Which PAH-specific therapies are indicated for patients with idiopathic pulmonary arterial hypertension (IPAH) in whom calcium channel blockers (CCBs) are contraindicated?

Updated: Jul 08, 2020
  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

For patients with IPAH in whom CCBs are contraindicated, ineffective, or poorly tolerated, ACCP guidelines recommend using the patient’s World Health Organization’s modified New York Heart Association (NYHA) functional class (WHO FC) to guide the choice of PAH-specific therapy. [43, 44] PAH-specific therapies by functional class from the ACCP are as follows: [45]

  • WHO FC II – Monotherapy may be initiated with a currently approved endothelin receptor antagonist (ETA), phosphodiesterase-5 (PDE5) inhibitor, or the soluble guanylate cyclase stimulator riociguat.

  • WHO FC III (treatment-naïve) - Monotherapy be initiated with a currently approved ERA, a PDE5 inhibitor, or the soluble guanylate cyclase stimulator riociguat.

  • WHO FC III (treatment-naïve with rapid progression or other markers of poor prognosis) - Parenteral prostanoid.

  • WHO FC III (non-treatment-naïve with rapid progression or other markers of poor prognosis) - Parenteral or inhaled prostanoid.

  • WHO FC IV (treatment-naïve) - Parenteral prostanoid. If unable to manage parenteral prostanoid therapy, use inhaled prostanoid in combination with an ERA.


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