What are the treatment options for functional class IV pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Patients with WHO functional class IV disease are typically started on parenteral prostanoid therapy.

For patients who progress or are poorly responsive to initial therapy, practitioners typically add agents from a different class. Agents within the same class (including PDE-5 inhibitors and guanylate cyclase stimulators) should not be used together.

For more details on the specifics of agents within these classes, see the Medication.

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