The primary aim of this study was to evaluate exercise capacity, respiratory muscle strength, pulmonary function, dyspnea and physical activity levels in pediatric PAH patients and compare them with healthy controls. The secondary aim of the study was; To investigate the relationship of dyspnea with exercise capacity, respiratory muscle strength, respiratory functions, physical activity and blood count parameters in pediatric PAH patients.
Pulmonary arterial hypertension (PAH) is a mean pulmonary artery pressure of 25 mmHg or more at rest. Since the diagnosis of PAH can be made mostly in the late phase of the disease, severe functional and hemodynamic problems are prevalent in patients. In addition, patients commonly present with dyspnea, fatigue, weakness, general exercise intolerance, chest pain, syncope, and abdominal distension. These signs and symptoms are due to reduced oxygen consumption and decreased cardiac output. Activity dyspnea is evident in patients in the early stages of the disease. There is limited number of studies in pediatric PAH patients investigating exercise intolerance. While the cardiopulmonary exercise test can be safely performed in pediatric PAH patients, the six-minute walk test is both an independent predictor of prognosis and reflects the severity of the disease in these patients. However, there are no studies in the literature investigating and comparing exercise capacity, respiratory muscle strength, respiratory functions, dyspnea, and physical activity levels in pediatric PAH patients compared to age- and sex-matched healthy children. For this reason, in this study, the level of physical impairments of children with pediatric PAH was investigated for the first time compared to healthy children. In addition, there has been no study in the literature investigated the relationship between dyspnea, which can be seen significantly from the early stages of the disease in pediatric PAH patients, with exercise capacity, respiratory function, respiratory muscle strength, physical activity levels and blood count parameters. It is a cross-sectional study. At least 15 PAH patients and 15 age-and sex-matched healthy controls were aimed to include in the study. Individuals' exercise capacity using six minute walk test, respiratory muscle strength using a mouth pressure device, pulmonary function using spirometry, dyspnea using Modified Borg scale, physical activity using multi-sensory activity monitor were evaluated. The assessments were completed in two days.
Study Type
OBSERVATIONAL
Enrollment
33
Gazi University Faculty of Health Sciences Department of Physical Therapy and Rehabilitation
Ankara, Çankaya, Turkey (Türkiye)
Exercise capacity
Functional exercise capacity was evaluated using the 6- Minute Walk Test. 6- Minute Walk Test will be applied according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) criteria.
Time frame: First Day
Respiratory muscle strength
Maximal inspiratory (MIP) and maximal expiratory (MEP) pressures expressing respiratory muscle strength were measured using a portable mouth pressure measuring device according to ATS and ERS criteria.
Time frame: First Day
Pulmonary function (Forced vital capacity (FVC))
Pulmonary function was evaluated using the spirometry. Dynamic lung volume measurements were conducted according to ATS and ERS criteria. With the device, forced vital capacity (FVC)was evaluated.
Time frame: First Day
Pulmonary function (Forced expiratory volume in the first second (FEV1))
Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were conducted according to ATS and ERS criteria. With the device, forced expiratory volume in the first second (FEV1) was evaluated.
Time frame: First day
Pulmonary function (FEV1 / FVC)
Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were conducted according to ATS and ERS criteria. With the device, FEV1 / FVC was evaluated.
Time frame: First day
Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
Pulmonary function was evaluated using the spirometry. Dynamic lung volume measurements were conducted according to ATS and ERS criteria. With the device, flow rate 25-75% of forced expiratory volume (FEF 25-75%)was evaluated.
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Time frame: First Day
Pulmonary function (Peak flow rate (PEF))
Pulmonary function was evaluated using the spirometry. Dynamic lung volume measurements were conducted according to ATS and ERS criteria. With the device, peak flow rate (PEF) was evaluated.
Time frame: First Day
Dyspnea
Subjectively, dyspnea at rest and during activity, orthopnea, paroxysmal nocturnal dyspnea, activities that increase dyspnea, and severity of dyspnea (using the Modified Borg scale) was evaluated.
Time frame: First day
Physical activity (Total energy expenditure)
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second Day
Physical activity (Active energy expenditure (joule / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second Day
Physical activity (Physical activity time (min / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Average metabolic equivalent (MET / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Number of steps (steps / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Time spent lying down (min / day) days))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Sleep time (min / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day