What is the role of goal-oriented therapy 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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Answer

A newer concept of goal-oriented therapy has been developed after observations that patients with an inadequate clinical response to an initial therapy have a much worse prognosis. Thus:

  • For WHO FC III or IV PAH patients with unacceptable clinical status despite established PAH-specific monotherapy, a 2nd class of PAH therapy to improve exercise capacity is recommended in addition to referral to a center with expertise in the evaluation and treatment of complex patients with PAH.

Finally, for patients who do not fall into the above categories, reference to the ACCP Guideline for specific evidence-based scenarios and simultaneous referral to an expert PAH center is suggested. Failure of medical therapy dictates prompt consideration for lung transplantation.


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