Primary Pulmonary Hypertension

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. Thus, much time can pass between the time the symptoms of PPH appear and a definite diagnosis is made.

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 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 PPH and not to some other condition.

• Electrocardiogram: The electrocardiogram (ECG) is a record of the electrical activity produced by the heart. An abnormal ECG may indicate that the heart is undergoing unusual stress.

In addition to the usual ECG performed while the patient is at rest, the doctor may order an exercise ECG. This ECG helps the doctor evaluate the performance of the heart during exercise, for example, walking a treadmill in the doctor's office.

• Echocardiogram: In an echocardiogram, the doctor uses sound waves to map the structure of the heart by placing a slim device that looks like a microphone on the patient's chest. The instrument sends sound waves into the heart, which then are reflected back to form a moving image of the beating heart's structure on a TV screen. A record is made on paper or videotape. The moving pictures show how well the heart is functioning. The still pictures permit the doctor to measure the size of the heart and the thickness of the heart muscle; in the patient with severe pulmonary hypertension, the still pictures will show that the right heart is enlarged, while the left heart is either normal or reduced in size. Echocardiograms are helpful in excluding some other causes of pulmonary hypertension and can be useful in monitoring the response to treatment.

• Pulmonary Function Tests: A variety of tests called pulmonary function tests (PFTs) evaluate lung function. In these procedures, the patient, with a nose clip in place, breathes in and out through a mouthpiece. The patient's breathing displaces the air held in a container suspended in water. As the container rises and falls in response to the patient's breathing, the movements produce a record, or spirogram, that helps the doctor measure lung volume (how much air the lungs hold) and the air flow in and out of the lungs. Some devices measure air flow electronically.

A mild restriction in air movement is commonly seen in patients with PPH. This restriction is thought to be due, in part, to the increased stiffness of the lungs resulting from both the changes in the structure and the high blood pressure in the pulmonary arteries.

• Perfusion Lung Scan: A perfusion lung scan shows the pattern of blood flow in the lungs; it can also tell the doctor whether a patient has large blood clots in the lungs. In the perfusion scan, the doctor injects a radioactive substance into a vein. Immediately after the injection, the chest is scanned for radioactivity. Areas in the lung where blood clots are blocking the flow of blood will show up as blank or clear areas.

Two patterns of pulmonary perfusion are seen in patients with PPH. One is a normal pattern of blood distribution; the other shows a scattering of patchy abnormalities in blood flow.

A major reason for doing a perfusion scan is to distinguish patients with PPH from those whose pulmonary hypertension is due to blood clots in the lungs.

• Right-Heart Cardiac Catheterization: In right-heart cardiac catheterization, the doctor places a thin, flexible tube, or catheter, through an arm, leg, or neck vein in the patient, and then threads the catheter into the right ventricle and pulmonary artery. Most important in terms of PPH is the ability of the doctor to get a precise measure of the blood pressure in the right side of the heart and the pulmonary artery with this procedure. It is the only way to get this measure, and must be performed in the hospital by a specialist.

During catheterization, the doctor can also evaluate the right heart's pumping ability; this is done by measuring the amount of blood pumped out of the right side of the heart with each heartbeat.

• Functional Classification: Once PPH is diagnosed, most doctors will classify the disease according to the functional classification system developed by the New York Heart Association. It is based on patient reports of how much activity they can comfortably undertake.

Class 1 — Patients with no symptoms of any kind, and for whom ordinary physical activity does not cause fatigue, palpitation, dyspnea, or anginal pain.

Class 2 — Patients who are comfortable at rest but have symptoms with ordinary physical activity.

Class 3 — Patients who are comfortable at rest but have symptoms with less-than-ordinary effort.

Class 4 — Patients who have symptoms at rest.

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