Study: Health-related Quality of Life in PAH Stays Stable Over Time

Marisa Wexler MS avatar

by Marisa Wexler MS |

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Health-related quality of life does not change substantially over time among people with pulmonary arterial hypertension (PAH) — though PAH patients who experience more substantial disease progression tend to report worse quality of life scores — a small study done at a hospital in France has found.

These findings indicate that measuring health-related quality of life might be a useful prognostic factor for clinicians, according to researchers, who say the results place new emphasis on the importance of understanding patients’ life stories when providing psychological support.

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The study, “Health-related quality of life and disease progression in pulmonary arterial hypertension patients: a 3-year study,” was published in the journal ERJ Open Research.

Called HRQoL, health-related quality of life refers to all the ways that a person’s health affects how much he or she enjoys living. Studies on PAH have frequently reported relatively low HRQoL among patients — however, until now, no PAH study has regularly monitored how HRQoL tends to change for these patients over a relatively long time span.

Given that limited data, “it is therefore still unclear whether poor HRQoL in PAH should be seen as a cofactor and eventually as an expected consequence of the disease or if it should be seen as a prognostic criterion,” the researchers wrote.

To address this knowledge gap, a team from the Université de Montpellier, in southern France, conducted a three-year study to evaluate HRQoL in PAH patients with stable disease compared with those who experienced acute disease worsening.

The team followed 55 people with PAH, with a mean age of 57.8. Nearly two-thirds (64%) were women. At baseline, or the start of the study, just over half (51%) had clinically significant anxiety and depression.

The patients regularly underwent a variety of PAH-related health assessments — for example, measures of heart function — during the study. In addition, HRQoL was assessed every three months for two years, and then again at year three, using a standardized questionnaire called the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36).

Over the study’s three years, 25 of the patients experienced disease progression, while the remaining 30 had relatively stable disease. Notably, individuals in the stable group generally reported better HRQoL at baseline, while those whose disease was unstable reported higher scores related to anxiety.

Also of note, in the overall group, patients reported dealing with stressful life events mainly through problem-coping. In the disease-progression group, however, participants were more likely to report coping strategies that focused on emotional responses.

Such coping strategies are “usually linked to poor HRQoL and associated with symptoms of depression and anxiety,” the researchers wrote.

“People living with PAH are particularly vulnerable when they are newly diagnosed or when they have to deal with the loss of a fellow patient or with a change in treatment,” the investigators said. “Emotion-focused coping may be the only realistic option when the source of stress is outside the person’s control.”

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Statistical analyses showed that every subscale on the SF-36, except for Bodily Pain and General Health, was significantly associated with disease progression — in other words, individuals with worse HRQoL were more likely to have disease progression.

Further analyses demonstrated that, over the three years of the study, HRQoL scores did not change much. The difference between those with and without disease progression was comparable at every time point in the study.

“Surprisingly, HRQoL did not statistically decline over time. However, baseline values were associated with disease progression,” the researchers wrote. “These results suggest that HRQoL monitoring can provide additional useful and complementary information to the traditional PAH work-up, but does not need to be carried out at overly frequent intervals.”

The findings imply that HRQoL is independently linked with disease progression in PAH, the team added, suggesting that HRQoL itself could be used as a prognostic marker.

According to the team, the results highlight the importance of “trait anxiety” — that is, the tendency to be anxious, as a part of an individual’s personality — in determining HRQoL.

Trait anxiety is “a nonmodifiable and functional personality trait,” the researchers wrote.

Prior studies had already drawn attention to anxiety brought on by a person’s circumstances, called “state anxiety,” in people with PAH.

The results of this study have important implications for psychological supports meant to help people with PAH, the researchers said. Specifically, behavioral and cognitive therapies, which tend to focus on state anxiety, may be less helpful than “in-depth work on [the patient’s] life story,” they wrote.

The key limitation of this study, the team noted, was that it was conducted at a single hospital, involving a small group of patients. Thus, the results may not be reflective of all PAH patients.

Additionally, during the study, patients who desired it were allowed access to treatments for anxiety and depression, which may have impacted the outcomes, the researchers said, noting that as another limitation.

“HRQoL is important complementary information in the work-up of PAH patients,” the researchers concluded, saying it “should be further studied to elucidate its prognostic potential.”