What is the prognostic value of a vasodilator challenge for idiopathic pulmonary arterial hypertension (IPAH)?

Updated: Jul 08, 2020
  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Patients who do not have an acute vasodilator response to a vasodilator challenge have a worse prognosis on long-term oral PAH-specific therapy compared with those who have an initial response. However, the absence of an acute response to intravenous or inhaled vasodilators does not preclude the use of intravenous prostanoid therapy. In fact, continuous intravenous prostanoid therapy is strongly suggested for these patients because CCBs are contraindicated.

Patients receiving epoprostenol or intravenous treprostinil therapy must have a central venous catheter placed surgically and receive their initial dose in an inpatient setting. This allows for monitoring of acute adverse effects and provides the opportunity for the patient and support personnel to master the drug preparation and administration technique before discharge.

Continuous intravenous prostanoid therapy is delivered via an ambulatory infusion pump.

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