The primary aim is to evaluate pulmonary function, respiratory muscle strength, upper and lower extremity exercise capacity, muscle oxygenation, dyspnea, peripheral muscle strength, arterial stiffness, physical activity level and balance in children with pulmonary arterial hypertension and compare with age- and sex-matched healthy controls. The second aim is to investigate the relationship between upper extremity exercise capacity, arterial stiffness, respiratory and peripheral muscle strength
Pulmonary arterial hypertension (PAH) is defined as higher than 25 mmHg in mean pulmonary arterial pressure at rest. Pulmonary arterial hypertension is chronic and progressive disease. Symptoms such as dyspnea, fatigue, exercise intolerance, cyanosis and syncope are shown in children with PAH. Most common symptom is dyspnea during exertion. Dyspnea at rest could occur while the severity of disease is progressed. All these symptoms have been associated with decreased cardiac output and mismatch oxygen transport. This study is planned as a cross-sectional study. At least 13 children with PAH and at least 13 age- and sex- matched healthy controls will be included in this study. Individuals' pulmonary function (spirometer), respiratory muscle strength (mouth pressure device), upper extremity exercise capacity (the six minute Peg Board Ring Test), lower extremity exercise capacity (six minute walk test), muscle oxygenation ('Moxy' monitor device), dyspnea (modified Borg Scale), peripheral muscle strength (hand-held dynamometer), arterial stiffness (arteriograph device), physical activity level (multi-sensor activity monitor) and static balance (balance system with computed) will evaluated in children with PAH and healthy controls. All assessments will be completed in two days.
Study Type
OBSERVATIONAL
Enrollment
26
Gazi University Faculty of Health Sciences Department of Physiotherapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit
Ankara, Çankaya, Turkey (Türkiye)
RECRUITINGUpper extremity exercise capacity
Six minutes PegBoard and Ring Test (6-PBRT) will be applied to evaluate upper extremity exercise capacity. There are a total of 20 rings and 2 wooden bars at above and 2 wooden bars at below. Patients will be asked to move the each two rings from upper bars to lower bars and than from lower bars to upper bars with hands as fast as. The total number of rings moved during the test will be recorded at the end of test.
Time frame: First day
Muscle oxygenation
Muscle oxygenation will be evaluated with the Moxy monitor (Moxy, Fortiori Design LLC, Minnesota, USA) as a non-invasive. The device will be attached with soft tape on the participants' skin surface. The device will be placed on the 1/3 lower motor point of the deltoid muscle group of the dominant arm during the the six minutes PegBoard and Ring Test and placed on the 1/3 lower motor point of the quadriceps muscle of dominant leg during the six minutes walk test. At rest and during the test, skeletal muscle oxygenation (StO2) and total hemoglobin (THb) values will be recorded.
Time frame: First day
Respiratory muscle strength
Maximal inspiratory (MIP) and maximal expiratory (MEP) pressures expressing respiratory muscle strength will be evaluated using a portable mouth pressure device. Measurements will be applied according to American Thoracic Society and European Respiratory Society criteria.
Time frame: First day
Peripheral muscle strength
Isometric peripheral muscle strength of quadriceps and deltoid muscles will be measured with a portable hand-held dynamometer (JTECH Commander, USA). Measurements will be repeated three times for both extremities.
Time frame: Second day
Dyspnea
Perception of dyspnea at rest and during the activities of daily living will be assessed with modified Borg scale. The scale is graded between '0' and '10'. The lower score for dyspnea means 'no shortness of breath' and higher score for dyspnea means 'very very hard'.
Time frame: Second day
Lower extremity exercise capacity
Six minute walk test (6-MWT) will be used to evaluate lower extremity exercise capacity. The test will be applied based on American Thoracic Society and European Respiratory Society criteria. The longest walking distance will be recorded to analyze.
Time frame: Second day
Physical activity (Total energy expenditure)
Total energy expenditure, one of the parameters of physical activity, will be evaluated with multisensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the right arm for 3 continuous days. Total energy expenditure will be expressed as joule/day. The parameter measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Active energy expenditure)
Active energy expenditure, one of the parameters of physical activity, will be evaluated with multisensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the right arm for 3 continuous days. Active energy expenditure will be expressed as joule/day. The parameter measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Physical activity duration)
Physical activity duration, one of the parameters of physical activity, will be evaluated with multisensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the right arm for 3 continuous days. Physical activity duration will be expressed as minute/day. The parameter measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Average metabolic equivalent)
Average metabolic equivalent, one of the parameters of physical activity, will be evaluated with multisensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the right arm for 3 continuous days. The parameter measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Number of steps)
Number of steps, one of the parameters of physical activity, will be evaluated with multisensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the right arm for 3 continuous days. Number of steps will be expressed as steps/day. The parameter measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Lying down duration)
Lying down duration, one of the parameters of physical activity, will be evaluated with multisensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the right arm for 3 continuous days. Lying down duration will be expressed as minutes/day. The parameter measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Physical activity (Sleep duration)
Sleep duration, one of the parameters of physical activity, will be evaluated with multisensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the right arm for 3 continuous days. Sleep duration will be expressed as minutes/day. The parameter measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second day
Pulmonary function (Forced expiratory volume in the first second (FEV1))
Pulmonary function will be evaluated with the spirometry based on American Thoracic Society and European Respiratory Society criteria. Forced expiratory volume in the first second (FEV1) will be measured.
Time frame: First day
Pulmonary function (Forced vital capacity (FVC))
Pulmonary function will be evaluated with the spirometry based on American Thoracic Society and European Respiratory Society criteria. Forced vital capacity (FVC) will be measured.
Time frame: First day
Pulmonary function (FEV1 / FVC)
Pulmonary function will be evaluated with the spirometry based on American Thoracic Society and European Respiratory Society criteria. FEV1/FVC will be measured.
Time frame: First day
Pulmonary function (Peak flow rate (PEF))
Pulmonary function will be evaluated with the spirometry based on American Thoracic Society and European Respiratory Society criteria. Peak flow rate (PEF) will be measured.
Time frame: First day
Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
Pulmonary function will be evaluated with the spirometry based on American Thoracic Society and European Respiratory Society criteria. Flow rate 25-75% of forced expiratory volume (FEF 25-75%) will be measured.
Time frame: First day
Balance
Static balance will be evaluated using a balance assessment device.
Time frame: Second day
Arterial Stiffness
Arterial stiffness will be assessed non-invasively with arteriography device. Carotid-femoral artery pulse wave velocity, augmented index, systolic and diastolic pressures and mean arterial pressures of aort and brachial arteries will be measured. Carotid-femoral artery pulse wave velocity will be measured with tonometer on carotid artery and augmented index will be measured with sphygmomanometer on brachial artery.
Time frame: Second day
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