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.

Background: Pulmonary arterial hypertension (PAH) is a rare, chronic, progressive, and life-threatening cardiopulmonary disease. This study investigated the impact of an individually tailored 12 weeks home-based physiotherapy program in PAH patients, with the aim to evaluate change in exercise capacity and daily physical activity level. Methods: This was an analysis of secondary outcomes from a prospective, randomized, controlled intervention study. Twenty-one participants were recruited from the Latvian PH registry based on inclusion criteria and randomized in a training group (TG) and control group (CG). Both groups continued a medical target therapy, but for TG, the individually tailored home-based physiotherapy program was added including physical exercises, relaxation, self-control, education, and supervision with telehealth elements. Outcomes included a 6-min walk test (6MWT) (m) and daily physical activity based on accelerometry results assessed at baseline, after 12 weeks, and at follow-up 6 months after commencement of intervention. Results: 6MWT distance significantly ( p < 0.05) and clinically (>33 m) increased within TG after 12 weeks (51.8 m, 95% CI = 25.7–77.9 m, Cohen’s d = 1.7) and at follow-up (75.5 m, 95% CI = 46.1–104.8 m, Cohen’s d = 2.1). A significant difference in 6MWT results between the groups at 12 weeks and follow-up was approved. In TG, low-intensity activities significantly ( p < 0.05) increased both after 12 weeks (Cohen’s d = 1.6) and at follow-up (Cohen’s d = 1.2), moderate-intensity activities significantly ( p < 0.05) increased at follow-up (Cohen’s d = 1.3), and no significant improvements were present in CG. Conclusion: The individually tailored 12 weeks home-based physiotherapy program comprising comprehensive physical exercise training, relaxation, self-control skills training, and education, added to stable medical target therapy and supervised by physiotherapist through telehealth elements, is effective in improving exercise capacity and increasing daily time in low or moderate physical activities 6 months after commencement of the intervention in patients with PAH.

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