Pulmonary hypertension is a rare lung disorder in which the arteries that carry blood from the heart to the lungs become narrowed, making it difficult for blood to flow through the vessels. As a result, the blood pressure in these arteries, called pulmonary arteries, rises far above normal levels. This abnormally high pressure strains the right ventricle of the heart, causing it to expand in size. Overworked and enlarged, the right ventricle gradually becomes weaker and loses its ability to pump enough blood to the lungs. This could lead to the development of right heart failure.
Pulmonary hypertension occurs in individuals of all ages, races, and ethnic backgrounds, although it is much more common in young adults and is approximately twice as common in women as in men.
Scientists believe that the process starts with injury to the layer of cells that line the small blood vessels of the lungs. This injury, which occurs for unknown reasons, may cause changes in the way these cells interact with the smooth muscle cells in the vessel wall. As a result, the smooth muscle contracts more than normal and narrows the vessel.
Symptoms of pulmonary hypertension do not usually occur until the condition has progressed. The first symptom of pulmonary hypertension is usually shortness of breath with everyday activities, such as climbing stairs. Fatigue, dizziness, and fainting spells also can be symptoms. Swelling in the ankles, abdomen or legs, bluish lips and skin, and chest pain may occur as strain on the heart increases. Symptoms range in severity and a given patient may not have all of the symptoms.
In more advanced stages of the disease, even minimal activity will produce some of the symptoms. Additional symptoms include:
Eventually, it may become difficult to carry out any activities as the disease worsens.
The following are some known causes of pulmonary hypertension:
Pulmonary hypertension may also be caused by other conditions, and in some cases, the cause is unknown.
Because pulmonary hypertension may be caused by many medical conditions, a complete medical history, physical exam, and description of your symptoms are necessary to rule out other diseases and make the correct diagnosis. During the physical exam, your health care provider will:
Other tests that might be ordered include:
Appropriate diagnosis and analysis of the problem is necessary before starting any treatment. Treatment varies by patient, based on the different underlying causes, but generally includes:
Listed below are medication and surgical treatment approaches.
Many different types of medications are available to treat pulmonary hypertension. Treatment choices, such as those listed below, depend on how severe the pulmonary hypertension is, how likely it is to progress, and a patient’s drug tolerance.
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or healthcare provider. Please consult your healthcare provider for advice about a specific medical condition.
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