Table 4

Abbreviated World Health Organization Clinical Classification of Pulmonary Hypertension

  1. Pulmonary arterial hypertension (PAH)

    • 1.1. Idiopathic (IPAH)

    • 1.2. Familial (FPAH)

    • 1.3. Associated with other underlying disease (including Gaucher disease, hemoglobinopathies, myeloproliferative disorders, splenectomy)

  2. Pulmonary hypertension with left heart disease

  3. Pulmonary hypertension associated with lung disease and/or hypoxia

  4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease

  5. Miscellaneous

 

  1. Pulmonary arterial hypertension (PAH)

    • 1.1. Idiopathic (IPAH)

    • 1.2. Familial (FPAH)

    • 1.3. Associated with other underlying disease (including Gaucher disease, hemoglobinopathies, myeloproliferative disorders, splenectomy)

  2. Pulmonary hypertension with left heart disease

  3. Pulmonary hypertension associated with lung disease and/or hypoxia

  4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease

  5. Miscellaneous

 

Adapted from Simmoneau et al54  with permission.

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