Document Detail

Severe pulmonary hypertension: data from the Swiss Registry.
MedLine Citation:
PMID:  11486567     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
H Stricker; G Domenighetti; W Popov; R Speich; L Nicod; J D Aubert; M Solèr;
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Publication Detail:
Type:  Journal Article; Multicenter Study    
Journal Detail:
Title:  Swiss medical weekly     Volume:  131     ISSN:  1424-7860     ISO Abbreviation:  Swiss Med Wkly     Publication Date:  2001 Jun 
Date Detail:
Created Date:  2001-08-06     Completed Date:  2001-12-07     Revised Date:  2011-02-15    
Medline Journal Info:
Nlm Unique ID:  100970884     Medline TA:  Swiss Med Wkly     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  346-50     Citation Subset:  IM    
Internal Medicine of Regional Hospital, Locarno.
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MeSH Terms
Age Distribution
Blood Pressure / physiology
Exercise Test
Hypertension, Pulmonary / diagnosis*,  physiopathology*,  therapy
Middle Aged
Pulmonary Wedge Pressure / physiology
Retrospective Studies
Survival Analysis
Vascular Resistance / physiology

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