Factors of impaired health-related quality of life in patients with precapillary pulmonary hypertension

INTRODUCTION Pulmonary hypertension (PH) is related to a variety of symptoms like dyspnea at rest, fatigue and exercise intolerance, all of which have a detrimental influence on patients' quality of life (QOL). Disease-specific health-related QOL (HRQOL) questionnaires are useful tools to objectively estimate the functional and psychological status of PH patients. The purpose of this study was to identify potential factors affecting physical and mental HRQOL in patients with precapillary PH. METHODS We conducted a prospective cross-sectional analysis of HRQOL questionnaires in individuals with precapillary PH (PH-Groups 1, 3, or 4) using the Short form 36-item health survey (SF-36) and the Minnesota living with pulmonary hypertension questionnaire (MLHF-PH). Between January and February 2019, 73 consecutive patients, being followed up in two Greek PH centers, being clinically stable at the last three months and receiving PHspecific therapies based on the European PH Guidelines, completed both questionnaires. RESULTS Patients with PH due to lung disease (PH-Group 3) presented significantly worse scores on MLHF-PH and significantly reduced scores in SF36 'physical functioning' and 'role physical' compared to the other PH-Groups. These patients were more frequently under oxygen therapy (p<0.001) and had a more advanced WHO FC (p=0.01). Oxygen treatment, WHO FC, 6MWD, and hemodynamic variables were also strongly associated with HRQOL. Patients receiving triple combination PH-therapy reported lower HRQOL scores. CONCLUSIONS Patients with precapillary PH had impaired QOL. Those with PH due to lung disease reported the worst scores in HRQOL instruments. Several clinical, functional and hemodynamic factors were associated with reduced QOL.