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Pulmonary Arterial Hypertension

(Primary Pulmonary Hypertension)



PAH/PPH Symptoms & Diagnosis

Symptoms Of Primary Arteriak Hypertension

The most common symptoms of primary pulmonary hypertension or pulmonary arterial hypertension are related to difficulty with breathing. They include:

Progressive shortness of breath (especially with activity)
Hyperventilation (breathing harder and faster)
Fatigue (tiring easily)
Progressive weakness
Fainting spells
Lightheadedness or dizziness
Coughing up blood
Progressive cyanosis (blue tinge to lips hands and feet)

These symptoms may occur at presentation, or progress over a period of time. A patient with primary pulmonary hypertension must be monitored for these symptoms by routine visits to their cardiologist or pulmonologist. If any new symptoms develop, patients should notify their doctor immediately.

A physical examination of patients with primary arterial hypertension may show:

Distension of veins in the neck
Swelling of the legs and hands due to fluid retention
Enlarged liver

All of these signs are related to the right ventricle working harder to pump blood into the high resistance of the lungs' blood vessels. (Click to see Pictures of PAH/PPH)

Primary Arterial Hypertension Diagnosis

PAH/PPH is rarely picked up in a routine medical examination. Even in its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs. Often too much time can pass between the time the symptoms of PAH appear and a definite diagnosis is made.

PAH/PPH remains a diagnosis of exclusion. This means that it is diagnosed only after the doctor finds pulmonary hypertension and excludes or cannot find other reasons for the hypertension, such as a chronic obstructive pulmonary disease (chronic bronchitis and emphysema), blood clots in the lung (pulmonary thromboemboli), or some forms of congenital heart disease.

The first tests for PPH or pulmonary arterial hypertension help the doctor determine how well the heart and lungs are performing. If the results of these tests do not give the doctor enough information, the doctor must perform a cardiac catheterization. The procedure, discussed below, is the way the doctor can make certain that the patient's problems are due to PAH/PPH and not to some other condition.

The tests are:

An electrocardiogram should be performed which will show enlargement of the right side of the heart.

A chest X-ray may show enlargement of the size of the heart, and is often a good way to follow the patient's heart size over time.

An echocardiogram, or ultrasound of the heart, will be performed initially and can also be used to follow the progression of the disease. An echo can show enlargement of the right ventricle and can sometimes estimate the pressures of the right ventricle and pulmonary arteries.

Cardiac Catheterization. The gold standard for making the diagnosis of primary pulmonary hypertension is a cardiac catheterization. This will actually measure the pressures on the right side of the heart and calculations can be made to measure the resistance of the blood vessels in the lungs. Various drugs may be used during a cardiac catheterization to determine if the blood vessels in the lungs will relax in response to them. This may lower the pressures in the lungs and can help guide therapy.

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