Document Detail

Bosentan therapy for portopulmonary hypertension.
MedLine Citation:
PMID:  15738295     Owner:  NLM     Status:  MEDLINE    
The dual endothelin receptor antagonist bosentan has been approved in several countries for pulmonary arterial hypertension, and patients with portopulmonary hypertension (PPHTN) have not specifically been excluded. However, no data have been published on the efficacy and safety of bosentan in this patient population. Here, the first clinical experiences with bosentan in patients with Child A cirrhosis and severe PPHTN are reported. In total, 11 consecutive patients with cirrhosis and severe PPHTN in New York Heart Association Functional Classes III and IV were treated for >1 yr with bosentan. After 1 yr of treatment with bosentan, all patients showed improved symptoms and exercise capacity. The 6-min walking distance increased from 310+/-102 m at baseline to 388+/-81 m at 1 yr. Cardiopulmonary exercise testing disclosed a significant increase in peak oxygen uptake, from 12.6+/-3.5 to 16.6+/-2.8 mL.min(-1).kg(-1). Pulmonary vascular resistance fell from 944+/-519 to 635+/-321 dynes.s.L(-1). The medication was well tolerated by all patients, and there was no evidence of drug-related liver injury. In conclusion, bosentan proved to be efficacious and safe in a small number of patients with portopulmonary hypertension.
M M Hoeper; M Halank; C Marx; G Hoeffken; H J Seyfarth; J Schauer; J Niedermeyer; J Winkler
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The European respiratory journal     Volume:  25     ISSN:  0903-1936     ISO Abbreviation:  Eur. Respir. J.     Publication Date:  2005 Mar 
Date Detail:
Created Date:  2005-03-01     Completed Date:  2005-07-12     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  8803460     Medline TA:  Eur Respir J     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  502-8     Citation Subset:  IM    
Dept of Respiratory Medicine, Hannover Medical School, 30623 Hannover, Germany.
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MeSH Terms
Antihypertensive Agents / therapeutic use*
Exercise Tolerance
Hemodynamics / drug effects
Hypertension, Portal / drug therapy*,  etiology,  physiopathology
Hypertension, Pulmonary / drug therapy*,  etiology,  physiopathology
Liver Cirrhosis, Alcoholic / complications
Middle Aged
Oxygen Consumption / drug effects
Retrospective Studies
Sulfonamides / therapeutic use*
Treatment Outcome
Reg. No./Substance:
0/Antihypertensive Agents; 0/Sulfonamides; Q326023R30/bosentan

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