Physical activity levels are low in patients with pulmonary hypertension.

BACKGROUND: Pulmonary hypertension (PH) leads to exercise capacity impairment, but limited data are available on the objective evaluation of physical activity (PA) levels in these patients. METHODS: We assessed PA levels using triaxial accelerometry in Spanish PH patients (n=75, 48±14 years, 65% female) and gender/age matched controls (n=107), and examined potential associations of meeting minimum international recommendations [moderate-vigorous PA (MVPA) ≥150 min/week] with survival predictors. RESULTS: With the exception of vigorous PA (with very low values in both groups), all accelerometry data showed significant differences between patients and controls, with lower PA levels and proportion of individuals meeting minimum PA guidelines, but higher inactivity time, in the former. Notably, the odds ratio (OR) of having a "low-risk" value of 6-minute walking distance (≥464 m) or ventilatory equivalent for carbon dioxide (≤39) was higher in patients following MVPA guidelines than in their less active peers [OR =4.3, 95% confidence interval (CI), 1.6-11.6, P=0.005, and OR =4.5, 95% CI, 0.9-21.1, P=0.054]. CONCLUSIONS: Daily PA is reduced in patients with PH, often to a level that may decrease their odds of survival. Efforts should be made to promote the implementation of healthy PA habits in this patient population.

Adaptación y validación del cuestionario Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) para uso en España / Adaptation and validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for use in Spain

Introducción y objetivos: El cuestionario CAMPHOR (Cambridge Pulmonary Hypertension Outcome Review) es un instrumento de calidad de vida relacionado con esta y la salud informado por el paciente y específico para pacientes con hipertensión pulmonar (HP). En esta área, el CAMPHOR se ha demostrado superior a otros instrumentos que evalúan aspectos similares. El objetivo del presente estudio es adaptar y validar la versión española del CAMPHOR. Métodos: La adaptación consistió en 3 etapas: traducción del inglés al español por medio de paneles bilingües y laicos, cognitive debriefing con los pacientes y la evaluación de las propiedades psicométricas por medio de una encuesta postal de validación. Resultados: Los paneles de traducción produjeron una versión del CAMPHOR adecuada para que la utilicen pacientes con HP españoles. La relevancia, la exhaustividad y la aceptabilidad de esta versión se confirmaron en entrevistas con pacientes con HP. Por último, el estudio de validación (n = 70) reveló que las 3 escalas CAMPHOR (síntomas, actividades y calidad de vida) muestran fuertes propiedades psicométricas. Los coeficientes de consistencia interna (alfa de Cronbach) de las escalas estuvieron por encima de 0,89 y la fiabilidad test-retest, por encima de 0,87. La validez convergente y de grupos conocidos de las escalas CAMPHOR también se confirmaron. Conclusiones: La versión española del CAMPHOR es un instrumento válido y fiable para la evaluación de la calidad de vida relacionada con la salud y la calidad de vida de los pacientes con HP españoles. Por lo tanto, se recomienda su uso en futuros estudios y la práctica clínica en la población española de pacientes con HP (AU)

Introduction and objectives: The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is a patient-reported outcome measure of health-related quality of life and quality of life specific to individuals with pulmonary hypertension (PH). This questionnaire has demonstrated superiority over other instruments assessing similar domains. The objective of the present study was to adapt and validate the Spanish version of the questionnaire. Methods: The adaptation consisted of 3 stages: translation from English to Spanish using bilingual and lay panels, cognitive debriefing interviews with patients, and assessment of psychometric properties by means of a postal validation survey. Results: The translation panels produced a version of the CAMPHOR that was considered suitable for use by Spanish PH patients. The relevance, comprehensiveness, and acceptability of this version were confirmed in interviews with PH patients. Finally, the validation survey (n = 70) revealed that the 3 CAMPHOR scales (Symptoms, Activities, and Quality of life) showed strong psychometric properties. The internal consistency (Cronbach α) coefficients of the scales were above 0.89, and the test-retest reliability was above 0.87. The convergent and known group validity of the CAMPHOR scales was also demonstrated. Conclusions The Spanish version of the CAMPHOR is a valid and reliable instrument for the assessment of health-related quality of life and quality of life in Spanish PH patients. Therefore, it is recommended for use in future research and clinical practice in the Spanish population of PH patients (AU)

Effects of an 8-month exercise intervention on physical capacity, NT-proBNP, physical activity levels and quality of life data in patients with pulmonary arterial hypertension by NYHA class.

This article provides descriptive detailed (pre and post) values of physical capacity variables, NT-proBNP, physical activity levels and quality of life in patients with pulmonary arterial hypertension (PH) (both, intervention and control group) by New York Heart Association (NYHA) class before and after an 8-month exercise intervention. The data are supplemental to our original Randomized Controlled Trial (RCT) entitled "Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+12 trial" (L. González-Saiz, C. Fiuza-Luces, F. Sanchis-Gomar, A. Santos-Lozano, C.A. Quezada-Loaiza, A. Flox-Camacho, D. Munguía-Izquierdo, I. Ara, A. Santalla, M. Morán, P. Sanz-Ayan, P. Escribano-Subías, A. Lucia A, 2017) [1].

Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+12 trial.

BACKGROUND: Pulmonary arterial hypertension is often associated with skeletal-muscle weakness. The purpose of this randomized controlled trial was to determine the effects of an 8-week intervention combining muscle resistance, aerobic and inspiratory pressure-load exercises on upper/lower-body muscle power and other functional variables in patients with this disease. METHODS: Participants were allocated to a control (standard care) or intervention (exercise) group (n=20 each, 45±12 and 46±11years, 60% women and 10% patients with chronic thromboembolic pulmonary hypertension per group). The intervention included five, three and six supervised (inhospital) sessions/week of aerobic, resistance and inspiratory muscle training, respectively. The primary endpoint was peak muscle power during bench/leg press; secondary outcomes included N-terminal pro-brain natriuretic peptide levels, 6-min walking distance, five-repetition sit-to-stand test, maximal inspiratory pressure, cardiopulmonary exercise testing variables (e.g., peak oxygen uptake), health-related quality of life, physical activity levels, and safety. RESULTS: Adherence to training sessions averaged 94±0.5% (aerobic), 98±0.3% (resistance) and 91±1% (inspiratory training). Analysis of variance showed a significant interaction (group×time) effect for leg/bench press (P<0.001/P=0.002), with both tests showing an improvement in the exercise group (P<0.001) but not in controls (P>0.1). We found a significant interaction effect (P<0.001) for five-repetition sit-to-stand test, maximal inspiratory pressure and peak oxygen uptake (P<0.001), indicating a training-induced improvement. No major adverse event was noted due to exercise. CONCLUSIONS: An 8-week exercise intervention including aerobic, resistance and specific inspiratory muscle training is safe for patients with pulmonary arterial hypertension and yields significant improvements in muscle power and other functional variables.

Adaptation and Validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for Use in Spain.

INTRODUCTION AND OBJECTIVES: The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is a patient-reported outcome measure of health-related quality of life and quality of life specific to individuals with pulmonary hypertension (PH). This questionnaire has demonstrated superiority over other instruments assessing similar domains. The objective of the present study was to adapt and validate the Spanish version of the questionnaire. METHODS: The adaptation consisted of 3 stages: translation from English to Spanish using bilingual and lay panels, cognitive debriefing interviews with patients, and assessment of psychometric properties by means of a postal validation survey. RESULTS: The translation panels produced a version of the CAMPHOR that was considered suitable for use by Spanish PH patients. The relevance, comprehensiveness, and acceptability of this version were confirmed in interviews with PH patients. Finally, the validation survey (n = 70) revealed that the 3 CAMPHOR scales (Symptoms, Activities, and Quality of life) showed strong psychometric properties. The internal consistency (Cronbach α) coefficients of the scales were above 0.89, and the test-retest reliability was above 0.87. The convergent and known group validity of the CAMPHOR scales was also demonstrated. CONCLUSIONS: The Spanish version of the CAMPHOR is a valid and reliable instrument for the assessment of health-related quality of life and quality of life in Spanish PH patients. Therefore, it is recommended for use in future research and clinical practice in the Spanish population of PH patients.

Rationale and Design of a Randomized Controlled Trial Evaluating Whole Muscle Exercise Training Effects in Outpatients with Pulmonary Arterial Hypertension (WHOLEi+12).

BACKGROUND AND AIMS: Physical exercise is an important component in the management of pulmonary artery hypertension (PAH). The aim of this randomized controlled trial (RCT) is to determine the effects of an 8-week intervention combining muscle resistance, aerobic and inspiratory pressure load exercises in PAH outpatients. METHODS: The RCT will be conducted from September 2015 to September 2016 following the recommendations of the Consolidated Standards of Reported Trials (CONSORT), with a total sample size of n ≥ 48 (≥24 participants/group). We will determine the effects of the intervention on: (i) skeletal-muscle power and mass (primary end points); and (ii) NT-proBNP, cardiopulmonary exercise testing variables (VO2peak, ventilatory equivalent for CO2 at the anaerobic threshold (VE/VCO2 at the AT), end-tidal pressure of CO2 at the anaerobic threshold (PETCO2 at the AT), 6-min walking distance (6MWD), maximal inspiratory pressure (PImax), health-related quality of life (HRQoL), objectively-assessed spontaneous levels of physical activity, and safety (secondary end points). CONCLUSIONS AND PERSPECTIVES: This trial will provide insight into biological mechanisms of the disease and indicate the potential benefits of exercise in PAH outpatients, particularly on muscle power.

Benefits of combining inspiratory muscle with 'whole muscle' training in children with cystic fibrosis: a randomised controlled trial.

BACKGROUND: The purpose of this study (randomised controlled trial) was to assess the effects of an 8-week combined 'whole muscle' (resistance+aerobic) and inspiratory muscle training (IMT) on lung volume, inspiratory muscle strength (PImax) and cardiorespiratory fitness (VO2 peak) (primary outcomes), and dynamic muscle strength, body composition and quality of life in paediatric outpatients with CF (cystic fibrosis, secondary outcomes). We also determined the effects of a detraining period. METHODS: Participants were randomly allocated with a block on gender to a control (standard therapy) or intervention group (initial n=10 (6 boys) in each group; age 10±1 and 11±1 years). The latter group performed a combined programme (IMT (2 sessions/day) and aerobic+strength exercises (3 days/week, in-hospital)) that was followed by a 4-week detraining period. All participants were evaluated at baseline, post-training and detraining. RESULTS: Adherence to the training programme averaged 97.5%±1.7%. There was a significant interaction (group×time) effect for PImax, VO2peak and five-repetition maximum strength (leg-press, bench-press, seated-row) (all (p<0.001), and also for %fat (p<0.023) and %fat-free mass (p=0.001), with training exerting a significant beneficial effect only in the intervention group, which was maintained after detraining for PImax and leg-press. CONCLUSION: The relatively short-term (8-week) training programme used here induced significant benefits in important health phenotypes of paediatric patients with CF. IMT is an easily applicable intervention that could be included, together with supervised exercise training in the standard care of these patients.

Aerobic fitness is associated with lower risk of hospitalization in children with cystic fibrosis.

BACKGROUND: Children with cystic fibrosis (CF) often have to be hospitalized because of acute exacerbation of their respiratory symptoms. Given the fact that improved peak oxygen uptake (VO2peak ) is positively associated with lung function and overall health in children with CF, this study examined the association between VO2peak and the need for hospitalization in a cohort of pediatric CF patients. METHODS: In a 3-year study, 77 CF children with mild-to-moderately severe CF (forced expiratory volume in 1 sec [FEV1 ] ≥ 50%) underwent a maximal exercise test to determine VO2peak . Anthropometric, lung function and muscle strength measurements were also conducted and dates of hospitalization were recorded for the study period. Associations were then determined between the variables recorded and hospitalization by univariate and multivariate Cox proportional hazards regression analyses. RESULTS: VO2peak was 38.6 ± 6.7 ml kg(-1) min(-1) for boys and 31.9 ± 6.9 ml kg(-1) min(-1) for girls. In multivariate analyses, VO2peak was the only variable significantly associated with time to hospitalization (hazard ratio 0.91, P = 0.03). CONCLUSION: A significant association was detected between greater aerobic fitness, and lower risk of hospitalization. Because hospitalization due to respiratory exacerbation is a powerful prognostic factor, our findings provide further support for the importance of aerobic fitness evaluation in the management of children with mild-to-moderately severe CF.

Health-related quality of life of Spanish children with cystic fibrosis.

PURPOSE: To investigate (1) the contributions of sex, age, nutritional status- and physical-fitness-related variables on health-related quality of life (HRQOL) in Spanish children with cystic fibrosis, and (2) the agreement on HRQOL between children and their parents. METHODS: In 28 children aged 6-17 years, body mass index percentile, percentage body fat, physical activity, pulmonary function, cardiorespiratory fitness, functional mobility, and dynamic muscle strength were determined using objective measures. HRQOL was measured using the revised version of the cystic fibrosis questionnaire. Simple and multiple linear regression analyses were performed to determine the variables associated with HRQOL. To assess the agreement on HRQOL between children and parents, intra-class correlation coefficients (ICCs) were calculated. RESULTS: Girls reported worse emotional functioning, a higher treatment burden, and more respiratory problems than boys. Greater functional mobility appeared associated with a less favourable body image and more eating disturbances. Agreement on HRQOL between children and parents was good to excellent, except for the domain of treatment burden. CONCLUSIONS: Sex and age were stronger predictors of HRQOL than nutritional status- or physical-fitness-related variables. Children reported a lower treatment burden than their parents perceived them to have.

Intrahospital weight and aerobic training in children with cystic fibrosis: a randomized controlled trial.

PURPOSE: The purpose of our study was to assess the effects of an 8-wk intrahospital combined circuit weight and aerobic training program performed by children with cystic fibrosis (of low-moderate severity and stable clinical condition) on the following outcomes: cardiorespiratory fitness (VO2peak) and muscle strength (five-repetition maximum (5RM) bench press, 5RM leg press, and 5RM seated row) (primary outcomes) and pulmonary function (forced vital capacity, forced expiratory volume in 1 s), weight, body composition, functional mobility (Timed Up and Down Stairs and 3-m Timed Up and Go tests), and quality of life (secondary outcomes). We also determined the effects of a detraining period (4 wk) on the aforementioned outcomes. METHODS: We performed a randomized controlled trial design. Eleven participants in each group (controls: 7 boys, age = 11 ± 3 yr, body mass index = 17.2 ± 0.8 kg · m(-2) (mean ± SEM); intervention: 6 boys, age = 10 ± 2 yr, body mass index = 18.4 ± 1.0 kg · m(-2)) started the study. RESULTS: Adherence to training averaged 95.1% ± 7.4%. We observed a significant group × time interaction effect (P = 0.036) for VO2peak. In the intervention group, VO2peak significantly increased with training by 3.9 mL · kg(-1) · min(-1) (95% confidence interval = 1.8-6.1 mL · kg(-1) · min(-1), P = 0.002), whereas it decreased during the detraining period (-3.4 mL · kg(-1) · min(-1), 95% confidence interval = -5.7 to -1.7 mL · kg(-1) · min(-1), P = 0.001). In contrast, no significant changes were observed during the study period within the control group. Although significant improvements were also observed after training for all 5RM strength tests (P < 0.001 for the interaction effect), the training improvements were not significantly decreased after the detraining period in the intervention group (all P > 0.1 for after training vs detraining). We found no significant training benefits in any of the secondary outcomes. CONCLUSIONS: A short-term combined circuit weight and aerobic training program performed in a hospital setting induces significant benefits in the cardiorespiratory fitness and muscle strength of children with cystic fibrosis.