Hoeper, Marius M., Pausch, Christine, Gruenig, Ekkehard, Staehler, Gerd, Huscher, Doerte, Pittrow, David, Olsson, Karen M., Vizza, Carmine Dario ORCID: 0000-0002-3540-4983, Gall, Henning ORCID: 0000-0001-7016-7373, Distler, Oliver, Opitz, Christian, Gibbs, J. Simon R., Delcroix, Marion, Ghofrani, H. Ardeschir, Rosenkranz, Stephan, Park, Da-Hee ORCID: 0000-0003-0902-4773, Ewert, Ralf, Kaemmerer, Harald, Lange, Tobias J., Kabitz, Hans-Joachim, Skowasch, Dirk, Skride, Andris, Claussen, Martin, Behr, Juergen, Milger, Katrin ORCID: 0000-0003-2914-8773, Halank, Michael, Wilkens, Heinrike, Seyfarth, Hans-Juergen, Held, Matthias, Dumitrescu, Daniel, Tsangaris, Iraklis, Vonk-Noordegraaf, Anton, Ulrich, Silvia and Klose, Hans (2022). Temporal trends in pulmonary arterial hypertension: results from the COMPERA registry. Eur. Resp. J., 59 (6). SHEFFIELD: EUROPEAN RESPIRATORY SOC JOURNALS LTD. ISSN 1399-3003

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Abstract

Background Since 2015, the European pulmonary hypertension guidelines recommend the use of combination therapy in most patients with pulmonary arterial hypertension (PAH). However, it is unclear to what extent this treatment strategy is adopted in clinical practice and if it is associated with improved long-term survival. Methods We analysed data from COMPERA, a large European pulmonary hypertension registry, to assess temporal trends in the use of combination therapy and survival of patients with newly diagnosed PAH between 2010 and 2019. For survival analyses, we looked at annualised data and at cumulated data comparing the periods 2010-2014 and 2015-2019. Results A total of 2531 patients were included. The use of early combination therapy (within 3 months after diagnosis) increased from 10.0% in patients diagnosed with PAH in 2010 to 25.0% in patients diagnosed with PAH in 2019. The proportion of patients receiving combination therapy 1 year after diagnosis increased from 27.7% to 46.3%. When comparing the 2010-2014 and 2015-2019 periods, 1-year survival estimates were similar (89.0% (95% CI 87.2-90.9%) and 90.8% (95% CI 89.3-92.4%), respectively), whereas there was a slight but nonsignificant improvement in 3-year survival estimates (67.8% (95% CI 65.0-70.8%) and 70.5% (95% CI 67.8-73.4%), respectively). Conclusions The use of combination therapy increased from 2010 to 2019, but most patients still received monotherapy. Survival rates at 1 year after diagnosis did not change over time. Future studies need to determine if the observed trend suggesting improved 3-year survival rates can be confirmed.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Hoeper, Marius M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pausch, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gruenig, EkkehardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Staehler, GerdUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huscher, DoerteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pittrow, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Olsson, Karen M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vizza, Carmine DarioUNSPECIFIEDorcid.org/0000-0002-3540-4983UNSPECIFIED
Gall, HenningUNSPECIFIEDorcid.org/0000-0001-7016-7373UNSPECIFIED
Distler, OliverUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Opitz, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gibbs, J. Simon R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Delcroix, MarionUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ghofrani, H. ArdeschirUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rosenkranz, StephanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Park, Da-HeeUNSPECIFIEDorcid.org/0000-0003-0902-4773UNSPECIFIED
Ewert, RalfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kaemmerer, HaraldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lange, Tobias J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kabitz, Hans-JoachimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skowasch, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skride, AndrisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Claussen, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Behr, JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Milger, KatrinUNSPECIFIEDorcid.org/0000-0003-2914-8773UNSPECIFIED
Halank, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wilkens, HeinrikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Seyfarth, Hans-JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Held, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dumitrescu, DanielUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tsangaris, IraklisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vonk-Noordegraaf, AntonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ulrich, SilviaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klose, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-698355
DOI: 10.1183/13993003.02024-2021
Journal or Publication Title: Eur. Resp. J.
Volume: 59
Number: 6
Date: 2022
Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD
Place of Publication: SHEFFIELD
ISSN: 1399-3003
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
COMBINATION THERAPY; DIFFUSION CAPACITY; GUIDELINES; DIAGNOSIS; MORTALITY; SURVIVAL; AMBRISENTAN; TADALAFILMultiple languages
Respiratory SystemMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/69835

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