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Pulmonary Hypertension

Pulmonary hypertension (PHT) is high blood pressure within the arteries that supply the lungs. When those blood vessels constrict, pressure builds up and overworks the heart. This compromises circulation, and the lack of oxygen able to get to the lungs results in shortness of breath, fatigue and dizziness. The vessels often become scarred from the stress placed on them and they become stiffer and thicker, worsening the condition further.

PHT is a chronic condition that can affect people of all ages. As long as its underlying cause exists, patients will continue to experience PHT. If the underlying cause can be treated, through procedures such as a heart valve repair surgery, PHT can be reversed. There are also minimally-invasive heart surgeries performed at the Heart and Vascular Institute including:

There is no cure for PHT; however, there are many treatment options available to help patients live with and manage PHT for an otherwise active, healthy life, improving survival rates.

When to Call Your Doctor
Symptoms
Causes
Prevention
Diagnosis
Treatment

When to Call Your Doctor
If you experience any of the symptoms described below, particularly shortness of breath after light activity, contact your physician for further evaluation.

Symptoms
Symptoms do not often present themselves until PHT is in its advanced stages. PHT often mimics symptoms of many other conditions, making them difficult to distinguish. Some symptoms may include:

  • Shortness of breath following light exertion.
  • Constant fatigue.
  • Lightheadedness, especially when climbing stairs or upon standing up.
  • Swollen ankles, legs or abdomen.
  • Chest pain, especially during physical activity.
  • Fainting.

Symptoms are often worse at higher altitudes, during air travel or with pregnancy.

Causes
The left side of the heart pumps blood out of the lungs to the rest of your body. When blood pressure is measured through an arm cuff, it is the left side blood pressure that is being measured. When that pressure is abnormally high, it is called high blood pressure or hypertension.

The right side of the heart pumps blood to the lungs where the blood can pick up oxygen. When the lung (pulmonary) arteries and vessels register a high blood pressure, which requires diagnostic tests to be measured, it is called pulmonary hypertension.

While it is not always possible to determine the cause of PHT, it is most often caused by diseases of the heart and lungs, failure of the left heart ventricle, pulmonary embolism (or blood clots traveling from the legs or pelvic veins) or a disease like scleroderma. When the underlying cause is unknown, it is called secondary pulmonary hypertension. Primary PHT, usually inherited, occurs when the condition is independent of another underlying cause and is extremely rare with approximately 300 new cases diagnosed in the U.S. each year.

Prevention
There are no specific prevention measures that can be taken to reduce the risk of developing pulmonary hypertension. However, the risk of PHT stemming from heart disease can be reduced by living a heart-healthy lifestyle to promote cardiac wellness.

Diagnosis
If PHT is suspected based on your medical history, physical exam and symptoms, one or more of the following tests may assist your physician in confirming the diagnosis:

  • Chest X-ray. An X-ray will help assess lung condition.
  • Echocardiogram. An “echo” uses ultrasound waves to produce a moving picture of the heart and heart valves.
  • Electrocardiogram. An ECG or EKG records the electrical activity of the heart and shows abnormal rhythms (arrhythmias or dysrhythmias).
  • Catheterization. This test provides direct measurement of the pressure in the pulmonary arteries.
  • Exercise test. A brief walk on a treadmill helps determine exercise capacity.

Additional tests that identify the cause of diagnosed PHT include:

  • Blood tests, to rule out hepatitis, collagen disease, HIV or other conditions.
  • Ventilation-perfusion scan, used to rule out a pulmonary embolism or blood clot in the arteries to the lungs.
  • Tomography, a CT or CAT scan, to rule out abnormalities of the lung tissue.
  • Pulmonary function test, to rule out lung tissue disease, such as emphysema or pulmonary fibrosis.

Treatment
While there is no cure for pulmonary hypertension, treatment plans can help manage the condition and vary based on the underlying cause. Treatment options can include:

  • Lifestyle changes. Such changes include weight loss in obese patients, therapy to quit smoking, medications to lower high cholesterol, a program of monitored exercise and relaxation or breathing techniques.
  • Oxygen supplements. Portable oxygen is often used to increase blood oxygen levels.
  • Calcium channel blockers. These medications, such as nifedipine, verapamil, diltiazem and amlodipine, dilate (widen) the coronary arteries. They may also decrease the heart muscle’s demand for oxygen/blood.

Physicians who treat this condition

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