Correlations between clinical variables and quality of life in pulmonary arterial hypertension

Background and objectives: Pulmonary Arterial Hypertension (PAH) is a chronic and disabling illness that impairs patient9s functionality and Quality of Life (QoL). Multiple biomarkers have been used to evaluate functionality and several QoL questionnaires to assess QoL. However, there is limited knowledge about their correlations. Methods: Study participants completed PAH-specific (CAMPHOR) and general (NHP) QoL questionnaires as part of a programmed evaluation and patients9 demographic, clinical and QoL variables were collected. Spearman9s correlation coefficient and Mann-Whitney or Kruskall-Wallis tests were used to investigate possible associations between the variables, as appropriate. Potential determinants of QoL were assessed in a multiple linear regression analysis. Results: Forty-nine adult patients (50.4±13.6 years) with confirmed PAH were included. Main etiologies were: chronic thromboembolic pulmonary hypertension (24.4%), idiopathic/heritable (22.5%), congenital heart disease (22.4%) and connective tissue disorders (14.2%). Most patients (87.8%) have been treated with specific drugs and 40.8% were under oxygen therapy. WHO functional class (FC), 6-minute walking distance (6-MWD), Borg dyspnea index (BDI) and oxygen therapy use significantly correlated with all CAMPHOR domains, but only with some of the NHP sections. Regression analysis showed high coefficient of determination (R 2 ) for all CAMPHOR domains (Symptoms: 0.709; Activities: 0.691; QoL: 0.649) and some NHP sections (Energy: 0.705; Emotional reactions: 0.636; Physical Mobility: 0.659). Conclusions: WHO FC, 6-MWD, BDI and oxygen therapy strongly correlate with all CAMPHOR domains.