Pulmonary hypertension is a progressive lung disorder associated with high pressure in the blood vessels that supply blood to the lungs or pulmonary arteries.
Generally, doctors need to perform multiple tests to diagnose pulmonary hypertension and determine the type of a person has — because the type can have a significant effect on what treatment is required. Late or incorrect diagnosis of pulmonary hypertension can lead to a far worse disease outcome.
Physical exam and medical history
Diagnosis generally begins with a physical exam to check a patient’s symptoms. This includes looking for leg and ankle swelling and listening to the heart and lungs.
The doctor will also review the medical history of both patient and family. Some rare types of pulmonary hypertension can be inherited. This means that if a family member has had it, the person seeking a diagnosis has a good chance of having it as well. Other conditions and some medications can also cause pulmonary hypertension. Spotting them in a patient’s medical records should be a red flag that alerts a doctor to the possibility of pulmonary hypertension.
Tests for pulmonary hypertension
If a doctor suspects pulmonary hypertension, the patient may be asked to take various tests. They include:
- Echocardiography uses sounds waves to produce a moving image of the heart. Doctors can use it to estimate the pressure in the pulmonary arteries and check how well the heart is pumping blood.
- Chest X-rays produce an image of the heart, lungs and blood vessels. Doctors use them to check for an enlarged heart, which is common in advanced satges of pulmonary hypertension, or lung scarring, which may be an alternate cause of shortness of breath.
- Electrocardiography or EKG measures the electrical activity of the heart. It can be used to diagnose an irregular heartbeat.
- Right heart catheterization is used to measure blood flow in the pulmonary arteries and heart and determine blood pressure.
Tests for the cause of pulmonary hypertension
Once pulmonary hypertension is diagnosed, doctors must determine the class of the disease so they can treat it correctly. The World Health Organization lists five classes of pulmonary hypertension. Each is associated with different causes of the disease. Certain tests can help determine, or exclude, certain causes of pulmonary hypertension. They are:
- A chest computerized tomography (CT) scan can produce detailed images of the structures of the heart and lungs. This can help identify blood clots in the arteries, and assess the size and functioning of the heart.
- Chest magnetic resonance imaging (MRI) can provide information on the blood flow in the lungs and how the heart is functioning.
- Lung function tests can assess the volume and speed of air flow through the lungs, which may indicate that an underlying lung disease is causing pulmonary hypertension. These tests can also detect how efficiently oxygen is being transferred from the lungs to the blood. Pulmonary hypertension patients commonly have less oxygen in their blood.
- A polysomnogram can help assess a number of measures, including blood pressure, heart rate, and oxygen level when sleeping. Low oxygen levels during sleep are common in pulmonary hypertension. Doctors can also use this measure to determine if a person has sleep apnea.
- A ventilation/perfusion (V/Q) scan measures the air and blood flow through the lungs to see if blood clots are causing pulmonary hypertension.
- Blood tests are used to check for conditions such as HIV, or thyroid, liver and autoimmune diseases that could be associated with pulmonary hypertension.
- Genetic tests can help identify mutations associated with pulmonary hypertension that runs in families.
Diagnosing the severity of pulmonary hypertension
The severity of pulmonary hypertension is normally diagnosed by determining the patient’s exercise ability. This is generally done in one of two ways. A cardiopulmonary exercise test can measure how well the lungs and heart work while a patient is on a treadmill or bicycle. The six-minute walk test measures how far a patient can walk in six minutes.
Doctors use the results of the tests to rank the severity of the disease from class 1 to 4:
- Class 1 patients have no symptoms with normal activity.
- Class 2 patients experience symptoms with regular physical activity.
- Class 3 patients experience symptoms with slight physical activity.
- Class 4 patients experience symptoms while resting, and severe symptoms with physical activity.
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