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Pulmonary Hypertension

Pulmonary hypertension is a rare and very serious blood vessel disorder of the lung. Primary pulmonary hypertension (PPH) — which is pulmonary hypertension that isn't a side effect of (or secondary to) another condition such as emphysema or lupus — strikes only between one and two people per million.

Managing this illness and its complications, including right heart failure, requires expertise and access to sophisticated treatment such as oral and continuous intravenous medication, and lung transplantation. Treatments for pulmonary hypertension can be highly complex and require intensive ongoing follow-up, all of which are available through our Pulmonary Hypertension Program.

Pulmonary hypertension is classified into five different types:

  • Pulmonary Arterial Hypertension (PAH) — This form affects blood vessels in the lungs that carry blood from the heart to the lungs where it picks up oxygen. This category is divided into two types:

    • Primary pulmonary hypertension (PPH) is very rare and can occur for no known reason. In about 10 percent of cases, it's inherited. It most often occurs in young adults and is more than twice as common in women as men.
    • PAH related to exposure to toxins including diet drugs such as fenphen; street drugs including cocaine and methamphetamine; HIV; collagen vascular diseases including scleroderma, lupus and rheumatoid arthritis; chronic liver disease; and congenital heart diseases.
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Pulmonary hypertension in its early stages is difficult to recognize on routine physical exam. It may be two or three years after onset that symptoms become severe enough to be noticed.

Pulmonary hypertension is diagnosed primarily with an echocardiogram, which is an ultrasound examination of the heart. The echocardiogram measures the heart's size and shape by using sound waves to create an image of the heart and can estimate the pulmonary artery pressure.

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The treatment for secondary pulmonary hypertension usually begins with treating the underlying cause. For instance, if a blood clot, or pulmonary embolism, is causing the hypertension, the clot may need to be surgically removed.

Several treatments are used to manage primary pulmonary hypertension, although they don't cure the condition. These include:

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Reviewed by health care specialists at UCSF Medical Center.

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UCSF Clinics & Centers

Heart & Vascular Center

Pulmonary Hypertension Clinic
400 Parnassus Ave., Plaza Level
San Francisco, CA 94143
Phone: (415) 353-9088
Fax: (415) 353-3889
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Heart Transplant Program
400 Parnassus Ave., Sixth Floor
San Francisco, CA 94143
Phone: (415) 353-4145
Fax: (415) 353-4166
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Lung Transplant Program
400 Parnassus Ave., Sixth Floor
San Francisco, CA 94143
Phone: (415) 353-4145
Fax: (415) 353-4166
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