Abstract 2613: Efficacy of Bosentan in Adults with Complex Anatomy and Eisenmenger Syndrome
Background: Non-selective endothelin receptor blockade with Bosentan improves functional capacity in adults with Eisenmenger syndrome (ES) who have isolated atrial septal defects (ASD), ventricular septal defects (VSD), or patent ductus arteriosus (PDA). The efficacy of Bosentan in adults with complex anatomic lesions and ES has not been specifically evaluated.
Hypothesis: Patients with complex ES derive a similar benefit from Bosentan as those with simple ES.
Methods: Single center retrospective analysis of adults with ES grouped into simple vs. complex lesions.
Results: 19 patients included (6M/13F), mean age 38 +/− 9 yrs. Simple ES (4 VSD, 2 PDA) in 6 pts, mean age 37 years (1M/5F). Complex ES (5 atrioventricular septal defect, 3 single ventricle, 2 Truncus Arteriosus, 2 transposition of great arteries, 1 tetralogy of Fallot with pulmonary atresia and multiple aortopulmonary collaterals) in 13 pts, mean age 38 years (5M/8F). All patients treated with bosentan. Simple ES: 4 on bosentan alone, 1 on bosentan and sildenafil, 1 on bosentan and iloprost. Complex ES: 7 on bosentan alone, 6 on bosentan and sildenafil. Six minute walk distance (6MWD) before Rx was lower in complex ES (266 vs. 274 meters, p=NS), and resting oxygen saturation was lower in complex ES (73% vs. 80%, p=0.09). Rx with bosentan improved 6 MWD in the entire group from 269 meters to 319 meters (+49 meters, p=0.03). Pts with complex ES improved by 57 meters (p=0.02), simple ES improved by 36 meters (p=0.06). In complex ES, resting room air saturation improved from 73% to 78% (+5%, p=0.009). In simple ES (n=5), resting saturation improved from 80% to 84% (+4%, p=0.06). One patient died, a 49 year old female with VSD (simple ES), treated with bosentan for 30 months and additional sildenafil for 24 months. Presumptive cause of death was arrhythmia.
Conclusions: Patients with complex congenital heart disease and ES have lower baseline 6MWD and resting oxygen saturation compared to those with simple ES. Treatment with bosentan lead to improvement in 6MWD and resting saturation in complex ES patients, the magnitude of improvement may exceed that seen simple ES.