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.
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.