Hoeper, Marius M. and Pausch, Christine and Gruenig, Ekkehard and Klose, Hans and Staehler, Gerd and Huscher, Doerte and Pittrow, David and Olsson, Karen M. and Vizza, Carmine Dario and Gall, Henning and Benjamin, Nicola and Distler, Oliver and Opitz, Christian and Gibbs, J. Simon R. and Delcroix, Marion and Ghofrani, H. Ardeschir and Rosenkranz, Stephan and Ewert, Ralf and Kaemmerer, Harald and Lange, Tobias J. and Kabitz, Hans-Joachim and Skowasch, Dirk and Skride, Andris and Jureviciene, Elena and Paleviciute, Egle and Miliauskas, Skaidrius and Claussen, Martin and Behr, Juergen and Milger, Katrin and Halank, Michael and Wilkens, Heinrike and Wirtz, Hubert and Pfeuffer-Jovic, Elena and Harbaum, Lars and Scholtz, Werner and Dumitrescu, Daniel and Bruch, Leonhard and Coghlan, Gerry and Neurohr, Claus and Tsangaris, Iraklis and Gorenflo, Matthias and Scelsi, Laura and Vonk-Noordegraaf, Anton and Ulrich, Silvia and Held, Matthias (2020) Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry. JOURNAL OF HEART AND LUNG TRANSPLANTATION, 39 (12). pp. 1435-1444. ISSN 1053-2498, 1557-3117
Full text not available from this repository. (Request a copy)Abstract
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% vs >= 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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | DIFFUSION CAPACITY; HEART-FAILURE; SURVIVAL; CAPILLARY; DIAGNOSIS; pulmonary arterial hypertension; phenotypes; cluster; survival; mortality |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Innere Medizin II |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 08 Mar 2021 07:01 |
| Last Modified: | 08 Mar 2021 07:01 |
| URI: | https://pred.uni-regensburg.de/id/eprint/43271 |
Actions (login required)
 |
View Item |
