Pneumologie 2005; 59 - V275
DOI: 10.1055/s-2005-864363

Bosentan in the Treatment of Pulmonary Arterial Hypertension in Adults with Congenital Heart Defects: Preliminary Experience of a Multicenter Cooperation Project

I Schulze-Neick 1, N Gilbert 1, K Lunze 1, R Ewert 2, C Witt 3, E Grünig 4, H Olschewski 5, MM Borst 4, P Lange 1, M Höper 6
  • 1Deutsches Herzzentrum Berlin
  • 2Universitätsklinikum Greifswald
  • 3Berlin, Charité
  • 4Ruprecht-Karls-Universität Heidelberg
  • 5Universität Gießen
  • 6Medizinische Hochschule Hannover

Introduction:

Bosentan is effective in the treatment of primary pulmonary arterial hypertension (PAH). As yet there are no published data concerning its use in adult PAH patients with congenital heart defects.

Methods:

Thirty-four patients (age 45±15 years; 23 pts with Eisenmenger syndrome) at 6 specialized centers were treated with a bosentan dose of 62,5–250mg/d. Before and after 16,4±5,7 months of therapy, clinical, laboratory, echocardiographic and hemodynamic parameters were measured.

Results:

The walking range improved from 359±109 m to 423±92 m (p=0,002); this correlated with an improvement of the mean NYHA class from 3,1 to 2,4 (p=0,0001). The maximal oxygen uptake and the right ventricular systolic pressure measured by echocardiography remained unchanged.

Conclusions:

This first multicentric observational study of bosentan treatment in adult patients with congenital heart defects and severe pulmonary hypertension confirms that the treatment is well-tolerated and significantly improves clinical condition and exercise tolerance. Further systematic studies are necessary and are underway.