title: Long-term experience with oral or inhaled vasodilator combination therapy in patients with pulmonary hypertension.
abstract: BACKGROUND
  Disease progression in pulmonary hypertension (PH) is common despite standard vasodilator monotherapy with iloprost, bosentan or sildenafil.
  
  
  OBJECTIVE
  To investigate if the combination of these non-invasively applicable treatments is an effective option to address the multiple pathophysiological mechanisms present in PH.
  
  
  METHODS
  We analysed the clinical course of 23 patients with PH, diagnosed as idiopathic (n = 15), chronic thromboembolic (n = 4), and associated with collagen vascular disease (n = 4), receiving combination vasodilator therapy at our institution.
  
  
  RESULTS
  Vasodilator therapy before combination therapy consisted of inhaled iloprost (I; n = 12), or oral bosentan (B; n = 6) at a mean duration of 19 +/- 3 months. The combination therapy added was B (n = 8), sildenafil (S; n = 6) or I (n = 4) and in five patients, combination therapy was given from the beginning (3x BS, 1x IS, 1x IBS). Under combination therapy, the 6-minute walk distance (6MWD) increased significantly by 46.7 +/- 24.8 m (p = 0.02) after three months, and after six months it was still 38.3 +/- 28.3 m (p = 0.17) longer than before combination therapy. Respective changes in the Borg Scale and the NYHA functional class were -1.05 +/- 0.49 (p = 0.014) and -0.42 +/- 0.19 (p = 0.02) after three months and -0.21 +/- 0.65 (p = 0.61) and -0.38 +/- 0.29 (p = 0.26) after six months. Only minor side effects were reported.
  
  
  CONCLUSIONS
  Combination vasodilator therapy in severe PH is safe and well tolerated. It significantly improves exercise capacity and stabilises the functional class in patients with severe PH deteriorating under single-agent therapy.
authors.affiliation: Department of Internal Medicine, University Hospital Zurich, Switzerland.
authors.forename: Swantje
authors.initials: S
authors.lastname: Beyer
authors.forename: Rudolf
authors.initials: R
authors.lastname: Speich
authors.forename: Manuel
authors.initials: M
authors.lastname: Fischler
authors.forename: Marco
authors.initials: M
authors.lastname: Maggiorini
authors.forename: Silvia
authors.initials: S
authors.lastname: Ulrich
mesh_terms: D000280:Administration, Inhalation; D000284:Administration, Oral; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013557:Switzerland; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D014665:Vasodilator Agents