Pulmonary Hypertension

What Is Pulmonary Arterial Hypertension?

Pulmonary hypertension is a life-threatening condition that gets worse over time, but treatments can help your symptoms so you can live better with the disease. It may take some planning, but plenty of people who have it find ways to do all the things they love, just as they did before they were diagnosed.Having pulmonary arterial hypertension (PAH) means that you have high blood pressure in the arteries that go from your heart to your lungs . It's different from having regular high blood pressure.With PAH, the tiny arteries in your lungs become narrow or blocked. It's harder for blood to flow through them, and that raises the blood pressure in your lungs. Your heart has to work harder to pump blood through those arteries, and after a while the heart muscle gets weak. Eventually, it can lead to heart failure.

Causes

Sometimes doctors can't find a reason for high blood pressure in the lungs. In that case, the condition is called idiopathic pulmonary hypertension. Genes may play a role in why some people get it.In other cases, there is another condition that's causing the problem. Any of these illnesses can lead to high blood pressure in your lungs:

Congestive heart failure
Blood clots in the lungs
HIV
Illegal drug use (like cocaine or methamphetamine)
Liver disease (such as cirrhosis of the liver)
Lupus, scleroderma, rheumatoid arthritis, and other autoimmune diseases
A heart defect you're born with
Lung diseases like emphysema, chronic bronchitis, or pulmonary fibrosis
Sleep apnea

Pulmonary hypertension signs and symptoms include:

Blue lips and skin (cyanosis)
Chest pressure or pain.
Dizziness or fainting spells (syncope)
Fast pulse or pounding heartbeat (palpitations)
Fatigue.
Shortness of breath (dyspnea), initially while exercising and eventually while at rest.

Pulmonary hypertension signs and symptoms include:

Blue lips and skin (cyanosis)
Chest pressure or pain.
Dizziness or fainting spells (syncope)
Fast pulse or pounding heartbeat (palpitations)
Fatigue.
Shortness of breath (dyspnea), initially while exercising and eventually while at rest.

Symptoms

You may not notice any symptoms for a while. The main one is shortness of breath when you're active. It usually starts slowly and gets worse as time goes on. You may notice that you can't do some of the things you used to without getting winded. Other symptoms include:

Chest pain
Fatigue
Passing out
Swelling in your ankles and legs

Getting a Diagnosis

If you have shortness of breath and see your doctor, they will ask you about your medical history. They may also ask you:

Do you smoke?
Does anyone in your family have heart or lung disease?
When did your symptoms start?
What makes your symptoms better or worse?
Do your symptoms ever go away?

Your doctor may order tests, including:

Echocardiogram: This ultrasound picture of the beating heart can check blood pressure in the pulmonary arteries. CT scan: This can show enlarged pulmonary arteries. A CT scan can also spot other problems in the lungs that could cause shortness of breath.

Ventilation-perfusion scan (V/Q scan): This test can help find blood clots that can cause high blood pressure in the lungs.Electrocardiogram (EKG or ECG): An EKG traces the heart's activity and can show whether the right side of the heart is under strain. That's a warning sign of pulmonary hypertension. Chest X-ray: An X-ray can show if your arteries or heart are enlarged. Chest X-rays can help find other lung or heart conditions that may be causing the problems. Exercise testing: Your doctor may want you to run on a treadmill or ride a stationary bike while you are hooked up to a monitor, so they can see any changes in your oxygen levels, heart function, lung pressure, or other things. Your doctor may also do blood tests to check for HIV and conditions like rheumatoid arthritis or lupus. If these tests show that you might have pulmonary hypertension, your doctor will need to do a right heart catheterization to be sure. Here's what happens during that test:

The doctor places a catheter into a large vein, most often the jugular vein in your neck or femoral vein in your leg, and then threads it into the right side of your heart.
A monitor records the pressures in the right side of the heart and in the pulmonary arteries.
The doctor may also inject medicines into the catheter to see if the pulmonary arteries are stiff. This is called a vasoreactivity test.

Right heart catheterization is safe. The doctor will give you a sedative and use local anesthesia. You can usually go home the same day, although you will need someone to drive you home.