TY - JOUR
T1 - Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities
T2 - A COMPERA analysis
AU - Rosenkranz, Stephan
AU - Pausch, Christine
AU - Coghlan, John G
AU - Huscher, Doerte
AU - Pittrow, David
AU - Grünig, Ekkehard
AU - Staehler, Gerd
AU - Vizza, Carmine Dario
AU - Gall, Henning
AU - Distler, Oliver
AU - Delcroix, Marion
AU - Ghofrani, Hossain A
AU - Ewert, Ralf
AU - Kabitz, Hans-Joachim
AU - Skowasch, Dirk
AU - Behr, Juergen
AU - Milger, Katrin
AU - Halank, Michael
AU - Wilkens, Heinrike
AU - Seyfarth, Hans-Jürgen
AU - Held, Matthias
AU - Scelsi, Laura
AU - Neurohr, Claus
AU - Vonk-Noordegraaf, Anton
AU - Ulrich, Silvia
AU - Klose, Hans
AU - Claussen, Martin
AU - Eisenmann, Stephan
AU - Schmidt, Kai-Helge
AU - Remppis, Bjoern Andrew
AU - Skride, Andris
AU - Jureviciene, Elena
AU - Gumbiene, Lina
AU - Miliauskas, Skaidrius
AU - Löffler-Ragg, Judith
AU - Lange, Tobias J
AU - Olsson, Karen M
AU - Hoeper, Marius M
AU - Opitz, Christian
N1 - Funding Information:
This work was supported by the German Centre of Lung Research (DZL). COMPERA is funded by unrestricted grants from Acceleron, Bayer, Ferrer, Janssen, and OMT . These companies were not involved in data analysis or the writing of this manuscript.
Publisher Copyright:
© 2022 The Authors
PY - 2023/1
Y1 - 2023/1
N2 - BACKGROUND: A diagnosis of idiopathic pulmonary arterial hypertension (IPAH) is frequently made in elderly patients who present with comorbidities, especially hypertension, coronary heart disease, diabetes 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 peptide (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 significantly 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 medication 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.
AB - BACKGROUND: A diagnosis of idiopathic pulmonary arterial hypertension (IPAH) is frequently made in elderly patients who present with comorbidities, especially hypertension, coronary heart disease, diabetes 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 peptide (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 significantly 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 medication 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.
KW - pulmonary arterial hypertension
KW - 4-strata approach
KW - comorbidities
UR - http://www.scopus.com/inward/record.url?scp=85141238845&partnerID=8YFLogxK
U2 - 10.1016/j.healun.2022.10.003
DO - 10.1016/j.healun.2022.10.003
M3 - Article
C2 - 36333206
SN - 1053-2498
VL - 42
SP - 102
EP - 114
JO - Journal of Heart and Lung Transplantation
JF - Journal of Heart and Lung Transplantation
IS - 1
ER -
