Management of pulmonary arterial hypertension

J Am Coll Cardiol. 2015 May 12;65(18):1976-97. doi: 10.1016/j.jacc.2015.03.540.


Pulmonary hypertension (PH) is common and may result from a number of disorders, including left heart disease, lung disease, and chronic thromboembolic disease. Pulmonary arterial hypertension (PAH) is an uncommon disease characterized by progressive remodeling of the distal pulmonary arteries, resulting in elevated pulmonary vascular resistance and, eventually, in right ventricular failure. Over the past decades, knowledge of the basic pathobiology of PAH and its natural history, prognostic indicators, and therapeutic options has exploded. A thorough evaluation of a patient is critical to correctly characterize the PH. Cardiac studies, including echocardiography and right heart catheterization, are key elements in the assessment. Given the multitude of treatment options currently available for PAH, assessment of risk and response to therapy is critical in long-term management. This review also underscores unique situations, including perioperative management, intensive care unit management, and pregnancy, and highlights the importance of collaborative care of the PAH patient through a multidisciplinary approach.

Keywords: echocardiography; endothelin receptor antagonists; hemodynamics; phosphodiesterase type 5 inhibitors; prostacyclins; pulmonary arterial hypertension.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antihypertensive Agents / therapeutic use
  • Atrial Septum / surgery
  • Calcium Channel Blockers / therapeutic use
  • Cardiac Catheterization
  • Echocardiography
  • Endothelin Receptor Antagonists / therapeutic use
  • Exercise Test
  • Extracorporeal Membrane Oxygenation
  • Heart Ventricles / pathology
  • Humans
  • Hypertension, Pulmonary / classification
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / therapy*
  • Lung Transplantation
  • Medical History Taking
  • Phosphodiesterase 5 Inhibitors / therapeutic use
  • Physical Examination
  • Pulmonary Artery / pathology
  • Registries
  • Tunica Intima / pathology
  • Tunica Media / pathology
  • Vascular Remodeling


  • Antihypertensive Agents
  • Calcium Channel Blockers
  • Endothelin Receptor Antagonists
  • Phosphodiesterase 5 Inhibitors