Table 1: Medications for SCD-related Pulmonary Hypertension.
Druga | Side Effectsb | Pregnancy Categoryb |
---|---|---|
Hydroxyurea Increases hemoglobin F levels in Red Blood Cells (RBCs) |
Nausea, Vomiting, Constipation, Diarrhea, Mucositis, Acute pulmonary reactions (rare), Genetic mutation (long-term use), Myelosuppression, Secondary leukemia (long-term use), Elevated BUN/Creatinine, Hyperuricemia, Renal failure, Rash, Hyperpigmentation, Skin ulcers, Gangrenous disorder |
Pregnancy Category: D Lactation: excreted in breast milk, do not nurse |
Bosentan Competitive antagonist of endothelin-1; blocks endothelin receptors on vascular endothelium and smooth muscle resulting in inhibition of vasoconstriction |
> 10%: Hgb decreased; > 1 g/dL; Inhibition of spermatogenesis; Headache; Nasopharyngitis; Transaminitis; Respiratory tract infection 1-10%: Edema, lower limb, Flushing, Hypotension, Hepatic abnormalities, Palpitations, Anemia, Dyspepsia, Edema, Fatigue, Pruritus < 1%: Hyperbilirubinemia, Vasculitis, Jaundice, Leukopenia, Thrombocytopenia, Leukocytoplastic |
Pregnancy Category: X Lactation: Not known if excreted in breast milk; not recommended |
Ambrisentan High affinity endothelin (ETa) receptor subtype antagonist, resulting in inhibition of vasoconstriction |
> 10%: Peripheral edema, Headache 1-10%: Nasal congestion, Palpitations, Constipation, Dyspnea, Flushing, Abdominal pain, Nasopharyngitis, Sinusitis |
Pregnancy Category: X Lactation: Excretion in milk unknown; not recommended |
Nitric Oxide (inhaled) Relaxes vascular smooth muscle, resulting in pulmonary vasodilation |
> 10%: Hypotension, withdrawal 1-10%: Atelectasis, Hematuria, Hyperglycemia, Sepsis, Infection, Cellulitis, Stridor |
Pregnancy Category: C Lactation: not known if excreted in breast milk |
Arginine Supplementation Converted by nitric oxide synthase to citrulline plus nitric oxide |
1-10%: Headache, Flushing, Nausea, Vomiting, Numbness, Local venous irritation < 1%: Macular rash, Swelling of hands and feet, Hematuria, Hyperkalemia, Skin burn/necrosis, Loss of consciousness, Perioral tingling |
Pregnancy Category: B Lactation: Enters breast milk, use caution |
a[2]; bAdapted from http://reference.medscape.com/drugs.