Table 1 Basic characteristics of enrolled studiesnote: RCT-randomized controlled studies; NRCT: Non-randomized controlled studies; ASD-atrial septal defect; VSD-ventricular septal defect; PDA-patent ductus arteriosus; Com-other complex or compound CHDs; DS-Down’s syndrome; CFG-cardiac functional grading
Author | Year | Type | Country | n | Drugs | Age | Etiological classification | DS | Time (mo) | CFG | Primary outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|
ERAs | |||||||||||
Galiè [10] | 2006 | RCT | Italy | 37 | Bosentan | 37.2 (12.0) | ASD (21.6%)/VSD (64.9%)/Com (13.5%) | No | 4 | - | 6MWD |
Crepaz [11] | 2013 | NRCT | Italy | 7 | Bosentan | 29.6 (11.2) | VSD (100%) | Yes | 24 | 3.0 (-) | 6MWD |
Serino [12] | 2013 | NRCT | Italy | 7 | Bosentan | 31.7 (-) | VSD (71.4%)/Com (28.6%) | Yes | 24 | 3.3 (0.5) | 6MWD |
D’alto [13] | 2007 | NRCT | Italy | 22 | Bosentan | 38.0 (10.0) | ASD (4.5%)/VSD (54.5%)/Com (41.0%) | No | 12 | 3.1 (0.7) | 6MWD |
Kaya [14] | 2012 | NRCT | Turkey | 22 | Bosentan | 31.0 (12.0) | ASD (26.1%)/VSD (65.2%)/PDA (8.7%) | No | 24 | 3.2 (0.4) | 6MWD |
Duffels [15] | 2009 | NRCT | Netherlands | 24 | Bosentan | 38.0 (-) | ASD (58.3%)/VSD (29.2%)/Com (12.5%) | Yes | 3 | - | 6MWD |
Gatzoulis [16] | 2018 | RCT | United Kingdom | 114 | Macetetan | 33.0 (12.8) | - | Yes | 4 | 2.4 (0.5) | 6MWD |
Zuckerman [17] | 2011 | NRCT | America | 17 | Anrisentan | 32.2 (11.9) | ASD (52.9%)/VSD (41.2%)/Com (5.9%) | Yes | 30 | - | 6MWD |
PDE5i | |||||||||||
Zhang [18] | 2011 | NRCT | China | 84 | Sildenafil | 28.0 (9.0) | ASD (29.8%)/VSD (40.5%)/PAD (27.4%)/Com (2.4%) | No | 12 | 2.6 (0.7) | 6MWD |
Mukhopadhyay [19] | 2011 | RCT | India | 28 | Tadalafil | 29.3 (11.7) | ASD (50.0%)/VSD (46.4%)/ Com (3.6%) | No | 1.5 | 2.2 (0.4) | 6MWD |
Prostanoids | |||||||||||
Chon [20] | 2017 | NRCT | Korea | 11 | Iloprost | 44.2 (12.2) | ASD (9.1%)/VSD (54.5%)/PAD (27.3%)/Com (9.1%) | No | 12 | 3.4 (0.5) | 6MWD |
Nashat [21] | 2019 | RCT | United Kingdom | 16 | Iloprost | 47.3 (9.8) | VSD (68%)/Com (32%) | No | 29 | 3 (-) | 6MWD |
Fernandes [22] | 2003 | NRCT | America | 8 | Epoprostenol | 36.3(14.9) | ASD (37.5%)/VSD (25%)/PAD (12.5%)/Com (12.5%) | No | 3 | 3.8 (0.4) | 6MWD |