Clinical Research in Cardiology

, Volume 103, Issue 9, pp 701–710 | Cite as

Effect of bosentan therapy on ventricular and atrial function in adults with Eisenmenger syndrome. A prospective, multicenter study using conventional and Speckle tracking echocardiography

  • Mohamed Y. Abd El Rahman
  • Axel Rentzsch
  • Philipp Scherber
  • Siegrun Mebus
  • Oliver Miera
  • Günther Balling
  • Petra Böttler
  • Karl-Otto Dubowy
  • Birgit Farahwaschy
  • Alfred Hager
  • Joachim Kreuder
  • Brigitte Peters
  • Felix Berger
  • Ingram Schulze-Neick
  • Hashim Abdul-Khaliq
Original Paper



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.


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.


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.


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.


Pulmonary arterial hypertension, echocardiography Myocardial contraction Remodeling 



Two-dimensional speckle tracking echocardiography


Six-minute walk test


Congenital heart defect


N-terminal pro-B-type natriuretic peptide


Peak atrial contraction strain


Pulmonary arterial hypertension


Peak atrial longitudinal strain


Pulmonary blood flow:systemic blood flow


Region of interest


Pulmonary resistance:systemic resistance


Tricuspid annular plane systolic excursion



The authors would like to acknowledge the collaboration and commitment of all the local investigators and their staff. Participating centers (listed in alphabetical order), in which patients have been included: Heart and Diabetes Center NRW, Bad Oeynhausen: D. Kececioglu, P. Beerbaum, S. Sarikouch, R. Schäffler. Deutsches Herzzentrum Berlin, Berlin: P. Ewert. Justus-Liebig University Giessen, Giessen: D. Schranz, R. Zimmermann. Saarland University Hospital, Homburg/Saar: A. Lindinger, J. Olchavary. Deutsches Herzzentrum Muenchen, Technische Universitaet Muenchen, Munich: J. Hess, H. Kaemmerer Consultant team in echocardiography: M. Vogel (Munich), M. Vogt (Munich). We are further indebted to Professor M. Böhm (Department of Internal Medicine III, Cardiology, Angiology and Intensive Care Medicine, Saarland University Hospital) for his careful review of and valuable comments on the manuscript. Ms. Verena Materna, Koordinierungszentrum fuer Klinische Studien, Charité, Berlin for the organizing support. Ms. Brigitte Tüllmann, Koordinierungszentrum fuer Klinische Studien, Charité, Berlin for the monitoring work. Anne M. Gale, ELS, of Deutsches Herzzentrum Berlin, for editorial assistance. Ulrike Bauer, MD and Thomas Pickardt, Ph.D., Competence Network for Congenital Heart Defects, for the central research management. This work was supported by the Competence Network for Congenital Heart Defects funded by the Federal Ministry of Education and Research (BMBF), FKZ 01G10210, and by an unrestricted grant by Actelion Pharmaceuticals Deutschland GmbH.

Conflict of interest

The authors certify that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Mohamed Y. Abd El Rahman
    • 1
    • 9
  • Axel Rentzsch
    • 1
  • Philipp Scherber
    • 1
  • Siegrun Mebus
    • 2
    • 3
  • Oliver Miera
    • 2
  • Günther Balling
    • 3
  • Petra Böttler
    • 4
  • Karl-Otto Dubowy
    • 5
  • Birgit Farahwaschy
    • 2
  • Alfred Hager
    • 3
  • Joachim Kreuder
    • 6
  • Brigitte Peters
    • 7
  • Felix Berger
    • 2
  • Ingram Schulze-Neick
    • 2
    • 8
  • Hashim Abdul-Khaliq
    • 1
  1. 1.Department of Pediatric CardiologySaarland University HospitalHomburg/SaarGermany
  2. 2.Department of Congenital Heart Disease/Pediatric CardiologyDeutsches HerzzentrumBerlinGermany
  3. 3.Department of Pediatric Cardiology and Congenital Heart Defects, German Heart Centre MunichTechnische Universitaet MuenchenMunichGermany
  4. 4.Department of Congenital Heart Defects/Pediatric CardiologyUniversity Hospital FreiburgFreiburgGermany
  5. 5.Center for Congenital Heart DefectsHeart and Diabetes Center NRWBad OeynhausenGermany
  6. 6.Pediatric Heart CenterJustus-Liebig University GiessenGiessenGermany
  7. 7.Department of Biometry and Medical InformaticsOtto von Guericke Universitaet MagdeburgMagdeburgGermany
  8. 8.UK Service for Pulmonary Hypertension in Children, Cardiac UnitGreat Ormond Street HospitalLondonUK
  9. 9.Department of Pediatrics and Pediatric CardiologyCairo UniversityCairoEgypt

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