Background and Objectives: Pulmonary arterial hypertension (PAH) is a rare, chronic, progressive, and life-threatening disease; however, the appropriate target medical treatment today allows patients with PAH not only to survive but also to live a relatively normal life. However, patients face the challenge of adapting and maintaining a good quality of life, thus it is important to consider complex interventions related not only to medical treatment. Methods: This was a prospective, randomized, controlled, single-blind study. Twenty-one (21) patients diagnosed with PAH were included and randomly assigned to training or control group. All participants continued target medical therapy. Furthermore, TG underwent the individually tailored 12-week remote physiotherapy program. As a primary outcome measurement, the Impact on Participation and Autonomy Questionnaire (IPA) was used. Secondary outcome included aerobic capacity (6MWT), accelerometery and general self-efficacy (GSE). Data were collected at baseline, after 12 weeks and at follow-up 6 months after the beginning of the intervention. Results: A significant difference between the groups was found in the follow-up assessment on three of the four IPA subscales analyzed, AO, RF, and AI. The total IPA score decreased significantly in TG after the program, indicating an improved participation. In addition, in TG a significant increase in 6MWT results, daily time in low- or moderate-intensity physical activities, and GSE was observed. Conclusions: In summary, the individually tailored physiotherapy program investigated added to stable target medical therapy in patients with PAH encourages improvement and prevents possible deterioration of perceived participation of patients in activities of their everyday life in the context of one's health condition in the long term, along with improved exercise capacity and increased time spent in low- or moderate-intensity physical activities. Future studies are needed to develop and evaluate long-term intervention to support patients living with this rare, chronic, and life-threatening disease.
Keywords: chronic disease; exercise; home-based; individualized; interdisciplinary; participation; physical activity; physiotherapy; pulmonary arterial hypertension; quality of life; self-efficacy.