Quality of life аssessment in patients with pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a severe progressive disease with a pure prognosis, which symptoms have a negative impact on patient's quality of life (QoL). Due to the implementation of new effective PAH-specific drugs, currently the task of therapy is not only to prolong the patient's life, but also to improve its quality. The term "QoL" combines information about the main areas of human life: psychological, social, physical and mental well-being. A set of laboratory and instrumental tests is being used to diagnose PAH, but they cannot characterize indicators of QoL. The main tool for studying QoL is a questionnaire. Questionnaires for assessing QoL can be divided into two groups: universal (general) and special (disease-specific). general questionnaires are used to study QoL both in healthy people and in various diseases. Special questionnaires contain specific components for a particular disease, and therefore must be the most sensitive. The first studies of QoL in patients with PAH showed significant disorders in all areas of patients' lives. Currently, many randomized controlled trials of the efficacy of PAH-specific therapy study QoL using various questionnaires as a secondary end-point. SF-36 and EuroQol-5D are used the most commonly among the general questionnaires. Special questionnaires have been developed and validated to assess QoL in patients with PAH relatively recently. Each questionnaire has its advantages and disadvantages. In this review we will consider the possibilities of assessing QoL in patients with PAH using universal (SF-36, EuroQol-5D) and disease-specific for PAH (CAMPHOR, PAH-SYMPACT, EmPHasis-10) questionnaires. These questionnaires have proven to be a valuable instrument in scientific and clinical practice, which is emphasized in actual 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Currently it is of interest to study the relationship between the results of QoL questionnaires and clinical, laboratory and instrumental tests.