Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry
复制标题
DOI：
10.1016/j.healun.2020.09.011
复制
发表时间：
2020-12-01
影响因子：
8.9
通讯作者：
Held, Matthias
中科院分区：
文献类型：
--
作者：
Hoeper, Marius M.;Pausch, Christine;Held, Matthias
特发性肺动脉高压（IPAH）一词用于分类原因不明的毛细血管肺动脉高压患者。这些患者的临床表现有相当大的差异。使用来自肺动脉高压新开始治疗的比较前瞻性登记的数据，我们对841例IPAH患者进行了基于年龄、性别、肺一氧化碳扩散能力（DLCO = 45%预测）、吸烟状况、是否存在合共病（肥胖、高血压、冠心病和糖尿病）的聚类分析。采用Ward最小方差法进行分层聚类算法。根据基线特征对聚类进行分析；生存而生存;以及对肺动脉高压（PAH）治疗的反应，表现为从基线到随访的功能类别、6分钟步行距离、心脏生物标志物和风险的变化。确定了三组：第1组（n = 106; 12.6%）：中位年龄45岁，76%为女性，合并症，大多数从不吸烟，DLCO >= 45%；第2组（301例，占35.8%）：中位年龄75岁，98%为女性，常见合并症，无吸烟史，DLCO多为bb0 = 45%；组群（n = 434, 51.6%）：中位年龄72岁，72%男性，常见合合症，吸烟史，低DLCO。第1组患者对PAH治疗的反应优于其他2组患者。5年以上生存率在第1组为84.6%，第2组为59.2%，第2组为42.2%（各组间比较未校正p < 0.001）。诊断为IPAH的患者群体具有异质性。该聚类分析确定了不同的表型，这些表型在临床表现、治疗反应和生存方面存在差异。(C) 2020作者。由爱思唯尔公司代表国际心肺移植学会出版。
The term idiopathic pulmonary arterial hypertension (IPAH) is used to categorize patients with capillary pulmonary hypertension of unknown origin. There is considerable variability in the clinical presentation of these patients.Using data from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension, we performed a cluster analysis of 841 patients with IPAH based on age, sex, diffusion capacity of the lung for carbon monoxide (DLCO; = 45% predicted), smoking status, presence of comorbidities (obesity, hypertension, coronary heart disease, and diabetes mellitus). hierarchical agglomerative clustering algorithm was performed using Ward's minimum variance method. The clusters were analyzed in terms of baseline characteristics; survival; and response to monary arterial hypertension (PAH) therapy, expressed as changes from baseline to follow-up in functional class, 6-minute walking distance, cardiac biomarkers, and risk.Three clusters were identified: Cluster 1 (n = 106; 12.6%): median age 45 years, 76% females, comorbidities, mostly never smokers, DLCO >= 45%; Cluster 2 (n = 301; 35.8%): median age 75 years, 98% females, frequent comorbidities, no smoking history, DLCO mostly >= 45%; and Cluster (n = 434; 51.6%): median age 72 years, 72% males, frequent comorbidities, history of smoking, low DLCO. Patients in Cluster 1 had a better response to PAH treatment than patients in the 2 other clusters. Survival over 5 years was 84.6% in Cluster 1, 59.2% in Cluster 2, and 42.2% in Cluster (unadjusted p < 0.001 for comparison between all groups).The population of patients diagnosed with IPAH is heterogenous. This cluster analysis identified tinct phenotypes, which differed in clinical presentation, response to therapy, and survival. (C) 2020 The Author(s). Published by Elsevier Inc. on behalf of International Society for Heart and Lung Transplantation.