The current treatment of pulmonary arterial hypertension: time to redefine success

Chest. 2006 Oct;130(4):1198-202. doi: 10.1378/chest.130.4.1198.


In the past decade, three classes of medications have been approved for the treatment of pulmonary arterial hypertension. A review of the clinical trial data for the prostanoids, endothelin antagonists, and phosphodiesterase-5 inhibitors has shown that all agents have similar efficacy on the 6-min walk distance over 12 to 16 weeks, which was the primary end point in the randomized clinical trials. However, little is known about their long-term efficacy or about how these drugs affect the underlying disease, if at all. Successful therapy is currently defined as an improvement in exercise tolerance over a 4-month period. Future trials need to better characterize how therapies affect the pulmonary vasculature pathologically, biologically, and hemodynamically, and whether survival is actually improved.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Bosentan
  • Endothelins / antagonists & inhibitors*
  • Epoprostenol / therapeutic use
  • Exercise Test / drug effects*
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Long-Term Care
  • Phosphodiesterase Inhibitors / therapeutic use*
  • Piperazines / therapeutic use
  • Prostaglandins / therapeutic use*
  • Purines
  • Randomized Controlled Trials as Topic
  • Sildenafil Citrate
  • Sulfonamides / therapeutic use
  • Sulfones
  • Treatment Outcome


  • Endothelins
  • Phosphodiesterase Inhibitors
  • Piperazines
  • Prostaglandins
  • Purines
  • Sulfonamides
  • Sulfones
  • Sildenafil Citrate
  • Epoprostenol
  • Bosentan