Patients with pulmonary arterial hypertension (PAH) have a poor health-related quality of life, a study published in ERJ Open Research confirmed. According to the authors, the physical functioning subscale is independently associated with disease progression, which may be explained by depression.

The roles of health-related quality of life and psychological variables in the progression of PAH have not been carefully quantified. To investigate this relationship, a team of researchers from the University of Montpellier in France led by Arnaud Bourdin, MD, PhD, initiated a 3-year longitudinal cohort study.

They included 55 patients with stable disease and prospectively recorded their standard clinical variables. They also used questionnaires to characterize patients’ psychological status and health-related quality of life. Finally, they recorded disease progression and progression-free survival over 36 months.

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They found that psychological distress was highly prevalent among patients at the start of the study. The physical and mental component summaries of the health-related quality of life were poor but stable over 36 months. Physical functioning was independently associated with disease progression, along with mean pulmonary arterial pressure and cardiac output. Depression was the only independent psychological characteristic associated with physical functioning.

“Identifying and treating depression is a major issue in the management of PAH,” the researchers wrote. They added that the perspective of the patient regarding their health status is essential in decision-making procedures. They stated, “Future studies by our team will focus on the words patients use to describe their disease, moods, behaviors, and [health-related quality of life].”

It is estimated that there are between 15 and 50 cases of PAH per million people worldwide. Even though the overall prognosis has improved due to the development of new medical treatments, mortality remains high. Improving patients’ health-related quality of life is also a priority, the study team wrote.


Halimi L, Suehs CM, Marin G, et al. Health-related quality of life and disease progression in pulmonary arterial hypertension patients: a 3-year study. ERJ Open Res. 2021;7(3):00617-2020. doi:10.1183/23120541.00617-2020