Hoeper, Marius M., Pausch, Christine, Gruenig, 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 ORCID: 0000-0001-6190-4757, Harbaum, Lars, Scholtz, Werner, Dumitrescu, Daniel, Bruch, Leonhard, Coghlan, Gerry, Neurohr, Claus, Tsangaris, Iraklis, Gorenflo, Matthias, Scelsi, Laura, Vonk-Noordegraaf, Anton, Ulrich, Silvia and Held, Matthias (2020). Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry. J. Heart Lung Transplant., 39 (12). S. 1435 - 1445. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1557-3117

Full text not available from this repository.

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: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Hoeper, Marius M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pausch, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gruenig, EkkehardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klose, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Staehler, GerdUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huscher, DoerteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pittrow, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Olsson, Karen M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vizza, Carmine DarioUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gall, HenningUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Benjamin, NicolaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Distler, OliverUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Opitz, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gibbs, J. Simon R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Delcroix, MarionUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ghofrani, H. ArdeschirUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rosenkranz, StephanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ewert, RalfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kaemmerer, HaraldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lange, Tobias J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kabitz, Hans-JoachimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skowasch, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skride, AndrisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jureviciene, ElenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Paleviciute, EgleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Miliauskas, SkaidriusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Claussen, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Behr, JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Milger, KatrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Halank, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wilkens, HeinrikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wirtz, HubertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pfeuffer-Jovic, ElenaUNSPECIFIEDorcid.org/0000-0001-6190-4757UNSPECIFIED
Harbaum, LarsUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scholtz, WernerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dumitrescu, DanielUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bruch, LeonhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Coghlan, GerryUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neurohr, ClausUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tsangaris, IraklisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorenflo, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scelsi, LauraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vonk-Noordegraaf, AntonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ulrich, SilviaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Held, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-309830
DOI: 10.1016/j.healun.2020.09.011
Journal or Publication Title: J. Heart Lung Transplant.
Volume: 39
Number: 12
Page Range: S. 1435 - 1445
Date: 2020
Publisher: ELSEVIER SCIENCE INC
Place of Publication: NEW YORK
ISSN: 1557-3117
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
DIFFUSION CAPACITY; HEART-FAILURE; SURVIVAL; CAPILLARY; DIAGNOSISMultiple languages
Cardiac & Cardiovascular Systems; Respiratory System; Surgery; TransplantationMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/30983

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item