What are the WHO classifications of pulmonary hypertension leading to cor pulmonale?

Updated: Dec 15, 2017
  • Author: Derek Leong, MD; Chief Editor: Henry H Ooi, MD, MRCPI  more...
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Several different pathophysiologic mechanisms can lead to pulmonary hypertension and, subsequently, to cor pulmonale. The World Health Organization (WHO) has five classifications for pulmonary hypertension, and all except one of these groups can result in cor pulmonale (WHO Classification group 2 is pulmonary artery hypertension due to left ventricular [LV] dysfunction). [4] Note the following WHO classifications:

  • Group 1: Pulmonary artery hypertension, including heritable causes; connective-tissue disorders, including scleroderma; and other idiopathic causes

  • Group 3: Pulmonary hypertension due to lung disease and/or hypoxia; these disorders include chronic obstructive pulmonary disease (COPD), which is the most common cause of for pulmonale. There have been studies correlating the degree of hypoxia with the severity of cor pulmonale. Other disorders that can result in cor pulmonale in this group include interstitial lung disease (ILD) and obstructive sleep apnea (OSA)

  • Group 4: Chronic thromboembolic pulmonary hypertension; blood clots that form in the lungs can lead to increased resistance, pulmonary hypertension and, subsequently, cor pulmonale

  • Group 5: Pulmonary hypertension caused by other diseases or conditions, including sarcoidosis, polycythemia vera (which can lead to increased blood viscosity and, subsequently, pulmonary hypertension), vasculitis, and other disorders.

The end result of the above mechanisms is increased pulmonary arterial pressure and resistance.

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