Version May 2024
| Names | Dose/titration | Side effects | Comments |
| Phosphodiesterase-5 inhibitor | |||
| Sildenafil (oral or IV) | Oral: 1 mg/kg every 6 to 8 hours; start with low dose (0.3 to 0.5 mg/kg/dose) and increase gradually to 1 mg/kg/dose as tolerated; slower as outpatient. Maximal dose of 10 mg every 8 hours per EMA guidelines for infants. Intravenous: 0.25 to 0.5 mg/kg/dose every 6 to 8 hours (titrate slowly and administer over 60 minutes). | Hypotension, GER, irritability (headache), bronchospasm, nasal stuffiness, fever, rarely priapism. | Monitor for adverse effects, lower the dose or switch to alternate therapy if not tolerated. |
| Endothelin receptor antagonist | |||
| Bosentan (oral) | 1 mg/kg orally every 12 hours as starting dose; may increase to 2 mg/kg twice a day in 2 to 4 weeks, if tolerated and liver enzymes stable. | Liver dysfunction especially during viral infections, VQ mismatch, hypotension, anemia (edema and airway issues rare in infants). | Monitor LFTs monthly (earlier with respiratory infections); monitor CBC quarterly. Teratogenicity precautions for caregivers. |
| Prostacyclin analogs | |||
| Iloprost (inhaled) | 2.5 to 5 micrograms every 2 to 4 hours. Can be given as continuous inhalation during mechanical ventilation. Can titrate dose from 1 to 5 micrograms and frequency from every 4 hours to continuous. | Bronchospasm, hypotension, ventilator tube crystallization and clogging, pulmonary hemorrhage, prostanoid side effects (GI disturbances), may be teratogenic to caregivers. | Need close monitoring for clogged tubing, may need further dilution. May need bronchodilators or inhaled steroid pretreatment with bronchospasm. |
| Epoprostenol (IV) | Start at 1 to 2 nanograms/kg/minute, titrate up slowly every 4 to 6 hours to 20 nanograms/kg/minute; need to increase dose at regular intervals because of tachyphylaxis. Further increases as guided by clinical targets and avoiding adverse effects. | Hypotension, VQ mismatch, GI disturbances. Needs dedicated line. Very short half-life with high risk for rebound PH with brief interruption of therapy. Line-related complications include infection, clogging, breaks in line, thrombosis, arrhythmia. | Monitor closely if added to other vasodilator therapies, such as milrinone; careful attention to line care is essential. |
| Treprostinil (IV or subcutaneous) | Start at 2 nanograms/kg/minute and titrate every 4 to 6 hours up to 20 nanograms/kg/minute, then slow increase dose as tolerated (dose often 1.5 to 2 times greater than equivalent epoprostenol dose, if switching medications). | Subcutaneous: local site pain. Intravenous: similar risks as with epoprostenol, but treprostinil has a longer half-life, which reduces risk for severe PH with interruption of infusion. | Site pain managed with local and systemic measures. |
آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟
