Diagnosis and Assessment of Disease Selecting a Treatment Regimen
Drug Class
Table 5. Medical Therapies for PAH (continued) Dosage Form Comments
Generic (Brand)
Prostanoids Inhaled treprostinil (Tyvaso®
)
Initial: 3 breaths (18 µg) qid Maximum: 9 breaths (54 µg) qid Tyvaso must be used only with the Tyvaso Inhalation System.
Endothelin Antagonists
Bosentan (Tracleer®
) PO
(62.5-, 125 mg tablets) (62.5 mg bid for 4 wks, then 125 mg bid)
Indicated to increase walk distance in patients with WHO Group I pulmonary arterial hypertension and NYHA Class III symptoms.
Warnings: Use with caution in hepatic and renal insufficiency.
Common Adverse Effects: Cough, headache, throat irritation, nausea, flushing, syncope
Indicated for the treatment of PAH (WHO Group I) in patients with WHO Class II - IV symptoms, to improve exercise ability and decrease the rate of clinical worsening.
Common Adverse Effects:a Headache, nasopharyngitis, flushing, lower limb edema Other Adverse Effects:a Possible dose-dependent, reversible elevations of ALT/AST to at least 3 times upper limit of normal with potential for serious liver injury. Potential injury to fetus. ALT/AST levels must be measured prior to initiation of treatment and then monthly. If elevated aminotransferase levels are seen, changes in monitoring and treatment must be initiated. If elevations are accompanied by clinical symptoms of liver injury, or increases in bilirubin ≥ 2 x ULN, treatment should be stopped.
Ambrisentan (Letairis®
)
PO (5- or 10 mg tablet daily)
Indicated for the treatment of PAH (WHO Group I) in patients with WHO class II or III symptoms to improve exercise capacity and delay clinical worsening.
Common Adverse Effects:a Peripheral edema, headache, nasal congestion, flushing Other Adverse Effects:a Possible elevation of ALT and AST to at least 3 times upper limit of normal with potential for serious liver injury. Potential injury to a fetus. Aminotransferase must be measured prior to initiation of ambrisentan and at least every month. If elevations are >3 x ULN and ≤5 x ULN, reduce daily dose or interrupt treatment and continue to monitor every 2 wks until the levels are <3 x ULN. If elevations are >5 x ULN and ≤8 x ULN, interrupt treatment and monitor until levels are <3 x ULN. Then re-initiate treatment with more frequent measurement of aminotransferase levels. If elevations are >8 x ULN, treatment should be stopped.
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