Assessment and treatment of pulmonary arterial hypertension: an Australian perspective in 2006

Intern Med J. 2007 Jan;37(1):38-48. doi: 10.1111/j.1445-5994.2006.01242.x.

Abstract

Pulmonary arterial hypertension is a group of diseases which forms a small subset of those with elevated pulmonary artery pressure (pulmonary hypertension). The recent development of selective pulmonary vasodilator has lead to a substantial resurgence of interest in what have been previously regarded as rare and incurable diseases. This review aims to describe the spectrum of pulmonary vascular diseases, the evolving understanding as to pathogenesis, the evolving evidence of efficacy for drug therapies, trying to put this into a contemporary Australian context. Several key pathogenic pathways may be involved: prostacycline, Nitric Oxide-cGMP-phosphodiesterase 5 and endothelin- all of which are exploited for therapeutic benefit by newly available drug therapies. A recently modified classification system reasserts the importance of precise diagnosis. The cardinal symptom of exertional dyspnea warrants careful evaluation in an attempt to prevent (frequently occurring) substantial delay in diagnosis. Echocardiogram is the cornerstone of screening for pulmonary arterial hypertension; however, a detailed evaluation including a carefully performed right heart catheterisation with sufficient data to allow calculation of pulmonary vascular resistance is key to accurate diagnosis. These new approaches to therapy are already substantially improving quality of life and prognosis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Arterioles / pathology
  • Arterioles / physiopathology
  • Australia / epidemiology
  • Bosentan
  • Cardiac Catheterization
  • Diagnostic Imaging
  • Disease Progression
  • Dyspnea / etiology
  • Endothelin A Receptor Antagonists
  • Epoprostenol / analogs & derivatives
  • Epoprostenol / therapeutic use
  • Exercise Test
  • Female
  • Forecasting
  • Heart-Lung Transplantation
  • Humans
  • Hypertension, Pulmonary / classification
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / epidemiology*
  • Hypertension, Pulmonary / therapy
  • Iloprost / therapeutic use
  • Lung Transplantation
  • Male
  • Middle Aged
  • Nitric Oxide / metabolism
  • Piperazines / therapeutic use
  • Prognosis
  • Pulmonary Artery / pathology
  • Pulmonary Artery / physiopathology
  • Purines
  • Randomized Controlled Trials as Topic
  • Sildenafil Citrate
  • Sulfonamides / therapeutic use
  • Sulfones
  • Vascular Resistance
  • Vasodilator Agents / therapeutic use
  • Ventricular Dysfunction, Right / etiology
  • Ventricular Dysfunction, Right / prevention & control

Substances

  • Endothelin A Receptor Antagonists
  • Piperazines
  • Purines
  • Sulfonamides
  • Sulfones
  • Vasodilator Agents
  • Nitric Oxide
  • Sildenafil Citrate
  • Epoprostenol
  • Iloprost
  • Bosentan
  • treprostinil