Change in health-related quality of life at early follow-up in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Abstract

Symptoms associated with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) impact patient's health-related quality of life (HRQoL). Studies on change and if a minimal clinically important difference (MCID) in HRQoL is reached within a year after diagnosis are lacking. The aim was to investigate the change in HRQoL as well as the proportion of patients that reached MCID at an early postdiagnosis visit. The study included adult patients from the Swedish PAH & CTEPH registry, diagnosed 2008-2021, with Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) at time of diagnosis and a follow-up. Data were analyzed as total population and dichotomized for sex, age (<65 vs. ≥65 years), time of diagnosis (≤2015 vs. >2015) and pulmonary hypertension (PH) subgroups. Data reported as median, interquartile range (IQR), and proportions (%). There were 151 patients (PAH = 119, CTEPH = 32) with an available CAMPHOR score at diagnosis and follow-up. CAMPHOR total sum was 31 (IQR: 21-43) and 25 (14-36); (p < 0.001) at diagnosis and follow-up, respectively. At follow-up, 56% had reached MCID in total sum, while for domains activity, symptoms, and QoL 27%, 33%, and 39% reached MCID, respectively. These results were independent of PH subgroup, diagnosis before or after 2015 and sex. Age below 65 years was related to improvements in activity and worsening of symptoms. In conclusion on a group level, improvements in CAMPHOR total sum as well as all domains were seen in the first year after diagnosis, however, only slightly more than half of the patients reached MCID for CAMPHOR total sum.

Keywords: chronic illness; disease management; newly diagnosed; patient‐reported outcome measures.

Figure 1

Relative change in Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) scores from diagnosis to the follow‐up visit shown by age (a) (<65 years = 54, ≥65 years = 97) and sex (b) (female = 97, male = 54). Minimal clinically important difference (MCID) were total sum = 4 points, activity = 4 points, symptoms = 4 points, and quality of life = 3 points. An MCID decrease in score was considered improvement and an MCID increase in score was considered worsened.

Figure 2

Relative change in Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) scores from diagnosis to the follow‐up visit shown by diagnosis subgroup (a) (idiopathic pulmonary arterial hypertension [IPAH], n = 52; associated pulmonary arterial hypertension[APAH]‐connective tissue disease [CTD] = 56) and diagnosis year (b) (≤2015 = 60, >2015 = 91). Minimal clinically important difference (MCID) were total sum = 4 points, activity = 4 points, symptoms = 4 points, and quality of life = 3 points. An MCID decrease in score was considered improvement and an MCID increase in score was considered worsened.