2008 fourth world symposium on pulmonary arterial hypertension classification

The classification system for pulmonary hypertension was revised at the 4 World Symposium on Pulmonary Hypertension held in Dana Point, California, in 2008 .

This system is as follows:

• group 1: pulmonary arterial hypertension
  • 1.1: idiopathic pulmonary arterial hypertension
  • 1.2: heritable pulmonary hypertension
  • 1.3: drug- and toxin-induced pulmonary hypertension
  • 1.4: pulmonary hypertension associated with 
    • connective tissue disease
    • HIV infection
    • portal hypertension - portopulmonary hypertension
    • congenital heart disease
    • chronic hemolytic anemia
    • schistosomiasis
  • 1.5: persistent pulmonary hypertension of the newborn
  • 1.6  pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis
• group 2: pulmonary hypertension due to left-sided heart disease
  • 2.1: systolic dysfunction
  • 2.2: diastolic dysfunction
  • 2.3: valvular disease
• group 3: pulmonary hypertension due to lung diseases or hypoxia
  • chronic obstructive pulmonary disease
  • interstitial lung disease
  • sleep apnea
  • alveolar hypoventilation syndrome
  • chronic high altitude syndrome 
• group 4: pulmonary hypertension due to chronic pulmonary thromboembolism
• group 5: pulmonary hypertension due to unclear multifactorial mechanisms
  • myeloproliferative disorders
  • splenectomy
  • systemic disorders
    • sarcoidosis
    • pulmonary Langerhans cell histiocytosis
    • lymphangioleiomyomatosis
    • neurofibromatosis type I
    • vasculitides
  • metabolic disorders
    • glycogen storage disease
    • Gaucher disease
    • thyroid disorders
  • other conditions
    • fibrosing mediastinitis
    • tumoral obstruction
    • dialysis-dependent chronic renal failure