Introduction. Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease that refers to one of the five subgroups of pulmonary hypertension (PH). PAH refers to a group of rare diseases. PAH is characterized by a progressive course of the disease with increased pulmonary vascular resistance and pulmonary arterial pressure, leading to overload and failure of the right ventricle. The initial clinical symptoms of PAH are commonly induced by exertion and can include shortness of breath, excessive fatigue and weakness, chest pain, and episodes of syncope. Pathologic processes in pulmonary arteries alter oxygen uptake and consumption in the body, decreasing SpO2 and restricting the ability to perform physical activities. Various factors including limited exercise tolerance in combination with muscle weakness, as well as sleep disorders, depression, and inability to adapt to the persistent anxiety caused by severe, chronic, and life-threatening disease, lead to poor quality of life and contribute to social isolation. Today, the appropriate target medical treatment 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. This study appears to be the first to develop and investigate a comprehensive structured physiotherapy program, which is expected to be implemented in an interdisciplinary treatment approach in patients with PAH in Latvia. Furthermore, the novelty of the thesis obviously shows in the approach used in the study to assess the quality of life of patients with PAH, including the perceived opportunity to live as one wants to in the context of one’s health condition, thus opening opportunities to shed more light on the perspective of the patient on one’s life with a rare, chronic, and life-threatening disease. The aim of the study was to investigate the effectiveness of the comprehensive home-based and remotely supervised physiotherapy program within the interdisciplinary treatment approach to improve and maintain participation in everyday life activities, along with exercise capacity and inspiratory muscle strength, perceived self-efficacy, daily physical activity and sleep quality in patients with PAH. Methods. This is a prospective, randomized, controlled and single-blind study. We recruited patients from the Latvian PH registry with PAH diagnosed by right heart catheterization based on the study inclusion criteria. We randomly assigned the patients to the training group (TG) or the control group (CG). All participants were evaluated at baseline, after 12 weeks and 24 weeks after the start (follow-up). Blinded assessor conducted the assessments in an on-site visit to the University Clinic, and accelerometer data with MOX sensor from 7 consecutive days were obtained too. All participants continued target medical therapy under the supervision of the study cardiologist specialized in PH. Furthermore, TG underwent the developed comprehensive home-based and remotely supervised physiotherapy program led by the study physiotherapist specialized in cardiopulmonology. As primary outcome, perceived autonomy and participation was assessed with the IPA scale. Furthermore, as secondary outcomes 6 minutes walking tests (6MWT), inspiratory muscle strength, perceived self-efficacy, daily physical activity, and sleep quality were evaluated. The choice of mathematical methods of statistics was made in a stepwise fashion, based on small group recommendations. α level 0.05 was chosen; therefore, the results were determined as statistically significant if p < 0.05. For repeated measures, the significance values were adjusted using the Bonferroni correction and set α level 0.025. To measure the effect size, Cohen’s d or rank biserial coefficient was calculated. For statistically significant results, the post hoc statistical power was calculated and a power of at least 80 % (1-β ≥ 0.8) was assumed to be appropriate to control β error. Results. A significant difference between the groups was found in the follow-up assessment in three of four IPA subscales analysed: autonomy outdoors (p = 0.01, rg = 0.66, 1- β = 0.95), family role (p = 0.04, rg = 0.55, 1-β = 0.8), autonomy indoors (p = 0.04, rg = 0.51, 1-β = 0.68) based on median scores. indicating greater limitations of perceived participation in CG compared to TG. The total IPA score significantly decreased in TG after the program: from baseline to 12 weeks assessment (p = 0.005, d = 1.1, 1-β = 0.96), from baseline to follow-up (p = 0.004, d = 1.1, 1-β = 0.97) pointing to improved participation of patients in their everyday life; no significant changes in CG were observed. A significant increase in 6MWT results in TG after 12 weeks and at follow-up. In contrast, no significant improvement was presented in CG. Significant differences in 6MWT results between the groups at 12 weeks and follow-up were approved. Accelerometry data show a significant reduction in sedentary time from baseline to 12 weeks in both groups, and from baseline to follow-up in TG. No significant differences were observed between the groups in sedentary time. Only in TG a significant increase in low or moderate intensity physical activities was observed from baseline to follow-up, and significant difference between groups at follow-up was present in the mentioned activities. Perceived general self-efficacy increased significantly in TG from baseline to 12 weeks; no improvement was present in CG. At follow-up, statistically significant differences between groups were observed, although the results did not reach the appropriate statistical power. Conclusions. In summary, the comprehensive home-based and remotely supervised physiotherapy program within the interdisciplinary treatment approach in patients with PAH prevents the possible deterioration of the perceived participation of patients in activities of their everyday life in the context of one’s health condition and is a resource to encourage the improvement of the perceived opportunity to live life as one wants, despite the long-term impact of PAH and its treatment.
