Health-related quality of life, treatment adherence and psychosocial support in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) share similar quality of life impairment. The aim of the present study was to investigate health-related quality of life (HRQoL) and its relation to the perception of treatment and psychosocial support among PAH and CTEPH patients. All adult PAH or CTEPH patients in the Swedish Pulmonary Arterial Hypertension Register were invited to participate in a national cohort survey. The survey included the EuroQol 5-dimensions (EQ-5D) instrument that measures an individual's HRQoL; the Beliefs about Medicines Questionnaire-Specific Scale that assesses the perception of PAH-specific treatment; the Mastery scale that evaluates the feeling of control and ability to cope with the disease; and the Social Network and Support Scale that maps the social support network. Of the 440 invited patients, 74% responded. Mean age was 66 +/- 14 years, 58% were female and 69% diagnosed with PAH. Patients with PAH were younger, more often female and had a lower EQ-5D index (0.67 +/- 0.29 vs. 0.73 +/- 0.25, p = 0.050) than patients with CTEPH. Patients with a low EQ-5D index had more concerns about treatment (p = 0.004), lower coping ability (p < 0.001), less emotional support (p = 0.003) and less accessible social network (p = 0.002). In conclusion, patients with an impaired HRQoL also reported negative effects on their social support network, ability to handle stressors and concerns about treatment.