Pulmonary Hypertension (cont.)

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What are primary and secondary pulmonary hypertension?

In the conventional classification, pulmonary hypertension, which is also called pulmonary arterial hypertension, is divided into two main categories; 1) primary pulmonary hypertension (not caused by any other disease or condition); and 2) secondary pulmonary hypertension (caused by another underlying condition). Secondary pulmonary hypertension is much more common than primary pulmonary hypertension.

A newer classification of this condition is based on the main underlying cause of pulmonary hypertension. This system classifies the condition based on whether it is due to:

  • Pulmonary hypertension from a variety of causes, some known and others unknown, such as:
    • drug-induced pulmonary artery hypertension,
    • pulmonary artery hypertension associated with collagen vascular diseases, HIV, and schistosomiasis (a parasitic infection), and
    • pulmonary arterial hypertension of unknown cause (idiopathic pulmonary arterial hypertension)
  • Pulmonary arterial hypertension from left-sided heart disease, often referred to as pulmonary venous hypertension, including thickening of the heart muscle, decrease heart pump function, and disorders of the heart valves
  • Pulmonary arterial hypertension associated with lung disease and or persistent drop in oxygen levels (hypoxia) including:
  • Pulmonary arterial hypertension from blood clotting disorders that deliver clots to the lung (pulmonary emboli) or are formed directly in the lung arteries (pulmonary thrombosis), also known as chronic thromboembolic pulmonary hypertension;
  • Pulmonary arterial hypertension from miscellaneous causes with unclear multifactorial mechanisms; including diseases such as:
    • sarcoidosis,
    • tumor obstruction,
    • metabolic disorders (glycogen storage disease), and
    • liver failure; so termed portopulmonary hypertension
Medically Reviewed by a Doctor on 2/19/2015

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