PMID- 18389984
OWN - NLM
STAT- MEDLINE
DCOM- 20080430
LR  - 20181201
IS  - 0125-2208 (Print)
IS  - 0125-2208 (Linking)
VI  - 91
IP  - 2
DP  - 2008 Feb
TI  - A retrospective study of bosentan in pulmonary arterial hypertension associated 
      with congenital heart disease.
PG  - 196-202
AB  - BACKGROUND: Pulmonary Arterial Hypertension (PAH) plays a significant role in 
      morbidity and mortality of patients with congenital heart disease (CHD). 
      Bosentan, a dual endothelin receptor antagonist has been approved for PAH 
      patients with Eisenmenger physiology (EP). The authors retrospectively reviewed 
      the efficacy and safety of bosentan in Thai PAH patients associated with CHD. 
      MATERIAL AND METHOD: The study population was obtained from the databases of the 
      CHD patients at Siriraj Hospital from October 2004 to April 2007 who received 6 
      months of bosentan treatment. Inclusion criteria are: CHD with Eisenmenger 
      physiology (EP) or those with severe PAH after surgical repair or interventional 
      cardiac catheterization. Clinical characteristics including the 6-- minute walk 
      test (6MWT) distances, oxygen saturation (O2 sat), New York Heart Association 
      (NYHA) functional class, and right ventricular systolic pressure (RVSP) at 
      baseline were compared with those at 1, 3, and 6 months post bosentan treatment. 
      Signs and symptoms of adverse events were also recorded. RESULTS: There were 11 
      patients from among those who fitted the inclusion criteria and whose records 
      were examined. Their average age was 51.1 +/- 10.1 years old (13-61 years old). 
      Patients were divided into 2 groups; Group A (6 patients) was PAH with EP and 
      Group B (5 patients) was PAH post intervention. In group A, the 6MWT increased 
      from 151 +/- 69 meters to 293 +/- 61 meters (p = 0.001) with the average increase 
      of 38 +/- 61 meters. The 2O sat increased from 83 +/- 12.7% to 91.8 +/- 5.6% (p = 
      0.038) with an average increase of 1.4 +/- 0.07%. There was no significant change 
      in right ventricular systolic pressure (RVSP). In group B, there was a trend in 
      6MWT improvement from 274 +/- 69 meters to 312 +/- 38 meters but this was not 
      statistically different. There were improvements in the NYHA functional class in 
      both groups. There was no significant increase in serum aminotransferase at the 
      end of 6 months in each patient. CONCLUSION: There are benefits of bosentan for 
      treatment of severe PAH in CHD, especially in patients with Eisenmenger 
      physiology. Obvious benefits are an improvement of 6MWT and O2 sat.
FAU - Durongpisitkul, Kritvikrom
AU  - Durongpisitkul K
AD  - Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, 
      Siriraj Hospital, Mahidol University, Bangkok, Thailand. sikdr@mahidol.ac.th
FAU - Jakrapanichakul, Decho
AU  - Jakrapanichakul D
FAU - Sompradikul, Suree
AU  - Sompradikul S
LA  - eng
PT  - Journal Article
PL  - Thailand
TA  - J Med Assoc Thai
JT  - Journal of the Medical Association of Thailand = Chotmaihet thangphaet
JID - 7507216
RN  - 0 (Antihypertensive Agents)
RN  - 0 (Endothelins)
RN  - 0 (Sulfonamides)
RN  - Q326023R30 (Bosentan)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Antihypertensive Agents/*therapeutic use
MH  - Bosentan
MH  - Databases as Topic
MH  - Eisenmenger Complex
MH  - Endothelins/drug effects
MH  - Exercise Test
MH  - Female
MH  - *Health Status
MH  - Health Status Indicators
MH  - Heart Defects, Congenital/*complications
MH  - Heart Ventricles
MH  - Humans
MH  - Hypertension, Pulmonary/*drug therapy/etiology
MH  - Male
MH  - Middle Aged
MH  - Pulmonary Artery/drug effects/*pathology
MH  - Retrospective Studies
MH  - Sulfonamides/*therapeutic use
MH  - Systole
MH  - Time Factors
MH  - *Treatment Outcome
EDAT- 2008/04/09 09:00
MHDA- 2008/05/01 09:00
CRDT- 2008/04/09 09:00
PHST- 2008/04/09 09:00 [pubmed]
PHST- 2008/05/01 09:00 [medline]
PHST- 2008/04/09 09:00 [entrez]
PST - ppublish
SO  - J Med Assoc Thai. 2008 Feb;91(2):196-202.