1. Long-term data from the Swiss pulmonary hypertension registry
- Author
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Mueller-Mottet, Séverine, Stricker, Hans, Domeninghetti, Guido, Azzola, Andrea, Geiser, Thomas, Schwerzmann, Markus, Weilenmann, Daniel, Schoch, Otto, Fellrath, Jean-Marc, Rochat, Thierry, Lador, Frederic, Beghetti, Maurice, Nicod, Laurent, Aubert, John-David, Popov, Vladimir, Speich, Rudolf, Keusch, Stephan, Hasler, Elisabeth, Huber, Lars, Grendelmeier, Peter, Tamm, Michael, Ulrich, Silvia, Mueller-Mottet, Séverine, Stricker, Hans, Domeninghetti, Guido, Azzola, Andrea, Geiser, Thomas, Schwerzmann, Markus, Weilenmann, Daniel, Schoch, Otto, Fellrath, Jean-Marc, Rochat, Thierry, Lador, Frederic, Beghetti, Maurice, Nicod, Laurent, Aubert, John-David, Popov, Vladimir, Speich, Rudolf, Keusch, Stephan, Hasler, Elisabeth, Huber, Lars, Grendelmeier, Peter, Tamm, Michael, and Ulrich, Silvia
- Abstract
Background: Registries are important for real-life epidemiology on different pulmonary hypertension (PH) groups. Objective: To provide long-term data of the Swiss PH-registry 1998-2012. Methods: PH-patients were classified in 5 groups and registered upon written informed consent at 5 University- and 8 associated hospitals since 1998. NYHA, 6 minute walk distance, hemodynamics and therapy were registered at baseline. Patients were regularly followed and therapy and events (death, transplantation, endarterectomy or lost to follow-up) registered. The data was stratified according to the time of diagnosis into prevalent before 2000 and incident during 2000-04, 2005-08 and 2009-12. Results: From 996 (53% female) PH-patients, 549 had pulmonary arterial hypertension (PAH), 36 PH due to left heart -, 127 due to lung disease, 249 chronic thromboembolic PH (CTEPH) and 35 miscellaneous PH. Age and BMI significantly increased over time whereas hemodynamic severity decreased. Overall, event-free survival was 84, 72, 64, 58% for the years 1-4 and similar for time periods since 2000, but better during the more recent periods for PAH and CTEPH. 89% of all PAH had target medical therapy, 43% combination-therapy. 14 resp. 2% of CTEPH underwent pulmonary endarterectomy or transplantation, 87 % were treated with PAH-target therapy. Conclusion: Since 2000, incident Swiss PH-patients registered were older, hemodynamically better and mostly treated with PAH-target therapies. Survival was better for PAH and CTEPH diagnosed since 2008 compared with earlier diagnosis or other classifications.
- Published
- 2015