A Retrospective Study of Bosentan in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease
Kritvikrom Durongpisitkul MD*, Decho Jakrapanichakul MD**, Suree Sompradikul MD***
Affiliation : * Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok ** Division of Cardiology, Department of Internal Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok *** Division of Pulmonary Medicine, Department of Internal Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok
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 O2 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.
Keywords : Pulmonary arterial hypertension, Congenital heart disease, Bosentan
