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`Types of Pulmonary Hypertension NHLBI, NIH
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`Types of Pulmonary Hypertension
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`The World Health Organization divides pulmonary hypertension (PH) into five groups. These groups are organized based on the cause of the condition.
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`In all groups, the average pressure in the pulmonary arteries is higher than 25 mmHg at rest or 30 mmHg during physical activity. The pressure in normal pulmonary
`arteries is 8–20 mmHg at rest.
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`(Note that group 1 is called pulmonary arterial hypertension (PAH) and groups 2 through 5 are called pulmonary hypertension. However, together all groups are called
`pulmonary hypertension.)
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`Group 1 Pulmonary Arterial Hypertension
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`Group 1 PAH includes:
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`PAH that has no known cause.
`PAH that's inherited (passed from parents to children through genes).
`PAH that's caused by drugs or toxins, such as street drugs and certain diet medicines.
`PAH that's caused by conditions such as:
`Connective tissue diseases. (Connective tissue helps support all parts of your body, including your skin, eyes, and heart.)
`HIV infection.
`Liver disease.
`Congenital heart disease. This is heart disease that's present at birth.
`Sickle cell disease.
`Schistosomiasis (SKIStoesoMIahsis). This is an infection caused by a parasite. Schistosomiasis is one of the most common causes of PAH in many parts of
`the world.
`PAH that's caused by conditions that affect the veins and small blood vessels of the lungs.
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`Group 2 Pulmonary Hypertension
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`Group 2 includes PH with left heart disease. Conditions that affect the left side of the heart, such as mitral valve disease or longterm high blood pressure, can cause
`left heart disease and PH. Left heart disease is likely the most common cause of PH.
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`Group 3 Pulmonary Hypertension
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`Group 3 includes PH associated with lung diseases, such as COPD (chronic obstructive pulmonary disease) and interstitial (INterSTISHal) lung diseases. Interstitial
`lung diseases cause scarring of the lung tissue.
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`Group 3 also includes PH associated with sleeprelated breathing disorders, such as sleep apnea.
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`Group 4 Pulmonary Hypertension
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`Group 4 includes PH caused by blood clots in the lungs or blood clotting disorders.
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`Group 5 Pulmonary Hypertension
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`Group 5 includes PH caused by various other diseases or conditions. Examples include:
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`Blood disorders, such as polycythemia vera (POLesiTHEmeah VAYrah or VErah) and essential thrombocythemia (THROMbosiTHEmeah).
`Systemic disorders, such as sarcoidosis (sarkoyDOsis) and vasculitis (vaskyuLItis). Systemic disorders involve many of the body's organs.
`Metabolic disorders, such as thyroid disease and glycogen storage disease. (In glycogen storage disease, the body's cells don't use a form of glucose (sugar)
`properly.)
`Other conditions, such as tumors that press on the pulmonary arteries and kidney disease.
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`Updated: August 2, 2011
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`https://www.nhlbi.nih.gov/health/healthtopics/topics/pah/types
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`WATSON LABORATORIES, INC. , IPR2017-01622, Ex. 1122 , p. 1 of 1
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