Rosenkranz, Stephan, Pausch, Christine ORCID: 0000-0002-1866-9015, Coghlan, John G., Huscher, Doerte, Pittrow, David, Gruenig, Ekkehard, Staehler, Gerd, Vizza, Carmine Dario ORCID: 0000-0002-3540-4983, Gall, Henning ORCID: 0000-0001-7016-7373, Distler, Oliver, Delcroix, Marion, Ghofrani, Hossain A., Ewert, Ralf, Kabitz, Han -Joachim, Skowasch, Dirk, Behr, Juergen, Milger, Katrin, Halank, Michael, Wilkens, Heinrike, Seyfarth, Hans-Juergen, Held, Matthias, Scelsi, Laura, Neurohr, Claus, Vonk-Noordegraaf, Anton, Ulrich, Silvia ORCID: 0000-0002-5250-5022, Klose, Hans, Claussen, Martin, Eisenmann, Stephan, Schmidt, Kai-Helge, Remppis, Bjoern Andrew, Skride, Andris, Jureviciene, Elena, Gumbiene, Lina, Miliauskas, Skaidrius, Loeffler-Ragg, Judith, Lange, Tobias J., Olsson, Karen M., Hoeper, Marius M. and Opitz, Christian (2023). Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis. J. Heart Lung Transplant., 42 (1). S. 102 - 115. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1557-3117

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Abstract

BACKGROUND: A diagnosis of idiopathic pulmonary arterial hypertension (IPAH) is frequently made in elderly patients who present with comorbidities, especially hypertension, coronary heart disease, diabe-tes mellitus, and obesity. It is unknown to what extent the presence of these comorbidities affects the response to PAH therapies and whether risk stratification predicts outcome in patients with comorbidities.METHODS: We assessed the database of COMPERA, a European pulmonary hypertension registry, to determine changes after initiation of PAH therapy in WHO functional class (FC), 6-minute walking distance (6MWD), brain natriuretic peptide (BNP) or N-terminal fragment of probrain natriuretic pep-tide (NT-pro-BNP), and mortality risk assessed by a 4-strata model in patients with IPAH and no comorbidities, 1-2 comorbidities and 3-4 comorbidities.RESULTS: The analysis was based on 1,120 IPAH patients (n = 208 [19%] without comorbidities, n = 641 [57%] with 1-2 comorbidities, and n = 271 [24%] with 3-4 comorbidities). Improvements in FC, 6MWD, BNP/NT-pro-BNP, and mortality risk from baseline to first follow-up were significantly larger in patients with no comorbidities than in patients with comorbidities, while they were not signifi-cantly different in patients with 1-2 and 3-4 comorbidities. The 4-strata risk tool predicted survival in patients without comorbidities as well as in patients with 1-2 or 3-4 comorbidities.CONCLUSIONS: Our data suggest that patients with IPAH and comorbidities benefit from PAH medica-tion with improvements in FC, 6MWD, BNP/NT-pro-BNP, and mortality risk, albeit to a lesser extent than patients without comorbidities. The 4-strata risk tool predicted outcome in patients with IPAH irrespective of the presence of comorbidities.J Heart Lung Transplant 2023;42:102-114 (c) 2022 The Author(s). Published by Elsevier Inc. on behalf of International Society for Heart and Lung Transplantation. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Rosenkranz, StephanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pausch, ChristineUNSPECIFIEDorcid.org/0000-0002-1866-9015UNSPECIFIED
Coghlan, John G.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huscher, DoerteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pittrow, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gruenig, EkkehardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Staehler, GerdUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vizza, Carmine DarioUNSPECIFIEDorcid.org/0000-0002-3540-4983UNSPECIFIED
Gall, HenningUNSPECIFIEDorcid.org/0000-0001-7016-7373UNSPECIFIED
Distler, OliverUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Delcroix, MarionUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ghofrani, Hossain A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ewert, RalfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kabitz, Han -JoachimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skowasch, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Behr, JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Milger, KatrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Halank, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wilkens, HeinrikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Seyfarth, Hans-JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Held, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scelsi, LauraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neurohr, ClausUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vonk-Noordegraaf, AntonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ulrich, SilviaUNSPECIFIEDorcid.org/0000-0002-5250-5022UNSPECIFIED
Klose, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Claussen, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Eisenmann, StephanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schmidt, Kai-HelgeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Remppis, Bjoern AndrewUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skride, AndrisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jureviciene, ElenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gumbiene, LinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Miliauskas, SkaidriusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Loeffler-Ragg, JudithUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lange, Tobias J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Olsson, Karen M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hoeper, Marius M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Opitz, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-698808
DOI: 10.1016/j.healun.2022.10.003
Journal or Publication Title: J. Heart Lung Transplant.
Volume: 42
Number: 1
Page Range: S. 102 - 115
Date: 2023
Publisher: ELSEVIER SCIENCE INC
Place of Publication: NEW YORK
ISSN: 1557-3117
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
SURVIVALMultiple languages
Cardiac & Cardiovascular Systems; Respiratory System; Surgery; TransplantationMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/69880

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