Table 1.
Drug | Dosage/Route of Administration | Common Adverse Effects |
---|---|---|
Nitric Oxide | Inhaled 5–20 ppm (OI > 20); Wean iNO—FiO2 < 60%; PaO2 > 60 mmHg; Keep SpO2 ≥ 91; Infants on chronic iNO therapy—wean last 5 ppm gradually to ↓ rebound PH | Monitor methemoglobin during use |
PDE5 Inhibitor—Sildenafil | Oral—0.5 mg/kg q8–6 ↑ to 2 mg/kg q8–6 over 2 weeks IV (continuous infusion): 0.4 mg/kg over 3 h (LD); Infusion—0.07 mg/kg/h | Systemic hypotension; watch for worsening oxygenation due to vasodilation of unventilated areas of the lung; flushing, diarrhea, nasal congestion, priapism |
Prostanoids *—Epoprostenol | IV/continuous Aerosolization—2 ng/kg/min ↑ to 20–50 ng/kg/min | Systemic hypotension, nausea, vomiting, flushing, diarrhea, thrombocytopenia, bloodstream infection |
Treprostinil | Subcutaneous—1.5 ng/kg/min ↑ to 20–40 ng/kg/min; Inhaled—3–9 breaths (6 µg/breath) q6 | Infusion site pain, site infection, flushing, diarrhea, nausea, jaw pain, bloodstream infection |
Iloprost | Inhalation: 1–2.5 µg/kg q2–4 h | Cough, syncope, hypotension, flushing, headache, trismus |
PDE3 Inhibitor—Milrinone | IV—50 µg/kg (LD) over 1–2 h; Infusion—20–75 µg/kg/min | Hypotension, tachycardia, arrhythmias, thrombocytopenia, low potassium, bronchospasm |
Endothelial Receptor Antagonist—Bosentan | Oral: 1 mg/kg q12 | Hypotension, flushing, hepatotoxicity, anemia, thrombocytopenia, teratogenesis |
IV: intravenous; SC: subcutaneous; LD: loading dose. Sildenafil is commonly administered per oral (PO) occasionally IV. * Second line drugs are not well studied in neonates and infants with PH.