PMID- 25661477 OWN - NLM STAT- MEDLINE DCOM- 20151216 LR - 20150303 IS - 1423-0356 (Electronic) IS - 0025-7931 (Linking) VI - 89 IP - 2 DP - 2015 TI - Long-term data from the Swiss pulmonary hypertension registry. PG - 127-40 LID - 10.1159/000370125 [doi] AB - 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 of 1998-2012. METHODS: PH patients have been classified into 5 groups and registered upon written informed consent at 5 university and 8 associated hospitals since 1998. New York Heart Association (NYHA) class, 6-min walk distance, hemodynamics and therapy were registered at baseline. Patients were regularly followed, and therapy and events (death, transplantation, endarterectomy or loss to follow-up) registered. The data were stratified according to the time of diagnosis into prevalent before 2000 and incident during 2000-2004, 2005-2008 and 2009-2012. RESULTS: From 996 (53% female) PH patients, 549 had pulmonary arterial hypertension (PAH), 36 PH due to left heart disease, 127 due to lung disease, 249 to chronic thromboembolic PH (CTEPH) and 35 to miscellaneous PH. Age and BMI significantly increased over time, whereas hemodynamic severity decreased. Overall, event-free survival was 84, 72, 64 and 58% for the years 1-4 and similar for time periods since 2000, but better during the more recent periods for PAH and CTEPH. Of all PAH cases, 89% had target medical therapy and 43% combination therapy. Of CTEPH patients, 14 and 2% underwent pulmonary endarterectomy or transplantation, respectively; 87% were treated with PAH target therapy. CONCLUSION: Since 2000, the incident Swiss PH patients registered were older, hemodynamically better and mostly treated with PAH target therapies. Survival has been better for PAH and CTEPH diagnosed since 2008 compared with earlier diagnosis or other classifications. CI - (c) 2015 S. Karger AG, Basel. FAU - Mueller-Mottet, Severine AU - Mueller-Mottet S AD - Pulmonary Clinic, University Hospital Zurich, Zurich, Switzerland. FAU - Stricker, Hans AU - Stricker H FAU - Domenighetti, Guido AU - Domenighetti G FAU - Azzola, Andrea AU - Azzola A FAU - Geiser, Thomas AU - Geiser T FAU - Schwerzmann, Markus AU - Schwerzmann M FAU - Weilenmann, Daniel AU - Weilenmann D FAU - Schoch, Otto AU - Schoch O FAU - Fellrath, Jean-Marc AU - Fellrath JM FAU - Rochat, Thierry AU - Rochat T FAU - Lador, Frederic AU - Lador F FAU - Beghetti, Maurice AU - Beghetti M FAU - Nicod, Laurent AU - Nicod L FAU - Aubert, John-David AU - Aubert JD FAU - Popov, Vladimir AU - Popov V FAU - Speich, Rudolf AU - Speich R FAU - Keusch, Stephan AU - Keusch S FAU - Hasler, Elisabeth AU - Hasler E FAU - Huber, Lars C AU - Huber LC FAU - Grendelmeier, Peter AU - Grendelmeier P FAU - Tamm, Michael AU - Tamm M FAU - Ulrich, Silvia AU - Ulrich S LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150131 PL - Switzerland TA - Respiration JT - Respiration; international review of thoracic diseases JID - 0137356 SB - IM EIN - Respiration. 2015;89(5)373. Domeninghetti, Guido [corrected to Domenighetti, Guido MH - Adolescent MH - Adult MH - Aged MH - Female MH - Humans MH - Hypertension, Pulmonary/*epidemiology MH - Male MH - Middle Aged MH - *Registries MH - Switzerland/epidemiology MH - Young Adult EDAT- 2015/02/11 06:00 MHDA- 2015/12/19 06:00 CRDT- 2015/02/10 06:00 PHST- 2014/10/09 00:00 [received] PHST- 2014/11/21 00:00 [accepted] PHST- 2015/02/10 06:00 [entrez] PHST- 2015/02/11 06:00 [pubmed] PHST- 2015/12/19 06:00 [medline] AID - 000370125 [pii] AID - 10.1159/000370125 [doi] PST - ppublish SO - Respiration. 2015;89(2):127-40. doi: 10.1159/000370125. Epub 2015 Jan 31.