Pulmonary hypertension: diagnosis and management

Mayo Clin Proc. 2009 Feb;84(2):191-207. doi: 10.4065/84.2.191.


Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. Effective management requires timely recognition and accurate diagnosis of the disorder and appropriate selection among therapeutic alternatives. Despite progress in treatment, obstacles remain that impede the achievement of optimal outcomes. The current article provides an overview of the pathobiologic mechanisms of pulmonary arterial hypertension, including genetic substrates and molecular and cellular mechanisms, and describes the clinical manifestations and classification of pulmonary arterial hypertension. The article also reviews established approaches to evaluation and treatment, with emphasis on the appropriate application of calcium channel blockers, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors. In addition, the authors discuss unresolved issues that may complicate patient management, such as the clinical importance of mild or exercise-related pulmonary arterial hypertension, and they identify avenues by which treatment may advance in the future through the use of combination treatment, outcomes assessment, and exploration of alternative pharmacologic strategies.

MeSH terms

  • Activin Receptors, Type II / genetics
  • Algorithms
  • Angina, Unstable / etiology
  • Antihypertensive Agents / therapeutic use
  • Bone Morphogenetic Protein Receptors, Type II / genetics
  • Calcium Channel Blockers / therapeutic use
  • Drug Therapy, Combination
  • Endothelin Receptor Antagonists
  • Exercise
  • Female
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / genetics
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy*
  • Insurance Coverage
  • Insurance, Health
  • Phosphodiesterase Inhibitors / therapeutic use
  • Pregnancy
  • Severity of Illness Index
  • Syncope / etiology


  • Antihypertensive Agents
  • Calcium Channel Blockers
  • Endothelin Receptor Antagonists
  • Phosphodiesterase Inhibitors
  • ACVRL1 protein, human
  • Activin Receptors, Type II
  • BMPR2 protein, human
  • Bone Morphogenetic Protein Receptors, Type II