title: Effect of bosentan therapy on ventricular and atrial function in adults with Eisenmenger syndrome. A prospective, multicenter study using conventional and Speckle tracking echocardiography.
abstract: BACKGROUND
  The effect of bosentan on the ventricular and atrial performance in patients with Eisenmenger syndrome is unclear. In adult patients with Eisenmenger syndrome, we aimed to evaluate the midterm effect of bosentan on physical exercise, ventricular and atrial function, and pulmonary hemodynamics.
  
  
  METHODS
  Forty adult patients before and after 24 weeks bosentan therapy underwent 6 min walk test, two-dimensional speckle tracking echocardiography, plasma NT-proBNP measurement and cardiac catheterization.
  
  
  RESULTS
  After 24 weeks, bosentan therapy an improvement was observed regarding the 6 min walk distance from a median (quartile 1-quartile 3) of 382.5 (312-430) to 450 (390-510) m (p = 0.0001), NT-proBNP from 527.5 (201-1,691.25) to 369 (179-1,246) pg/ml (p = 0.021), right ventricular mean longitudinal systolic strain from 18 (13-22) to 19 (14.5-25) % (p = 0.004), left ventricular mean longitudinal systolic strain from 16 (12-21) to 17 (16-22) % (p = 0.001), right atrial mean peak longitudinal strain from 26 (18-34) to 28 (22-34) % (p = 0.01) and right atrial mean peak contraction strain from 11 (8-16) to 13 (11-16) % (p = 0.005). The invasively obtained Qp:Qs and Rp:Rs did not significantly change under bosentan therapy.
  
  
  CONCLUSIONS
  In adult patients with Eisenmenger syndrome, bosentan therapy improves ventricular and atrial functions resulting in enhancement of physical exercise and reduction in the NT-proBNP level, while the pulmonary vascular resistance does not change substantially.
authors.affiliation: Department of Pediatric Cardiology, Saarland University Hospital, Kirrberger Straße, 66421, Homburg/Saar, Germany.
authors.forename: Mohamed Y
authors.initials: MY
authors.lastname: Abd El Rahman
authors.forename: Axel
authors.initials: A
authors.lastname: Rentzsch
authors.forename: Philipp
authors.initials: P
authors.lastname: Scherber
authors.forename: Siegrun
authors.initials: S
authors.lastname: Mebus
authors.forename: Oliver
authors.initials: O
authors.lastname: Miera
authors.forename: Günther
authors.initials: G
authors.lastname: Balling
authors.forename: Petra
authors.initials: P
authors.lastname: Böttler
authors.forename: Karl-Otto
authors.initials: KO
authors.lastname: Dubowy
authors.forename: Birgit
authors.initials: B
authors.lastname: Farahwaschy
authors.forename: Alfred
authors.initials: A
authors.lastname: Hager
authors.forename: Joachim
authors.initials: J
authors.lastname: Kreuder
authors.forename: Brigitte
authors.initials: B
authors.lastname: Peters
authors.forename: Felix
authors.initials: F
authors.lastname: Berger
authors.forename: Ingram
authors.initials: I
authors.lastname: Schulze-Neick
authors.forename: Hashim
authors.initials: H
authors.lastname: Abdul-Khaliq
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D016275:Atrial Function / Q000187:drug effects*; D000077300:Bosentan; D006328:Cardiac Catheterization; D004452:Echocardiography / Q000379:methods; D004541:Eisenmenger Complex / Q000188:drug therapy* / Q000503:physiopathology; D005080:Exercise Test; D005260:Female; D006801:Humans; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D011446:Prospective Studies; D013449:Sulfonamides / Q000627:therapeutic use*; D014655:Vascular Resistance / Q000187:drug effects; D016276:Ventricular Function / Q000187:drug effects*; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents; D010446:Peptide Fragments; D013449:Sulfonamides; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D000077300:Bosentan