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.

中文翻译：

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肺动脉高压和慢性血栓栓塞性肺动脉高压患者早期随访时健康相关生活质量的变化

与肺动脉高压 (PAH) 或慢性血栓栓塞性肺动脉高压 (CTEPH) 相关的症状会影响患者的健康相关生活质量 (HRQoL)。缺乏关于变化以及诊断后一年内 HRQoL 是否达到最小临床重要差异 (MCID) 的研究。目的是调查 HRQoL 的变化以及在诊断后早期就诊时达到 MCID 的患者比例。该研究纳入了瑞典 PAH 和 CTEPH 登记处的成年患者，这些患者于 2008 年至 2021 年诊断，并在诊断时进行了剑桥肺动脉高压结果审查 (CAMPHOR) 并进行了随访。数据按总人口进行分析，并按性别、年龄（<65 岁 vs. ≥65 岁）、诊断时间（≤2015 年 vs. >2015 年）和肺动脉高压 (PH) 亚组进行二分。数据以中位数、四分位距 (IQR) 和比例 (%) 形式报告。有 151 名患者（PAH = 119，CTEPH = 32）在诊断和随访时具有可用的 CAMPHOR 评分。CAMPHOR 总和为 31（IQR：21-43）和 25（14-36）；( p  < 0.001) 分别在诊断和随访时。随访时，56% 的人总体达到 MCID，而在活动、症状和生活质量方面，分别有 27%、33% 和 39% 达到 MCID。这些结果与 PH 亚组、2015 年之前或之后的诊断以及性别无关。65 岁以下的年龄与活动改善和症状恶化有关。总而言之，在组水平上，诊断后第一年发现 CAMPHOR 总和以及所有域都有改善，但是，只有略多于一半的患者达到 CAMPHOR 总和的 MCID。