Pulmonary 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.

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

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

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.

Treatment #

• Pulmonary hypertension varies from person to person, so your treatment plan will be specific to your needs. Ask your doctor what your options are and what to expect.

• First, your doctor will treat the cause of your condition. For example, if emphysema is causing the problem, you’ll need to treat that to improve your pulmonary hypertension.

• Most people also get treatment to improve their breathing, which makes it easier to be active and do daily tasks.

• Oxygen therapy, when you breathe pure oxygen through prongs that fit in your nose, will help if you’re short of breath and have low oxygen levels in your blood.

• It helps you live longer when you have pulmonary hypertension.

• If you are at risk for blood clots your doctor will recommend blood thinners.

• Other medicines improve how well your heart works and keep fluid from building up in your body.

• If you have severe pulmonary hypertension, your doctor may prescribe medications called calcium channel blockers. These medicines lower blood pressure in the lungs and the rest of the body.

• If calcium channel blockers aren’t enough, your doctor may refer you to a specialized treatment center.

• You may need more targeted therapies that can open up your narrowed blood vessels.

• They may be pills, medicines you breathe in, or drugs that are given through an IV. Options include:

• Pills: ambrisentan (Letairis), bocentan (Tracleer), macitentan (Opsumit), riociguat (Adempas), selexipag (Uptravi), sildenafil (Revatio), tadalafil (Adcirca), treprostinil (Orenitram)

• Inhalers: Iloprost tromethamine (Ventavis), treprostinil (Tyvaso)

• IV drugs: epoprostenol sodium (Flolan, Veletri), treprostinil

In more severe cases, or if medicines don’t help, your doctor may recommend a lung transplant or a procedure called atrial septostomy. A surgeon creates an opening between the right and left sides of the heart. This surgery can have serious side effects.