Temporal trends in pulmonary arterial hypertension: results from the COMPERA registry

Hoeper, Marius M. and Pausch, Christine and Gruenig, Ekkehard and Staehler, Gerd and Huscher, Doerte and Pittrow, David and Olsson, Karen M. and Vizza, Carmine Dario and Gall, Henning and Distler, Oliver and Opitz, Christian and Gibbs, J. Simon R. and Delcroix, Marion and Ghofrani, H. Ardeschir and Rosenkranz, Stephan and Park, Da-Hee and Ewert, Ralf and Kaemmerer, Harald and Lange, Tobias J. and Kabitz, Hans-Joachim and Skowasch, Dirk and Skride, Andris and Claussen, Martin and Behr, Juergen and Milger, Katrin and Halank, Michael and Wilkens, Heinrike and Seyfarth, Hans-Juergen and Held, Matthias and Dumitrescu, Daniel and Tsangaris, Iraklis and Vonk-Noordegraaf, Anton and Ulrich, Silvia and Klose, Hans (2022) Temporal trends in pulmonary arterial hypertension: results from the COMPERA registry. EUROPEAN RESPIRATORY JOURNAL, 59 (6): 2102024. ISSN 0903-1936, 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: Article
Uncontrolled Keywords: COMBINATION THERAPY; DIFFUSION CAPACITY; GUIDELINES; DIAGNOSIS; MORTALITY; SURVIVAL; AMBRISENTAN; TADALAFIL;
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Innere Medizin II
Depositing User: Dr. Gernot Deinzer
Date Deposited: 12 Dec 2023 10:31
Last Modified: 12 Dec 2023 10:31
URI: https://pred.uni-regensburg.de/id/eprint/58797

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