Ivarsson Bodil, Johansson Anders, Kjellström Barbro
Clinical Sciences Lund University Lund Sweden.
Department of Medicine Karolinska Institute Stockholm Sweden.
Pulm Circ. 2024 Feb 27;14(1):e12349. doi: 10.1002/pul2.12349. eCollection 2024 Jan.
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); ( < 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.
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）。目前缺乏关于HRQoL变化以及在诊断后一年内是否达到最小临床重要差异（MCID）的研究。本研究旨在调查HRQoL的变化以及在诊断后早期随访时达到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，这些患者在2008年至2021年期间被诊断，诊断时和随访时均采用剑桥肺动脉高压结局评估（CAMPHOR）。数据按总体人群进行分析，并按性别、年龄（<65岁与≥65岁）、诊断时间（≤2015年与>2015年）和肺动脉高压（PH）亚组进行二分法分析。数据报告为中位数、四分位数间距（IQR）和比例（%）。共有151例患者（PAH = 119例，CTEPH = 32例）在诊断和随访时有可用的CAMPHOR评分。CAMPHOR总分在诊断时和随访时分别为31（IQR：21 - 43）和25（14 - 36）；（P < 0.001）。在随访时，56%的患者总分达到MCID，而在活动、症状和生活质量领域，分别有27%、33%和39%的患者达到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。总体而言，在诊断后的第一年，CAMPHOR总分以及所有领域均有改善，然而，只有略超过一半的患者CAMPHOR总分达到MCID。
