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Table 127-1

Classification of Pulmonary Hypertension

  1. Pulmonary Arterial Hypertension
    • Idiopathic
    • Heritable (mutations/familial cases)
    • Collagen vascular diseases (e.g., scleroderma, SLE, RA)
    • 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)

Abbreviations: RA, rheumatoid arthritis.