Improved survival among patients with Eisenmenger syndrome receiving ad- vanced therapy for pulmonary arterial hypertension. Circulation. 2010;121:20 5. S4.4.6.2-2. Gatzoulis MA, Beghetti M, Gali N, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008;127:27 32. S4.4.6.2-3. Gali N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, dou- ble-blind, randomized, placebo-controlled study. Circulation. 2006;114:48 54. S4.4.6.2-4. Diller G-P, Alonso-Gonzalez R, Dimopoulos K, et al. Disease targeting therapies in patients with Eisenmenger syndrome: re- sponse to treatment and long-term efficiency. Int J Cardiol. 2013;167:840 7. S4.4.6.2-5. D Alto M, Romeo E, Argiento P, et al. Therapy for pulmonary ar- terial hypertension due to congenital heart disease and Down s syndrome. Int J Cardiol. 2013;164:323 6. S4.4.6.2-6. Iversen K, Jensen AS, Jensen TV, et al. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a ran- domized, placebo-controlled, double-blinded trial. Eur Heart J. 2010;31:1124 31. S4.4.6.2-7. Baptista R, Castro G, da Silva AM, et al. Long-term effect of bosentan in pulmonary hypertension associated with complex congenital heart disease. Rev Port Cardiol. 2013;32:123 9. S4.4.6.2-8. Crepaz R, Romeo C, Montanaro D, et al. Long-term results of treatment with bosentan in adult Eisenmenger s syndrome patients with Down s syndrome related to congenital heart di- sease. BMC Cardiovasc Disord. 2013;13:74. S4.4.6.2-9. Duffels MGJ, Vis JC, van Loon RLE, et al. Down patients with Eisenmenger syndrome: is bosentan treatment an option? Int J Cardiol. 2009;134:378 83. S4.4.6.2-10. Serino G, Guazzi M, Micheletti A, et al. Effect of bosentan on exercise capacity and clinical worsening in patients with dual Down and Eisenmenger syndrome. Clin Med Insights Cardiol. 2013;7:29 34. S4.4.6.2-11. Zhang Z-N, Jiang X, Zhang R, et al. 