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
UNIL restricted access
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
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
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