Exercise Capacity Muscle Oxygenation and Arterial Stiffness in Children With Pulmonary Arterial Hypertension

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.

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.

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.

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.

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'.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Pulmonary function will be evaluated with the spirometry based on American Thoracic Society and European Respiratory Society criteria. FEV1/FVC will be measured.

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.

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.

Static balance will be evaluated using a balance assessment device.

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.