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Pulmonary Hypertension (cont.)

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What are primary (idiopathic) and secondary pulmonary hypertension?

The conventional classification, primary or secondary pulmonary hypertension, which also is called pulmonary arterial hypertension, has been reclassified and is now is based on the main underlying cause, characteristics, and treatments of pulmonary hypertension. These terms were updated in 2013.This system classifies the condition based on five classes.

Class 1 includes:

  • Pulmonary arterial hypertension including idiopathic previously referred to as primary pulmonary arterial hypertension), inherited pulmonary arterial hypertension, drug-induced pulmonary arterial hypertension
  • Pulmonary arterial hypertension associated with the following:
  • Connective tissue diseases like systemic lupus erythematosus, or scleroderma
  • HIV
  • Liver disease (elevated pressures in the liver, portal hypertension);
  • Congenital heart disease
  • Schistosomiasis (a parasite infection that can clog the pulmonary arteries)
  • Pulmonary Veno-occlusive disease (very rare)
  • Persistent pulmonary hypertension of the newborn

Class 2 includes:

Class 4 includes:

  • Chronic thromboembolic pulmonary hypertension (chronic blood clots to the lungs that don't dissolve).

Class 5 includes:

  • Pulmonary hypertension with unclear multifactorial mechanisms, for example,
    • blood disorders (including hemolytic anemias),
    • systemic disorders(such as sarcoid),
    • metabolic disorders where cellular chemistry is abnormal (such as glycogen storage diseases),
    • tumors obstructing flow through the lungs.
Medically Reviewed by a Doctor on 3/30/2016


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