Indications

Pulmonary Hypertension (see Pulmonary Hypertension, [[Pulmonary Hypertension]])

• Congenital Heart Disease with L->R Shunt-Associated Pulmonary Hypertension
• Connective Tissue Disease-Associated Pulmonary Hypertension
• Idiopathic Pulmonary Arterial Hypertension (IPAH) (see Idiopathic Pulmonary Arterial Hypertension, [[Idiopathic Pulmonary Arterial Hypertension]])

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Contraindications

• Pregnancy: teratogenic

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Pharmacology

• Endothelin Receptor Antagonist (see Endothelin Receptor Antagonists, [[Endothelin Receptor Antagonists]])
  • xxx

Metabolism

• xxx

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Administration

• PO: xxx

Dose Adjustment

• Hepatic
• Renal

Drug Interactions

• Cyclosporine A: coadministration of cyclosporine A and bosentan markedly increases plasma bosentan concentration
• Glyburide: coadministration of glyburide and bosentan may result in increased liver function tests

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Adverse Effects

Cardiovascular Adverse Effects

• Fluid Retention/Peripheral Edema (see Peripheral Edema, [[Peripheral Edema]])
  • Known effect of endothelin receptor antagonists
  • May occur within the first few weeks of starting bosentan
• Hypotension (see Hypotension, [[Hypotension]])

Dermatologic Adverse Effects

• Rash

Gastroenterologic Adverse Effects

• Drug-Induced Hepatotoxicity (see Drug-Induced Hepatotoxicity, [[Drug-Induced Hepatotoxicity]])
  • Transaminitis: elevated AST/ALT

Hematologic Adverse Effects

• Anemia (see Anemia, [[Anemia]])

Immunologic Adverse Effects

• Angioedema (see Urticaria-Angioedema, [[Urticaria-Angioedema]])

Pulmonary Adverse Effects

• Pulmonary Edema: may occur in patients with pulmonary veno-occlusive disease (VOD), suggesting this as an alternate diagnosis (see Pulmonary Veno-Occlusive Disease, [[Pulmonary Veno-Occlusive Disease]])

Other Adverse Effects

• xxx

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References

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