Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry
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Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry. / Hoeper, Marius M; Pausch, Christine; Grünig, Ekkehard; Klose, Hans; Staehler, Gerd; Huscher, Doerte; Pittrow, David; Olsson, Karen M; Vizza, Carmine Dario; Gall, Henning; Benjamin, Nicola; Distler, Oliver; Opitz, Christian; Gibbs, J Simon R; Delcroix, Marion; Ghofrani, H Ardeschir; Rosenkranz, Stephan; Ewert, Ralf; Kaemmerer, Harald; Lange, Tobias J; Kabitz, Hans-Joachim; Skowasch, Dirk; Skride, Andris; Jureviciene, Elena; Paleviciute, Egle; Miliauskas, Skaidrius; Claussen, Martin; Behr, Juergen; Milger, Katrin; Halank, Michael; Wilkens, Heinrike; Wirtz, Hubert; Pfeuffer-Jovic, Elena; Harbaum, Lars; Scholtz, Werner; Dumitrescu, Daniel; Bruch, Leonhard; Coghlan, Gerry; Neurohr, Claus; Tsangaris, Iraklis; Gorenflo, Matthias; Scelsi, Laura; Vonk-Noordegraaf, Anton; Ulrich, Silvia; Held, Matthias.
in: J HEART LUNG TRANSPL, Jahrgang 39, Nr. 12, 12.2020, S. 1435-1444.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry
AU - Hoeper, Marius M
AU - Pausch, Christine
AU - Grünig, Ekkehard
AU - Klose, Hans
AU - Staehler, Gerd
AU - Huscher, Doerte
AU - Pittrow, David
AU - Olsson, Karen M
AU - Vizza, Carmine Dario
AU - Gall, Henning
AU - Benjamin, Nicola
AU - Distler, Oliver
AU - Opitz, Christian
AU - Gibbs, J Simon R
AU - Delcroix, Marion
AU - Ghofrani, H Ardeschir
AU - Rosenkranz, Stephan
AU - Ewert, Ralf
AU - Kaemmerer, Harald
AU - Lange, Tobias J
AU - Kabitz, Hans-Joachim
AU - Skowasch, Dirk
AU - Skride, Andris
AU - Jureviciene, Elena
AU - Paleviciute, Egle
AU - Miliauskas, Skaidrius
AU - Claussen, Martin
AU - Behr, Juergen
AU - Milger, Katrin
AU - Halank, Michael
AU - Wilkens, Heinrike
AU - Wirtz, Hubert
AU - Pfeuffer-Jovic, Elena
AU - Harbaum, Lars
AU - Scholtz, Werner
AU - Dumitrescu, Daniel
AU - Bruch, Leonhard
AU - Coghlan, Gerry
AU - Neurohr, Claus
AU - Tsangaris, Iraklis
AU - Gorenflo, Matthias
AU - Scelsi, Laura
AU - Vonk-Noordegraaf, Anton
AU - Ulrich, Silvia
AU - Held, Matthias
N1 - Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.
PY - 2020/12
Y1 - 2020/12
N2 - The term idiopathic pulmonary arterial hypertension (IPAH) is used to categorize patients with pre-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, and presence of comorbidities (obesity, hypertension, coronary heart disease, and diabetes mellitus). A 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 pulmonary 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, no 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 3 (n = 434; 51.6%): median age 72 years, 72% males, frequent comorbidities, history of smoking, and 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 3 (unadjusted p < 0.001 for comparison between all groups). The population of patients diagnosed with IPAH is heterogenous. This cluster analysis identified distinct phenotypes, which differed in clinical presentation, response to therapy, and survival.
AB - The term idiopathic pulmonary arterial hypertension (IPAH) is used to categorize patients with pre-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, and presence of comorbidities (obesity, hypertension, coronary heart disease, and diabetes mellitus). A 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 pulmonary 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, no 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 3 (n = 434; 51.6%): median age 72 years, 72% males, frequent comorbidities, history of smoking, and 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 3 (unadjusted p < 0.001 for comparison between all groups). The population of patients diagnosed with IPAH is heterogenous. This cluster analysis identified distinct phenotypes, which differed in clinical presentation, response to therapy, and survival.
U2 - 10.1016/j.healun.2020.09.011
DO - 10.1016/j.healun.2020.09.011
M3 - SCORING: Journal article
C2 - 33082079
VL - 39
SP - 1435
EP - 1444
JO - J HEART LUNG TRANSPL
JF - J HEART LUNG TRANSPL
SN - 1053-2498
IS - 12
ER -