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, Vol. 39, No. 12, 12.2020, p. 1435-1444.

Research output: SCORING: Contribution to journalSCORING: Journal articleResearchpeer-review

Harvard

Hoeper, MM, Pausch, C, Grünig, E, Klose, H, Staehler, G, Huscher, D, Pittrow, D, Olsson, KM, Vizza, CD, Gall, H, Benjamin, N, Distler, O, Opitz, C, Gibbs, JSR, Delcroix, M, Ghofrani, HA, Rosenkranz, S, Ewert, R, Kaemmerer, H, Lange, TJ, Kabitz, H-J, Skowasch, D, Skride, A, Jureviciene, E, Paleviciute, E, Miliauskas, S, Claussen, M, Behr, J, Milger, K, Halank, M, Wilkens, H, Wirtz, H, Pfeuffer-Jovic, E, Harbaum, L, Scholtz, W, Dumitrescu, D, Bruch, L, Coghlan, G, Neurohr, C, Tsangaris, I, Gorenflo, M, Scelsi, L, Vonk-Noordegraaf, A, Ulrich, S & Held, M 2020, 'Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry', J HEART LUNG TRANSPL, vol. 39, no. 12, pp. 1435-1444. https://doi.org/10.1016/j.healun.2020.09.011

APA

Hoeper, M. M., Pausch, C., Grünig, E., Klose, H., Staehler, G., Huscher, D., Pittrow, D., Olsson, K. M., Vizza, C. D., Gall, H., Benjamin, N., Distler, O., Opitz, C., Gibbs, J. S. R., Delcroix, M., Ghofrani, H. A., Rosenkranz, S., Ewert, R., Kaemmerer, H., ... Held, M. (2020). Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry. J HEART LUNG TRANSPL, 39(12), 1435-1444. https://doi.org/10.1016/j.healun.2020.09.011

Vancouver

Bibtex

@article{e1b1ad2c9cd84c78b6505d62cf5260bc,
title = "Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry",
abstract = "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.",
author = "Hoeper, {Marius M} and Christine Pausch and Ekkehard Gr{\"u}nig and Hans Klose and Gerd Staehler and Doerte Huscher and David Pittrow and Olsson, {Karen M} and Vizza, {Carmine Dario} and Henning Gall and Nicola Benjamin and Oliver Distler and Christian Opitz and Gibbs, {J Simon R} and Marion Delcroix and Ghofrani, {H Ardeschir} and Stephan Rosenkranz and Ralf Ewert and Harald Kaemmerer and Lange, {Tobias J} and Hans-Joachim Kabitz and Dirk Skowasch and Andris Skride and Elena Jureviciene and Egle Paleviciute and Skaidrius Miliauskas and Martin Claussen and Juergen Behr and Katrin Milger and Michael Halank and Heinrike Wilkens and Hubert Wirtz and Elena Pfeuffer-Jovic and Lars Harbaum and Werner Scholtz and Daniel Dumitrescu and Leonhard Bruch and Gerry Coghlan and Claus Neurohr and Iraklis Tsangaris and Matthias Gorenflo and Laura Scelsi and Anton Vonk-Noordegraaf and Silvia Ulrich and Matthias Held",
note = "Copyright {\textcopyright} 2020 The Authors. Published by Elsevier Inc. All rights reserved.",
year = "2020",
month = dec,
doi = "10.1016/j.healun.2020.09.011",
language = "English",
volume = "39",
pages = "1435--1444",
journal = "J HEART LUNG TRANSPL",
issn = "1053-2498",
publisher = "Elsevier USA",
number = "12",

}

RIS

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 -