Severe pulmonary hypertension: data from the Swiss Registry.

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Version: Final published version
Serval ID
serval:BIB_0A87E9F19B56
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Severe pulmonary hypertension: data from the Swiss Registry.
Journal
Swiss medical weekly
Author(s)
Stricker H., Domenighetti G., Popov W., Speich R., Nicod L., Aubert J.D., Solèr M.
Working group(s)
Swiss Group for Severe Pulmonary Hypertension
ISSN
1424-7860 (Print)
ISSN-L
0036-7672
Publication state
Published
Issued date
16/06/2001
Peer-reviewed
Oui
Volume
131
Number
23-24
Pages
346-350
Language
english
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
Abstract
Severe pulmonary hypertension (PH) is a rare disease with a dismal prognosis if untreated. Progress in diagnosis and in the development of effective therapeutic options has created new interest in this pathology. There are, however, only limited data on the prevalence of severe PH unrelated to chronic left ventricular failure or COPD, on the associated conditions and on the parameters with a prognostic impact. With the aid of a retrospective registry we have collected data from 5 centres in Switzerland and attempted to answer the above questions.
Data on patients with PH from 4 university facilities (Zurich, Basle, Geneva and Lausanne) and one well-defined geographical area (Ticino) were retrospectively collected and analysed up to December 1999. Clinical and haemodynamic parameters and associated diseases were noted. We were also interested in the age distribution of the patients and the year of diagnosis of PH.
We found 106 patients with severe PH (43 men, 63 women, median age 43 years); 79% were in NYHA class III or IV. There was a steep rise in diagnosis of PH after 1995. In 74% PH was either primary or associated with collagen vascular disease or thromboembolic disease. By the end of the observation period 30% of the patients had died. The best distinguishing parameters between surviving patients and those who eventually died were the 6-minute walking test (363 vs. 235 metres, p = 0.002), the NYHA class (II vs III/IV, p = 0.015), and mixed venous saturation (66.5 vs. 57.9%, p = 0.006). Therapy consisted of calcium antagonists in 18% and of (inhaled) prostanoids, chiefly iloprost, in 33%. Seven patients underwent lung transplantation.
We conclude that PH is diagnosed more often as diagnostic and therapeutic options improve; that primary forms, and those associated with collagen vascular disease and with chronic venous thromboembolism, make up three-quarters of the aetiologies; and that the 6-minute walking test, the functional class and mixed venous saturation are the best prognostic parameters.

Keywords
Adolescent, Adult, Age Distribution, Aged, Blood Pressure/physiology, Child, Exercise Test, Female, Humans, Hypertension, Pulmonary/diagnosis, Hypertension, Pulmonary/physiopathology, Hypertension, Pulmonary/therapy, Male, Middle Aged, Prognosis, Pulmonary Wedge Pressure/physiology, Registries, Retrospective Studies, Survival Analysis, Switzerland, Vascular Resistance/physiology
Pubmed
Web of science
Create date
21/01/2008 13:54
Last modification date
20/08/2019 13:32
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