Abstract
Abbreviated versions of the ESC/ERS pulmonary hypertension guidelines risk stratification have been recently validated in patients with pulmonary arterial hypertension. We aimed to investigate their prognostic value in medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients from the COMPERA registry, which collects six variables of interest (WHO functional class, 6-minute walking distance, brain natriuretic peptide, right atrial pressure, cardiac index and mixed venous oxygen saturation).
We included patients with at least one follow-up visit, no pulmonary endarterectomy, and at least three of the six variables available, and classified the patients into low, intermediate and high risk groups. As secondary analysis, the number of non-invasive low risk criteria was counted. The association between risk assessment and survival was evaluated.
Data from inclusion and follow-up (median, 7 months) visits were available for 561 and 231 patients, respectively. Baseline 1- and 5-year survival estimates were significantly different (p<0.0001) in the baseline low (98.6;88.3%), intermediate (94.9;61.8%), and high risk (75.5;32.9%) cohorts. Follow-up data were even more discriminative with, respectively, 100, 92 and 69% 1-year survival. The number of low risk non-invasive criteria was also associated with survival.
These analyses suggest that the ESC/ERS risk assessment may be applicable in patients with medically treated CTEPH.
Footnotes
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Conflict of interest: Dr. Delcroix reports grants and personal fees from Actelion, personal fees from Bayer, personal fees from GSK, personal fees from MSD, personal fees from Reata, personal fees from Bellarophon, personal fees from Eli Lilly, outside the submitted work.
Conflict of interest: I have received speakers honoraria from Bayer, Actelion
Conflict of interest: Dr. Gall reports grants and personal fees from Actelion, AstraZeneca, Bayer, BMS, GSK, Janssen-Cilag, Lilly, MSD, Novartis, OMT, Pfizer, and United Therapeutics, during the conduct of the study.
Conflict of interest: Prof. Grünig reports grants and personal fees from Actelion, grants and personal fees from Bayer / MSD, grants from GSK, personal fees from SCOPE, personal fees from OrPha Swiss GmbH, personal fees from Zurich Heart House, grants from United Therapeutics, grants from Novartis, grants from Actelion, Bayer, GSK and MSD, outside the submitted work.
Conflict of interest: Dr. Held reports grants and other from Actelion, other from Actelion, Bayer Healthcare, Berlin Chemie, Boehringer Ingelheim, GSK, MSD, Novartis, Pfizer, other from Actelion, Bayer, GSK, Pfizer, United Therapeutics, other from Actelion, Bayer, Boehringer, GSK, MSD, outside the submitted work.
Conflict of interest: Dr. Halank reports personal fees and non-financial support from Actelion, Bayer, GlaxoSmithKline, MSD Sharp & Dohme, Novartis, and OMT (optimal medical therapies), outside the submitted work.
Conflict of interest: Dr. Klose reports grants and personal fees from Bayer, personal fees from MSD, grants and personal fees from Actelion, personal fees from GSK, personal fees from United Therapeutics, outside the submitted work.
Conflict of interest: Dr. Rosenkranz reports grants and personal fees from Actelion, grants and personal fees from Bayer, personal fees from GSK, grants and personal fees from Pfizer, grants and personal fees from Novartis, personal fees from Gilead, personal fees from MSD, grants and personal fees from United Therapeutics, during the conduct of the study.
Conflict of interest: Dr. Pepk-Zaba reports grants from Compera-Registry during the conduct of the study; grants, personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Bayer, personal fees and non-financial support from MERCK.
Conflict of interest: Dr. Opitz reports other from Actelion, other from Bayer, other from MSD, outside the submitted work.
Conflict of interest: Dr. Gibbs reports grants and personal fees from Actelion, personal fees from Arena, grants and personal fees from Bayer, grants and personal fees from GSK, grants and personal fees from MSD, personal fees from Pfizer, grants from United Therapeutics, personal fees from Bellepheron, during the conduct of the study.
Conflict of interest: Dr. Lange reports grants from Compera-Registry, during the conduct of the study; grants, personal fees and non-financial support from Actelion, personal fees and non-financial support from AOP orphan pharmaceuticals, grants, personal fees and non-financial support from Bayer, grants, personal fees and non-financial support from GSK, personal fees and non-financial support from MSD, grants, personal fees and non-financial support from Pfizer, grants and personal fees from United Therapeutics, outside the submitted work.
Conflict of interest: Dr. Tsangaris reports grants, personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from GSK, grants, personal fees and non-financial support from Bayer, personal fees and non-financial support from MSD, personal fees and non-financial support from United Therapeutics, outside the submitted work.
Conflict of interest: Dr. Huscher reports personal fees from Actelion Pharmaceuticals Ltd, Allschwil, Switzerland, outside the submitted work.
Conflict of interest: Dr. Pittrow reports personal fees from Actelion, personal fees from Bayer, personal fees from Aspen, personal fees from Boehringer Ingelheim, personal fees from Astra Zeneca , personal fees from MSD, outside the submitted work.
Conflict of interest: Dr. Olsson reports personal fees from Actelion, personal fees from Bayer, personal fees from GSK, personal fees from Pfizer, personal fees from United therapeutics, outside the submitted work.
Conflict of interest: Dr. Hoeper reports personal fees from Actelion, personal fees from Bayer, personal fees from Gilead, personal fees from GSK, personal fees from MSD, personal fees from Pfizer, outside the submitted work.
Conflict of interest: Dr. vonk noordegraaf reports grants from NWO-VICI, speaker fees from Actelion and consultation fees from Arena, outside the submitted work.
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