Drug Class
Table 5. Medical Therapies for PAH (continued) Dosage Form Comments
Generic (Brand)
Phospho- diesterase Inhibitors
Sildenafil (Revatio®
)
PO (20 mg tablet tid, 4-6 h apart)
IV (10 mg tid)
Indicated for the treatment of PAH (WHO Group I) to improve exercise ability
Common Adverse Effects:a Headache, dyspepsia, flushing, epistaxis Other Adverse Effects:a Non-arteritic anterior ischemic optic neuropathy, including permanent loss of vision (rare); may potentiate hypotensive effects of nitrates, alpha blockers
Tadalafil (Adcirca®
)
PO (2x20 mg tablets daily)
Indicated for the treatment of PAH to improve exercise ability
Warnings: Use with caution/reduce dose in hepatic and renal impairment; avoid if ClCr <30 mL/min. Common Adverse Effects: Headache, myalgia, nasopharyngitis, flushing, respiratory infection, extremity pain, nausea, back pain, dyspepsia, nasal congestion
a See full prescribing information for complete list of adverse effects. b Therapy should be referred to a center with experience in managing patients with PAH for proper dosing of
these agents.
a
b
To maintain oxygen at 92%. APAH, associated pulmonary arterial hypertension; ERA, endothelin receptor antagonist; HPAH, heritable pulmonary arterial hypertension; IPAH, idiopathic pulmonary arterial hypertension; IV, intravenous; PAH, pulmonary arterial hypertension; PDE-5, phosphodiesterase type 5; subcutaneous; WHO, World Health Organization.
These therapies have been evaluated mainly for patients with IPAH or PAH associated with connective tissue disease or anorexigen use. Extrapolation to other forms of PAH should be made with caution.
