Fifth world symposium on pulmonary arterial hypertension classification

In 2013, the 5 World Symposium on pulmonary hypertension took place in Nice, France and modified the classification system for pulmonary hypertension.

The modified system divides pulmonary hypertension into five groups:

• group 1: pulmonary arterial hypertension (disorders of the pulmonary arteries themselves)
  • 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 - HIV-associated pulmonary arterial hypertension
    • portal hypertension
    • congenital heart disease
    • schistosomiasis
• group 2: pulmonary hypertension due to left-sided heart disease
  • 2.1: systolic dysfunction
  • 2.2: diastolic dysfunction
  • 2.3: valvular disease
  • 2.4: congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
• group 3: pulmonary hypertension due to lung diseases and/or hypoxia
  • chronic obstructive pulmonary disease
  • interstitial lung disease
  • other pulmonary diseases with mixed restrictive and obstructive pattern
  • sleep-disordered breathing
  • alveolar hypoventilation disorders
  • chronic exposure to high altitude
  • developmental lung disease 
• group 4: pulmonary hypertension due to chronic pulmonary thromboembolism
• group 5: pulmonary hypertension due to unclear multifactorial mechanisms
  • hematologic disorders
    • chronic hemolytic anemia
    • myeloproliferative disorders
    • splenectomy
  • systemic disorders
    • sarcoidosis
    • pulmonary histiocytosis
    • lymphangioleiomyomatosis
  • metabolic disorders
    • glycogen-storage disease
    • Gaucher disease
    • thyroid disorders
  • other conditions
    • fibrosing mediastinitis
    • tumoral obstruction
    • chronic renal failure
    • segmental pulmonary hypertension