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Table 129-1

Classification of Pulmonary Hypertension

  1. Pulmonary Arterial Hypertension

    Idiopathic

    Heritable (mutations/familial cases)

    Collagen vascular diseases (e.g., scleroderma, SLE, rheumatoid arthritis)

    Congenital systemic to pulmonary shunts (e.g., ventricular septal defect, patent ductus arteriosus, atrial septal defect)

    Portal hypertension

    HIV infection

    Drugs or toxins (e.g., fenfluramine)

  2. Pulmonary Hypertension Due to Left Heart Disease

    LV systolic or diastolic dysfunction

    Left-sided valvular disease

  3. Pulmonary Hypertension Due to Lung Disease or Hypoxia

    Chronic obstructive lung disease

    Interstitial lung disease

    Sleep-disordered breathing

    Chronic hypoventilation

  4. Chronic Pulmonary Thromboembolic Disease

    Chronic pulmonary embolism

  5. Miscellaneous

    Systemic conditions (e.g., sarcoidosis, pulmonary histiocytosis)

    Hematologic conditions (e.g., myeloproliferative diseases)