Table 1: Medications for SCD-related Pulmonary Hypertension.

Druga Side Effectsb Pregnancy Categoryb


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


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


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