Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis

被引：38

作者：

Rosenkranz, Stephan ^{[1
,2
,3
]}

Pausch, Christine ^{[4
]}

Coghlan, John G. ^{[5
]}

Huscher, Doerte ^{[6
,7
]}

Pittrow, David ^{[4
,8
]}

Gruenig, Ekkehard ^{[9
]}

Staehler, Gerd ^{[10
]}

Vizza, Carmine Dario ^{[11
]}

Gall, Henning ^{[12
,13
,14
]}

Distler, Oliver ^{[15
]}

Delcroix, Marion ^{[16
,17
]}

Ghofrani, Hossain A. ^{[12
,13
,14
,18
]}

Ewert, Ralf ^{[19
]}

Kabitz, Han -Joachim ^{[20
]}

Skowasch, Dirk ^{[21
]}

Behr, Juergen ^{[22
,23
]}

Milger, Katrin ^{[23
]}

Halank, Michael ^{[24
]}

Wilkens, Heinrike ^{[25
]}

Seyfarth, Hans-Juergen ^{[26
]}

Held, Matthias ^{[27
]}

Scelsi, Laura ^{[28
]}

Neurohr, Claus ^{[29
]}

Vonk-Noordegraaf, Anton ^{[30
]}

Ulrich, Silvia ^{[31
]}

Klose, Hans ^{[32
]}

Claussen, Martin ^{[33
]}

Eisenmann, Stephan ^{[34
]}

Schmidt, Kai-Helge ^{[35
,36
]}

Remppis, Bjoern Andrew ^{[37
]}

Skride, Andris ^{[38
]}

Jureviciene, Elena ^{[39
]}

Gumbiene, Lina ^{[39
]}

Miliauskas, Skaidrius ^{[40
]}

Loeffler-Ragg, Judith ^{[41
]}

Lange, Tobias J. ^{[42
]}

Olsson, Karen M. ^{[14
,43
]}

Hoeper, Marius M. ^{[14
,43
]}

Opitz, Christian ^{[26
,44
]}

机构：

[1] Univ Cologne, Clin Internal Med 3, Cardiol, Cologne, Germany

[2] Univ Cologne, Ctr Mol Med CMMC, Cologne, Germany

[3] Univ Cologne, Cologne Cardiovasc Res Ctr CCRC, Cologne, Germany

[4] GWT TUD GmbH, Epidemiol Ctr, Dresden, Germany

[5] Royal Free Hosp, Dept Cardiol, London, England

[6] Charite, Inst Biometry & Clin Epidemiol, Berlin, Germany

[7] Charitte Univ Med, Berlin Inst Hlth, Berlin, Germany

[8] Tech Univ, Med Fac, Inst Clin Pharmacol, Dresden, Germany

[9] German Ctr Lung Res DZL, Heidelberg Univ Hosp, Ctr Pulm Hypertens, Translat Lung Res Ctr Heidelberg TLRC,Thoraxklin, Heidelberg, Germany

[10] Fachklin Lowenstein, Dept Resp Med, Lowenstein, Germany

[11] Sapienza Univ Rome, Dipartimento Sci Clin Internist Anestiol & Cardio, Rome, Italy

[12] Univ Giessen, Justus Liebig Univ Giessen, Dept Internal Med, Giessen, Germany

[13] Marburg Lung Ctr UGMLC, Giessen, Germany

[14] German Ctr Lung Res DZL, Berlin, Germany

[15] Univ Hosp, Dept Rheumatol, Zurich, Switzerland

[16] Univ Hosp Leuven, Clin Dept Resp Dis, Leuven, Belgium

[17] Univ Leuven, KU Leuven, Lab Resp Dis & Thorac Surg BREATHE, Dept Chron Dis & Metab CHROMETA, Leuven, Belgium

[18] Imperial Coll London, Dept Med, London, England

[19] Univ Med Grewald, Dept Resp Med, Clin Internal Med, Grewald, Germany

[20] Gemeinnutzige Krankenhausbetriebsgesellschaft Kon, Med Klin 2, Constance, Germany

[21] Univ Klinikum Bonn, Med Klin & Poliklin 2, Innere Med Kardiol Pneumol, Bonn, Germany

[22] Helmholtz Zentrum, Comprehens Pneumol Ctr, Lungenforsch Ambulanz, Munich, Germany

[23] Ludwig Maximilians Univ Munchen, Comprehens Pneumol Ctr Munich CPC M, Univ Hosp, Dept Med 5, Munich, Germany

[24] Techn Univ Dresden, Med Klin & Poliklin 1, Univ Klinikum Carl Gustav Carus, Dresden, Germany

[25] Univ Klinikum Saarlandes, Klin Innere Med 5, Pneumol, Homburg, Germany

[26] Univ Klinikum Leipzig, Med Klin & Poliklin 2, Abt Pneumol, Leipzig, Germany

[27] Cent Clin Wurzburg, Med Miss Hosp, Dept Internal Med Resp Med & Ventilatory Support, Wurzburg, Germany

[28] Azienda Sanit Univ Giuliano Isontina, Fdn IRCSS S Matteo Pavia, Div Cardiol Stolfo Davide, Pavia, Italy

[29] Robert Bosch Krankenhaus Stuttgart, Dept Pulmonol & Resp Med, Stuttgart, Germany

[30] Vrije Univ Amsterdam, Amsterdam UMC, Dept Pulm Med, Amsterdam Cardiovasc Sci, Amsterdam, Netherlands

[31] Univ Hosp Zurich, Clin Pulmonol, Zurich, Switzerland

[32] Eppendorf Univ Hosp, Dept Resp Med, Hamburg, Germany

[33] LungenClin Grosshansdorf, Fachabteilung Pneumol, Grohansdorf, Germany

[34] Univ Klinikum Halle, Dept Resp Med, Halle, Germany

[35] Univ Med Ctr Mainz, Dept Cardiol, Mainz, Germany

[36] Univ Med Ctr Mainz, Ctr Thrombosis & Hemostasis CTH, Mainz, Germany

[37] Herz & Gefazentrum Bad Bevensen, Bad Bevensen, Germany

[38] Riga Stradins Univ, Rare Dis Unit VSIA Pauls Stradins Clin Univ Hosp, Riga, Latvia

[39] Vilnius Univ, Vilnius Univ Hosp Santaros Klin, Competence Ctr Pulm Hypertens, Fac Med, Vilnius, Lithuania

[40] Lithuanian Univ Hlth Sci, Dept Pulmonol, Kaunas, Lithuania

[41] Med Univ Innsbruck, Dept Internal Med 2, Innsbruck, Austria

[42] Univ Med Ctr Regensburg, Dept Internal Med 2, Regensburg, Germany

[43] Hannover Med Sch, Dept Resp Med, Carl Neuberg Str 1, D-30623 Hannover, Germany

[44] DRK Kliniken Berlin Westend, Dept Cardiol, Berlin, Germany

来源：

JOURNAL OF HEART AND LUNG TRANSPLANTATION | 2023年 / 42卷 / 01期

关键词：

pulmonary arterial hypertension; kt; risk; 4-strata approach; mortality; comorbidities; SURVIVAL;

D O I：

10.1016/j.healun.2022.10.003

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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/)

引用

页码：102 / 114

页数：13

共 18 条

[1] External validation of a refined four-stratum risk assessment score from the French pulmonary hypertension registry
Boucly, Athenais
Weatherald, Jason
Savale, Laurent
de Groote, Pascal
Cottin, Vincent
Prevot, Gregoire
Chaouat, Ari
Picard, Francois
Horeau-Langlard, Delphine
Bourdin, Arnaud
Jutant, Etienne-Marie
Beurnier, Antoine
Jevnikar, Mitja
Jais, Xavier
Simonneau, Gerald
Montani, David
Sitbon, Olivier
Humbert, Marc
[J]. EUROPEAN RESPIRATORY JOURNAL, 2022, 59 (06)
[2] Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension
Boucly, Athenais
Weatherald, Jason
Savale, Laurent
Jais, Xavier
Cottin, Vincent
Prevot, Gregoire
Picard, Francois
de Groote, Pascal
Jevnikar, Mitja
Bergot, Emmanuel
Chaouat, Ari
Chabanne, Celine
Bourdin, Arnaud
Parent, Florence
Montani, David
Simonneau, Gerald
Humbert, Marc
Sitbon, Olivier
[J]. EUROPEAN RESPIRATORY JOURNAL, 2017, 50 (02)
[3] The Changing Picture of Patients With Pulmonary Arterial Hypertension in the United States How REVEAL Differs From Historic and Non-US Contemporary Registries
Frost, Adaani E.
Badesch, David B.
Barst, Robyn J.
Benza, Raymond L.
Elliott, Gregory
Farber, Harrison W.
Krichman, Abby
Liou, Theodore G.
Raskob, Gary E.
Wason, Prieya
Feldkircher, Kathleen
Turner, Michelle
McGoon, Michael D.
[J]. CHEST, 2011, 139 (01) : 128 - 137
[4] Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension
Galie, N.
Barbera, J. A.
Frost, A. E.
Ghofrani, H-A.
Hoeper, M. M.
McLaughlin, V. V.
Peacock, A. J.
Simonneau, G.
Vachiery, J-L.
Gruenig, E.
Oudiz, R. J.
Vonk-Noordegraaf, A.
White, R. J.
Blair, C.
Gillies, H.
Miller, K. L.
Harris, J. H. N.
Langley, J.
Rubin, L. J.
[J]. NEW ENGLAND JOURNAL OF MEDICINE, 2015, 373 (09) : 834 - 844
[5] Risk stratification and medical therapy of pulmonary arterial hypertension
Galie, Nazzareno
Channick, Richard N.
Frantz, Robert P.
Gruenig, Ekkehard
Jing, Zhi Cheng
Moiseeva, Olga
Preston, Ioana R.
Pulido, Tomas
Safdar, Zeenat
Tamura, Yuichi
McLaughlin, Vallerie V.
[J]. EUROPEAN RESPIRATORY JOURNAL, 2019, 53 (01)
[6] 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
Galie, Nazzareno
Humbert, Marc
Vachiery, Jean-Luc
Gibbs, Simon
Lang, Irene
Torbicki, Adam
Simonneau, Gerald
Peacock, Andrew
Noordegraaf, Anton Vonk
Beghetti, Maurice
Ghofrani, Ardeschir
Sanchez, Miguel Angel Gomez
Hansmann, Georg
Klepetko, Walter
Lancellotti, Patrizio
Matucci, Marco
McDonagh, Theresa
Pierard, Luc A.
Trindade, Pedro T.
Zompatori, Maurizio
Hoeper, Marius
[J]. EUROPEAN RESPIRATORY JOURNAL, 2015, 46 (04) : 903 - 975
[7] Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension
Hjalmarsson, Clara
Radegran, Goran
Kylhammar, David
Rundqvist, Bengt
Multing, Jonas
Nisell, Magnus D.
Kjellstrom, Barbro
[J]. EUROPEAN RESPIRATORY JOURNAL, 2018, 51 (05)
[8] COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension
Hoeper, Marius M.
Pausch, Christine
Olsson, Karen M.
Huscher, Doerte
Pittrow, David
Gruenig, Ekkehard
Staehler, Gerd
Vizza, Carmine Dario
Gall, Henning
Distler, Oliver
Opitz, Christian
Gibbs, J. Simon R.
Delcroix, Marion
Ghofrani, H. Ardeschir
Park, Da-Hee
Ewert, Ralf
Kaemmerer, Harald
Kabitz, Hans-Joachim
Skowasch, Dirk
Behr, Juergen
Milger, Katrin
Halank, Michael
Wilkens, Heinrike
Seyfarth, Hans-Juergen
Held, Matthias
Dumitrescu, Daniel
Tsangaris, Iraklis
Vonk-Noordegraaf, Anton
Ulrich, Silvia
Klose, Hans
Claussen, Martin
Lange, Tobias J.
Rosenkranz, Stephan
[J]. EUROPEAN RESPIRATORY JOURNAL, 2022, 60 (01)
[9] Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry
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
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
[J]. JOURNAL OF HEART AND LUNG TRANSPLANTATION, 2020, 39 (12) : 1435 - 1444
[10] Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model
Hoeper, Marius M.
Kramer, Tilmann
Pan, Zixuan
Eichstaedt, Christina A.
Spiesshoefer, Jens
Benjamin, Nicola
Olsson, Karen M.
Meyer, Katrin
Vizza, Carmine Dario
Vonk-Noordegraaf, Anton
Distler, Oliver
Opitz, Christian
Gibbs, J. Simon R.
Delcroix, Marion
Ghofrani, H. Ardeschir
Huscher, Doerte
Pittrow, David
Rosenkranz, Stephan
Gruenig, Ekkehard
[J]. EUROPEAN RESPIRATORY JOURNAL, 2017, 50 (02)

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