to assess ADL related dyspnoea and fadigue. Results: iPAH patients used a significantly higher proportion of their peak aerobic capacity to perform ADLs when compared to healthy subjects, accompained by higher Borg dyspnea scores. Many ADLs were performed above the anaerobic threshold. Oxygen pulse was reduced in lower limb ADLs. The VE/VCO2 was higher in all ADLs. Conclusion: Patients with iPAH perform their ADLs above anaerobic threshold and very close to their peak aerobic capacity, accompanied by significantly high symptoms scores.

OBJETIVO: Validar e desenvolver um método de dosagem de alfa-1 antitripsina (AAT) por imunonefelometria em amostras de sangue em papel-filtro em pacientes com DPOC no Brasil.MÉTODOS:Amostras de soro e de sangue em papel-filtro de 192 pacientes com DPOC foram utilizadas para a dosagem de AAT. Para a preparação das amostras de sangue em papel-filtro, um disco do papel com diâmetro de 6 mm foi colocado em um tubo e eluído com 200 µL de PBS, permanecendo por toda a noite a 4ºC. Todas as amostras foram analisadas em duplicata por imunonefelometria. O método de reamostragem bootstrap foi utilizado para a determinação de um ponto de corte para o nível de AAT nas amostras de sangue em papel-filtro.RESULTADOS:O coeficiente de correlação entre os níveis de AAT em soro e em sangue em papel-filtro foi de r = 0,45. Para as amostras em papel-filtro, o ponto de corte foi de 2,02 mg/dL (IC97%: 1,45-2,64 mg/dL), com sensibilidade, especificidade, valor preditivo positivo e valor preditivo negativo de 100%, 95,7%, 27,2% e 100%, respectivamente.CONCLUSÕES:Este método de determinação dos níveis de AAT em sangue em papel-filtro se mostrou uma ferramenta confiável para o rastreamento de pacientes com deficiência de AAT. OBJECTIVE:To validate and develop an immunonephelometric assay for the determination of alpha-1 antitrypsin (AAT) levels in dried blood spots from COPD patients in Brazil.METHODS:We determined AAT levels in serum samples and dried blood spots from 192 COPD patients. For the preparation of dried blood spots, a disk (diameter, 6 mm) was placed into a tube, eluted with 200 µL of PBS, and stored overnight at 4ºC. All of the samples were analyzed by immunonephelometry in duplicate. We used the bootstrap resampling method in order to determine a cut-off point for AAT levels in dried blood spots.RESULTS:The correlation coefficient between the AAT levels in serum samples and those in dried blood spots was r = 0.45. For dried blood spots, the cut-off value was 2.02 mg/dL (97% CI: 1.45-2.64 mg/dL), with a sensitivity, specificity, positive predictive value, and negative predictive value of 100%, 95.7%, 27.2%, and 100%, respectively.CONCLUSIONS:This method for the determination of AAT levels in dried blood spots appears to be a reliable screening tool for patients with AAT deficiency. Universidade Federal de São Paulo (UNIFESP) Department of Pulmonology Pulmonary Rehabilitation Center Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Department of Pulmonology Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Department of Psychobiology Afip Laboratory Department of Molecular Biology Vall d'Hebron University Hospital Departments of Biochemistry and Pulmonology UNIFESP, Department of Pulmonology Pulmonary Rehabilitation Center UNIFESP, EPM, Department of Pulmonology UNIFESP, EPM, Department of Psychobiology SciELO