Disease characteristics and clinical outcome over two decades from the Swiss pulmonary hypertension registry.

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State: Public
Version: author
License: CC BY-NC 4.0
Serval ID
serval:BIB_870E468B29FA
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Disease characteristics and clinical outcome over two decades from the Swiss pulmonary hypertension registry.
Journal
Pulmonary circulation
Author(s)
Appenzeller P., Lichtblau M., Berlier C., Aubert J.D., Azzola A., Fellrath J.M., Geiser T., Lador F., Pohle S., Opitz I., Schwerzmann M., Stricker H., Tamm M., Saxer S., Ulrich S.
ISSN
2045-8932 (Print)
ISSN-L
2045-8932
Publication state
Published
Issued date
01/2022
Peer-reviewed
Oui
Volume
12
Number
1
Pages
e12001
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Pulmonary hypertension (PH), especially pulmonary arterial and chronic thromboembolic pulmonary hypertension (PAH/CTEPH), are rare and progressive conditions. Despite recent advances in treatment and prognosis, PH is still associated with impaired quality of life and survival. Long-term PH-registry data provide information on the changing PH-epidemiology and may help to direct resources to patient's needs. This retrospective analysis of the Swiss Pulmonary Hypertension Registry includes patients newly diagnosed with PH (mainly PAH/CTEPH) registered from January 2001 to June 2019 at 13 Swiss hospitals. Patient characteristics (age, body mass index, gender, diagnosis), hemodynamics at baseline, treatment, days of follow-up, and events (death, transplantation, pulmonary endarterectomy, or loss to follow-up) at last visit were analyzed. Patients were stratified into four time periods according to their date of diagnosis. Survival was analyzed overall and separately for PAH/CTEPH and time periods. 1427 PH patients were included (thereof 560 PAH, 383 CTEPH). Over the years, age at baseline (mean ± SD) significantly increased from 59 ± 14 years in 2001-2005 to 66 ± 14 years in 2016-2019 (p < 0.001) while the gender distribution tended toward equality. Mean pulmonary artery pressure and pulmonary vascular resistance significantly decreased over time (from 46 ± 15 to 41 ± 11 mmHg, respectively, 9 ± 5 to 7 ± 4 WU, p < 0.001). Three-year survival substantially increased over consecutive periods from 69% to 91% (for PAH 63%-95%, for CTEPH 86%-93%) and was poorer in PAH than CTEPH independently of time period (p < 0.001). Most patients were treated with mono- or combination therapy and an increasing number of CTEPH underwent pulmonary endarterectomy (40% 2016-2019 vs. 15% 2001-2005). This long-term PH registry reveals that over two decades of observation, newly diagnosed patients are older, less predominantly female, have less impaired hemodynamics and a better survival.
Keywords
Pulmonary and Respiratory Medicine, chronic thromboembolic pulmonary hypertension, pulmonary arterial hypertension, survival
Pubmed
Web of science
Open Access
Yes
Create date
08/04/2022 15:46
Last modification date
12/05/2023 5:56
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