Pulmonary Hypertension

  • Medical Author:
    George Schiffman, MD, FCCP

    Dr. Schiffman received his B.S. degree with High Honors in biology from Hobart College in 1976. He then moved to Chicago where he studied biochemistry at the University of Illinois, Chicago Circle. He attended Rush Medical College where he received his M.D. degree in 1982 and was elected to the Alpha Omega Alpha Medical Honor Society. He completed his Internal Medicine internship and residency at the University of California, Irvine.

  • Medical Editor: William C. Shiel Jr., MD, FACP, FACR
    William C. Shiel Jr., MD, FACP, FACR

    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

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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