Health-related quality of life and disease progression in pulmonary arterial hypertension patients: a 3-year study

Laurence Halimi, Carey M Suehs, Gregory Marin, Clement Boissin, Anne-Sophie Gamez, Isabelle Vachier, Nicolas Molinari, Arnaud Bourdin, Laurence Halimi, Carey M Suehs, Gregory Marin, Clement Boissin, Anne-Sophie Gamez, Isabelle Vachier, Nicolas Molinari, Arnaud Bourdin

Abstract

Objectives: The role of health-related quality of life (HRQoL) and psychological variables in pulmonary arterial hypertension (PAH) progression remains poorly quantified. We aimed to investigate the relationship between disease progression in PAH patients and HRQoL and psychological characteristics.

Methods: A 3-year longitudinal cohort was initiated. Patients with stable PAH (groups I-IV ineligible for angioplasty/endarterectomy) were included (n=55). Standard clinical variables, including invasive haemodynamic parameters, were prospectively recorded. A battery of questionnaires was used to characterise the psychological status of patients upon study initiation, and HRQoL was quantified using the SF-36 Questionnaire every 3 months for 24 months, and then again at 36 months. Guideline-defined disease progression and progression-free survival were recorded for 36 months.

Measurements and main results: Psychological distress was highly prevalent at baseline. The Physical Component Summary (PCS) and the Mental Component Summary (MCS) of the HRQoL were poor (PCS=37.13±8.18; MCS=42.42±10.88) but stable over 3 years of follow-up. Among PCS subscales, Physical Functioning (PF) (p=0.012) was identified as being independently associated with disease progression (Cox survival model), along with mean pulmonary arterial pressure (p=0.003) and cardiac output (p=0.005). Depression was the unique independent psychological characteristic associated with PF (p=0.0001).

Conclusions: PAH patients have poor HRQoL. In addition to already known criteria related to disease severity, the HRQoL PF subscale is independently associated with disease progression in PAH. This may be explained by depression.

Conflict of interest statement

Conflict of interest: L. Halimi has nothing to disclose. Conflict of interest: C.M. Suehs has nothing to disclose. Conflict of interest: G. Marin has nothing to disclose. Conflict of interest: C. Boissin has nothing to disclose. Conflict of interest: A-S. Gamez has nothing to disclose. Conflict of interest: I. Vachier has nothing to disclose. Conflict of interest: N. Molinari has nothing to disclose. Conflict of interest: A. Bourdin reports grants, personal fees, nonfinancial support and other from AstraZeneca, Boehringer Ingelheim, GlaxoSmithKline and Novartis; personal fees and nonfinancial support from Teva; personal fees, nonfinancial support and other from Regeneron and Chiesi Pharmaceuticals; grants, personal fees, nonfinancial support and other from Actelion; personal fees from Gilead; nonfinancial support and other from Roche; and other from Nuvaira, all outside the submitted work.

Copyright ©The authors 2021.

Figures

FIGURE 1
FIGURE 1
Progression-free survival. Aggravation occurred linearly.
FIGURE 2
FIGURE 2
Physical functioning (PF) subscale of 36-item Short-Form Health Survey (SF-36) values over time. No association with time or the interaction term (stability × time) was found, indicating that the PF subscale was stable throughout the 3 years of complete follow-up.
FIGURE 3
FIGURE 3
Mental Component Summary (MCS) and Physical Component Summary (PCS) values over time. No association with time or the interaction term (stability × time) was found, indicating that PCS and MCS were stable throughout the 3 years of complete follow-up. SF-36: 36-item Short-Form Health Survey.

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