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

被引：37

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

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