Table 1.
Beneficial evidences of drugs for pulmonary hypertensive crisis after congenital heart surgery in children and adults
| Drug, study | No CHD cases | Beneficial evidences for PAH-CHD children | Beneficial evidences for PAH-CHD adults |
|---|---|---|---|
| iNO | |||
| Miller et al.21 | 124 | Fewer PHC, shorter postoperative courses without side effects | |
| Journois et al.22 | 64 | Lower postoperative mortality | |
| Beghetti et al.20 | 7 | Reduced PAP | |
| Alprostadil | |||
| Lang et al.38 | 5 | Maintaining and/or reopening DA | |
| Masutani et al.39 | 1 | Effective treatment for PPAH complicated with TGA/IVS | |
| Dong et al.40 | 31 | Reduced mPAP, lower death risk after CHD surgery (mean age: 25.6 ± 6.3 years; range: 13–51) | |
| Kermode et al.41 | 20 | Both alprostadil and epoprostenol were effective vasodilators | |
| Iloprost | |||
| Limsuwan et al.67 | 12 | Effective alternative treatment for postoperative PHC | |
| Loukanov et al.33 | 15 | Favorable safety profile compared with iNO | |
| Kirbas et al.68 | 16 | Both iloprost and iNO were effective and comparable | |
| Bosentan | |||
| Eicken et al.76 | 1 | Clinical improvement with lower PVR after DA occlusion and bosentan treatment (age: years) | |
| Hoetzenecker et al.77 | 1 | Successful surgical closure of ASD with severe PAH after 10 months bosentan therapy (age: 71 years) | |
| Ovaert et al.78 | 9 | Improved oxygen saturation in patients with failing Fontan circulation after bosentan medication (mean age: 12.12 years; range: 4.41–33.41) | |
| Schuuring et al.80 Beghetti et al.81,82 |
3 | Advanced therapy with bosentan reduced perioperative pulmonary vasoconstriction (age: 25, 38, and 39 years) | |
| Milrinone | |||
| Momeni et al.91 | 5a | Well tolerated and helpful for weaning from CPB (mean age: 65 ± 11 years) | |
| Singh et al.97 | 35 | Inhaled milrinone significantly decreased mPAP, PVRI without significant effects on systemic hemodynamics | |
| Sildenafil | |||
| Atz et al.105 | 1 | Additive effects with iNO for PHC and facilitated weaning from iNO | |
| Nemoto et al.106 | 66 | Safe and effective alternate for PAH-CHD | |
| Lee et al.107 | 7 | Facilitated iNO withdrawal, prevented rebound | |
| Palma et al.34 | 38 | Controlled PAH-CHD safely and effectively | |
| Lammers et al.109 | 1 | Intravenous sildenafil was effective in treating PH crisis | |
| Stocker et al.110 | 15 | Intravenous sildenafil augmented the effects of iNO | |
| Stocker et al.110, Schulze-Neick et al.111 |
24 | Intravenous sildenafil was as effective as iNO | |
| Sildenafil alone or with milrinone | |||
| Fraisse et al.119 | 17 | Intravenous sildenafil, alone or combination with milrinone reduced PAP, shortened time to extubation and ICU stay |
Note
ASD: atrial septal defect; DA: ductus arteriosus; ICU: intensive care unit; mPAP: mean pulmonary arterial pressure; iNO: inhaled nitric oxide; PAP: pulmonary arterial pressure; PPAH: persistent pulmonary arterial hypertension; PHC: pulmonary hypertensive crises; PVR: pulmonary vascular resistance; PVRI: pulmonary vascular resistance index; TGA/IVS: transposition of the great arteries and an intact ventricular septum.
a
There were 5 ASD patients among 73 cases.