PMID- 16919007
OWN - NLM
STAT- MEDLINE
DCOM- 20070222
LR  - 20201216
IS  - 0014-2972 (Print)
IS  - 0014-2972 (Linking)
VI  - 36 Suppl 3
DP  - 2006 Sep
TI  - Bosentan for the treatment of pulmonary arterial hypertension associated with 
      congenital heart defects.
PG  - 25-31
AB  - BACKGROUND: Bosentan is an effective first-line therapy in New York Heart 
      Association (NYHA) III patients with idiopathic pulmonary arterial hypertension 
      (PAH). Pre-clinical data support the rationale for the potential benefit of 
      bosentan in PAH associated with congenital heart disease (CHD). MATERIALS AND 
      METHODS: We performed a retrospective analysis of patients with PAH-associated 
      CHD who were treated with bosentan on top of conventional therapy. Bosentan was 
      started at 62.5 mg bid for 4 weeks, then titrated to 125 mg bid. New York Heart 
      Association (NYHA) functional class, 6-min walking distance (6MWD), Borg dyspnoea 
      index, arterial oxygen saturation and cardiopulmonary haemodynamic data (cardiac 
      output, pulmonary blood flow and systemic and pulmonary vascular resistances) 
      were collected at baseline and at follow up. RESULTS: Twenty-seven patients (23 
      females, mean 35 +/- 15 years) with NYHA class III-IV PAH-associated CHD (not 
      repaired in 23 cases) were treated with bosentan for a mean 18.3 +/- 9.9 months. 
      Bosentan improved 6MWD from 298 +/- 92 m at baseline to 355 +/- 82 m at 3 months 
      (P = 0.0002) and to 364 +/- 92 m (P = 0.0001) at the last follow up (mean 15.2 
      +/- 9.7 months). At the last follow up, 13 patients had improved (= 1 NYHA class) 
      and 14 remained stable. A favourable effect was observed in pulmonary blood flow 
      and pulmonary vascular resistance for the 11 available patients. No change in 
      pulse oximetry or liver enzyme elevation was reported. CONCLUSIONS: Bosentan 
      improves exercise capacity, functional class and haemodynamics in most patients 
      with PAH-associated CHD, without serious side-effects, suggesting bosentan may be 
      an important treatment option for these patients.
FAU - Sitbon, O
AU  - Sitbon O
AD  - Hopital Antoine Beclere, Service de Pneumologie, Clamart, France. 
      olivier.sitbon@abc.aphp.fr
FAU - Beghetti, M
AU  - Beghetti M
FAU - Petit, J
AU  - Petit J
FAU - Iserin, L
AU  - Iserin L
FAU - Humbert, M
AU  - Humbert M
FAU - Gressin, V
AU  - Gressin V
FAU - Simonneau, G
AU  - Simonneau G
LA  - eng
PT  - Journal Article
PL  - England
TA  - Eur J Clin Invest
JT  - European journal of clinical investigation
JID - 0245331
RN  - 0 (Antihypertensive Agents)
RN  - 0 (Prostaglandins, Synthetic)
RN  - 0 (Sulfonamides)
RN  - Q326023R30 (Bosentan)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Antihypertensive Agents/*administration & dosage
MH  - Bosentan
MH  - Child
MH  - Drug Therapy, Combination
MH  - Exercise Test/methods
MH  - Female
MH  - Heart Defects, Congenital/*complications/physiopathology
MH  - Humans
MH  - Hypertension, Pulmonary/complications/*drug therapy/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Prostaglandins, Synthetic/therapeutic use
MH  - Pulmonary Circulation/drug effects
MH  - Retrospective Studies
MH  - Sulfonamides/*administration & dosage
MH  - Treatment Outcome
MH  - Vascular Resistance/drug effects
MH  - Walking/physiology
EDAT- 2006/08/22 09:00
MHDA- 2007/02/23 09:00
CRDT- 2006/08/22 09:00
PHST- 2006/08/22 09:00 [pubmed]
PHST- 2007/02/23 09:00 [medline]
PHST- 2006/08/22 09:00 [entrez]
AID - ECI1685 [pii]
AID - 10.1111/j.1365-2362.2006.01685.x [doi]
PST - ppublish
SO  - Eur J Clin Invest. 2006 Sep;36 Suppl 3:25-31. doi: 
      10.1111/j.1365-2362.2006.01685.x.