Idiopathic pulmonary arterial hypertension

Semin Respir Crit Care Med. 2013 Oct;34(5):560-7. doi: 10.1055/s-0033-1355439. Epub 2013 Sep 13.


Idiopathic pulmonary arterial hypertension (IPAH), formerly called primary pulmonary hypertension, is a rare disease (incidence and prevalence rates of approximately one and six cases per million inhabitants, respectively) with different clinical phenotypes. A group of diverse conditions manifest pulmonary arterial hypertension (PAH) and share similar pathological and/or clinical findings with IPAH. By definition, IPAH is diagnosed only after alternative diagnoses have been ruled out. Extensive investigation is needed to determine if PAH is associated with thyroid diseases, infectious diseases, autoimmune conditions, exposure to certain drugs (particularly anorexigens), certain genetic mutations, and so on. The presence of genetic abnormalities and risk factors (such as specific drug exposures) reinforces the "multiple hit" concept for the development of pulmonary hypertension. Fortunately, within the past two decades, therapeutic options have become available for IPAH, resulting in improved survival and clinical outcomes. At least seven different compounds have been registered for PAH treatment. However, even with aggressive PAH-specific therapy, mortality rates remain high (∼40% at 5 years). Given the high mortality rates, the use of combinations of agents that work by different pathways has been advocated (either as "add-on" therapy or initial "up front" therapy). Further, new therapeutic agents and treatment strategies are on the near horizon, aiming to further improve survival from the remarkable progress already seen.

MeSH terms

  • Aminorex / adverse effects
  • Antihypertensive Agents / therapeutic use*
  • Appetite Depressants / adverse effects
  • Bosentan
  • Dasatinib
  • Epoprostenol / analogs & derivatives
  • Epoprostenol / therapeutic use
  • Familial Primary Pulmonary Hypertension
  • Fenfluramine / adverse effects
  • Genetic Predisposition to Disease
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Iloprost / therapeutic use
  • Phenylpropionates / therapeutic use
  • Piperazines / therapeutic use
  • Protein Kinase Inhibitors / adverse effects
  • Purines / therapeutic use
  • Pyrazoles / therapeutic use
  • Pyridazines / therapeutic use
  • Pyrimidines / adverse effects
  • Pyrimidines / therapeutic use
  • Risk Factors
  • Sildenafil Citrate
  • Sulfonamides / therapeutic use
  • Sulfones / therapeutic use
  • Thiazoles / adverse effects
  • Vasodilator Agents / therapeutic use*


  • Antihypertensive Agents
  • Appetite Depressants
  • Phenylpropionates
  • Piperazines
  • Protein Kinase Inhibitors
  • Purines
  • Pyrazoles
  • Pyridazines
  • Pyrimidines
  • Sulfonamides
  • Sulfones
  • Thiazoles
  • Vasodilator Agents
  • Fenfluramine
  • Aminorex
  • Sildenafil Citrate
  • Epoprostenol
  • ambrisentan
  • Iloprost
  • Bosentan
  • Dasatinib
  • riociguat
  • treprostinil
  • macitentan