Study Study Population n Study Design Drug Main Findings
Galié et al. (BREATH-5), 2006 [69] Septal defects
>12 years
37 Randomized Controlled Trial Bosentan improved exercise capacity and pulmonary hemodynamics
Gatzoulis et al. 2005 [70] Eisenmenger
Adults
10 Prospective open trial Bosentan improved exercise capacity, pulmonary hemodynamics and  RV systolic function
Sitbon et al., 2006 [71] ASD, VSD and Eisenmenger
Adults
27 Retrospective analysis Bosentan improved functionnal class, exercise capacity and pulmonary hemodynamic
Jing et al. , 2010 [72] Heart septal defects
Adults
34 Multi-open label trial Bosentan improved functionnal class, exercise capacity and pulmonary hemodynamic
Uhm et al. 2010 [73] post-operative (AVSD repair, Fontan)
Children
75 Retrospective analysis Oral Sildenafil Well tolerated, no significant clinical improvement
Zeng et al., 2011 [74] AVSD, PDA
Adults
55 Prospective Multicenter trial Oral Sildenafil improved exercise capacity and pulmonary hemodynamics without any side effects on systemic vasculature
Schulze-Neick et al., 2003 [75] AVSD, PDA, miscellaneous
Children
12 Prospective trial IntravenousSildenafil more effective than inhaled NO to improve pulmonary hemodynamics; increased post-operative intrapulmonary shunting and had no significant clinical benefits
CHD: Congenital Heart Disease; ASD: Atrial Septal Defect; VSD: Ventricular Septal Defect; PDA: Patent Ductus Arteriosus; NO: Nitric Oxyde
Table 5: Trials on endothelin receptor antagonists and sildenafil in CHD affecting the right heart.