title: Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome.
abstract: BACKGROUND
  Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down's syndrome, the effect of bosentan is largely unknown. Aim of the study was to evaluate the long-term effects of bosentan in adult patients with CHD-related PAH with and without Down's syndrome.
  
  
  METHODS
  WHO functional class, resting oxygen saturation, 6-minute walk test (6 MWT) and hemodynamics were assessed at baseline and after 12 months of bosentan therapy in patients with CHD-related PAH with and without Down's syndrome.
  
  
  RESULTS
  Seventy-four consecutive patients were enrolled: 18 with and 56 without Down's syndrome. After 12 months of bosentan therapy, both with and without Down's syndrome patients showed an improvement in WHO functional class (Down: 2.5 ± 0.5 vs 2.9 ± 0.6, p=0.005; controls: 2.5 ± 0.5 vs 2.9 ± 0.5, p=0.000002), 6-minute walk distance (Down: 288 ± 71 vs 239 ± 74 m, p=0.0007; controls: 389 ± 80 vs 343 ± 86 m, p=0.00003), and hemodynamics (pulmonary flow, Down: 4.0 ± 1.6 vs 3.5 ± 1.4 l/m/m(2), p=0.006; controls: 3.5 ± 1.4 vs 2.8 ± 1.0 l/m/m(2), p=0.0005; pulmonary to systemic flow ratio, Down: 1.4 ± 0.7 vs 1.0 ± 0.4, p=0.003; controls: 1.1 ± 0.7 vs 0.9 ± 0.3, p=0.012; pulmonary vascular resistance index, Down: 15 ± 9 vs 20 ± 13 WUm(2), p=0.007; controls: 2 0 ± 10 vs 26 ± 15 WUm(2), p=0.002). No differences in the efficacy of therapy were observed between the two groups.
  
  
  CONCLUSIONS
  Bosentan was safe and well tolerated in adult patients with CHD-related PAH with and without Down's syndrome during 12 months of treatment. Clinical status, exercise tolerance, and pulmonary hemodynamics improved, regardless of the presence of Down's syndrome.
authors.affiliation: Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy. mic.dalto@tin.it
authors.forename: Michele
authors.initials: M
authors.lastname: D'Alto
authors.forename: Emanuele
authors.initials: E
authors.lastname: Romeo
authors.forename: Paola
authors.initials: P
authors.lastname: Argiento
authors.forename: Antonello
authors.initials: A
authors.lastname: D'Andrea
authors.forename: Berardo
authors.initials: B
authors.lastname: Sarubbi
authors.forename: Anna
authors.initials: A
authors.lastname: Correra
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Scognamiglio
authors.forename: Silvia
authors.initials: S
authors.lastname: Papa
authors.forename: Eduardo
authors.initials: E
authors.lastname: Bossone
authors.forename: Raffaele
authors.initials: R
authors.lastname: Calabrò
authors.forename: Carmine D
authors.initials: CD
authors.lastname: Vizza
authors.forename: Maria G
authors.initials: MG
authors.lastname: Russo
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage; D000077300:Bosentan; D004314:Down Syndrome / Q000150:complications*; D017079:Exercise Tolerance / Q000187:drug effects; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications*; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013449:Sulfonamides / Q000008:administration & dosage*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan