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. 2008 Jul 31;2(4):225–231.

Table 2.

Clinical classification of pulmonary arterial hypertension (adapted from Galie 2004)

  1. Pulmonary arterial hypertension
    1. Idiopathic
    2. Familial
    3. Associated with:
      1. Connective tissue disease
      2. Congenital systemic to pulmonary shunts
      3. Portal hypertension
      4. HIV infection
      5. Drugs and toxins
      6. Other systemic disorders
  2. Pulmonary hypertension associated with left-sided heart diseases
    1. Left-sided atrial or ventricular heart disease
    2. Left-sided valvular heart disease
  3. Pulmonary hypertension associated with lung respiratory diseases and/or hypoxia
    1. Chronic obstructive lung disease
    2. Interstitial lung disease
    3. Sleep disordered breathing
    4. Alveolar hypoventilation disorders
    5. Chronic exposure to high altitude
    6. Developmental abnormalities
  4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease
    1. Thromboembolic obstruction of proximal pulmonary arteries
    2. Thromboembolic obstruction of distal pulmonary arteries
    3. Nonthrombotic pulmonary embolism (tumor, parasites, foreign material)
  5. Miscellaneous
    1. Sarcoidosis, histiocytosis X, lymphangioma

HIV, human immunodeficiency virus.