| 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 |
