Long-term data from the swiss pulmonary hypertension registry

Publication

Long-term data from the swiss pulmonary hypertension registry

Journal Paper/Review - Jan 31, 2015

Mueller-Mottet Séverine, Nicod Laurent, Aubert John-David, Popov Vladimir, Speich Rudolf, Keusch Stephan, Hasler Elisabeth, Huber Lars C, Grendelmeier Peter, Tamm Michael, Beghetti Maurice, Lador Frederic, Stricker Hans, Domeninghetti Guido, Azzola Andrea, Geiser Thomas, Schwerzmann Markus, Weilenmann Daniel, Schoch Otto, Fellrath Jean-Marc, Rochat Thierry, Ulrich Silvia

Citation

Mueller-Mottet S, Nicod L, Aubert J, Popov V, Speich R, Keusch S, Hasler E, Huber L, Grendelmeier P, Tamm M, Beghetti M, Lador F, Stricker H, Domeninghetti G, Azzola A, Geiser T, Schwerzmann M, Weilenmann D, Schoch O, Fellrath J, Rochat T, Ulrich S. Long-term data from the swiss pulmonary hypertension registry. Respiration 2015; 89:127-40.

Type

Journal Paper/Review (English)

Journal

Respiration 2015; 89

Publication Date

Jan 31, 2015

Issn Electronic

1423-0356

Pages

127-40

Brief description/objective

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. © 2015 S. Karger AG, Basel.