Pulmonary Hypertension

Pulmonary hypertension (PH) means you have high blood pressure in your pulmonary arteries, which carry blood from your heart to your lungs. It has many possible causes, but it’s usually a complication of heart disease or lung disease.

Over time, the raised pressure damages your pulmonary arteries and narrows the space where blood can flow. This means your right ventricle (lower right heart chamber) must work harder to pump blood through those arteries so it can reach your lungs. This extra work makes your right ventricle get bigger than it should be (right ventricular hypertrophy), which may lead to right-sided heart failure. Without treatment, PH can be fatal.

Types of this condition

The World Health Organization (WHO) divides pulmonary hypertension into five groups based on its cause:

• Group 1: Tissue damage in your pulmonary arteries (this type is commonly called pulmonary arterial hypertension, or PAH)
• Group 2: Problems with heart function — specifically on the left side
• Group 3: Problems with lung function
• Group 4: Blockages in your pulmonary arteries (when blood clots are to blame, it’s called chronic thromboembolic pulmonary hypertension, or CTEPH)
• Group 5: Underlying conditions that are linked to PH in ways experts don’t fully understand

Groups 2 and 3 are the most common. Group 1 (PAH) is rare, but people often confuse it with PH overall. That’s why you might hear that pulmonary hypertension is rare. It’s not. Experts estimate that PH affects about 1 in 100 people around the world. It’s most common among people over age 65. But it can affect anyone, including newborns, children and younger adults.

Symptoms of pulmonary hypertension

PH symptoms typically start off mild and get worse over time. No matter which type of PH you have, you might experience:

• Chest pain or discomfort
• Dizziness or fainting
• Fatigue
• Loss of appetite
• Shortness of breath
• Swelling in your feet, ankles, legs or belly

What are the stages of pulmonary hypertension?

There are four main stages or “functional classes.” They’re based on your symptoms and ability to carry out your daily tasks. As PH gets worse, the symptoms become more obvious and disruptive.

• Class 1: You don’t have symptoms.
• Class 2: You feel some symptoms from ordinary activity like cleaning your house or shopping. But you feel fine when you’re resting.
• Class 3: It’s much harder to do normal tasks because you feel very tired or short of breath. But you still feel fine when resting.
• Class 4: You have symptoms even when you’re resting. The symptoms get worse when you try to move around at all.

Pulmonary hypertension causes

There are five main types of PH — and each type has its own unique set of causes. Overall, heart and lung diseases are the most common ones. But you can also develop PH from other medical conditions or certain drugs or toxins. The exact cause matters because it determines your treatment plan. So, your healthcare provider will want to find out what it is.

Here are some lists of possible causes by PH type.

Group 1 (PAH):

• Drugs and toxins like cocaine and methamphetamine
• Congenital heart disease
• Connective tissue disease
• Gene changes you can inherit
• HIV
• Portal hypertension
• Schistosomiasis
• Unknown causes (idiopathic)

Group 2 (left-sided heart disease):

• Aortic valve disease
• Left-sided heart failure
• Mitral valve disease

Group 3 (lung diseases or hypoxia):

• Chronic obstructive lung disease (COPD)
• Restrictive lung disease
• Low oxygen levels (hypoxia) without lung disease — for example, being at high altitudes
• Respiratory depression

Group 4 (blockages in your lungs):

• Scars from prior blood clots in your lungs (pulmonary embolism)
• Tumors that block pulmonary arteries
• Pulmonary artery stenosis that’s present at birth

Group 5 (conditions that cause PH in complex or unclear ways):

• Chronic kidney disease
• Fibrosing mediastinitis
• Gaucher disease
• Glycogen storage disease
• Neurofibromatosis type 1
• Pulmonary Langerhans cell histiocytosis
• Sarcoidosis
• Sickle cell disease

Complications of this condition

Pulmonary hypertension can cause serious problems in your body, including:

• Anemia
• Abnormal heart rhythms
• Blood clots in your pulmonary arteries
• Pericardial effusion
• Pregnancy complications (PH is dangerous for both you and the fetus)
• Right-sided heart failure

Untreated PH is life-threatening. It’s important to get a diagnosis and start treatment as early as possible.

How doctors diagnose high blood pressure in your lungs

Healthcare providers diagnose pulmonary hypertension by doing a physical exam and running some tests. During the exam, your provider will check for signs of PH and other heart or lung issues by:

• Measuring your vital signs, including your blood pressure and blood oxygen level
• Listening to your heart and lungs with a stethoscope
• Checking for bulging neck veins, which can be a sign of heart failure
• Checking your belly, legs and ankles for swelling

PH is difficult to diagnose. It shares signs with many other conditions. So, after your exam, your provider may do tests to learn more. You may need to see a lung or heart doctor for other tests.

Tests you may need to diagnose PH and its cause include:

• Blood tests to check for issues related to organ function, hormone levels and infections
• Chest CT scan to look for blood clots and other lung conditions that may cause PH or make it worse
• Chest X-ray to show if your right ventricle or pulmonary arteries are enlarged (common signs of PH)
• Doppler echocardiogram to show your heart’s pumping action and measure blood flow through your valves
• Right heart catheterization to measure the pressure inside your pulmonary arteries and check how much blood your heart can pump per minute (this test confirms a PH diagnosis)
• Six-minute walk test to see how much exercise you can handle and how much oxygen is in your blood as you move
• VQ scan to look for blood clots in your lungs

How is pulmonary hypertension treated?

Most cases of pulmonary hypertension can’t be cured. But treatment can help ease your symptoms, improve your quality of life and slow disease progression. You may need medications, procedures or surgeries to treat PH directly or target its cause. The exact treatments you’ll receive vary by PH type:

• Group 1: Your provider may prescribe medications called pulmonary vasodilators. These help your lung arteries relax and open up better. This improves blood flow and lowers the strain on your heart. You may also need oxygen therapy.
• Groups 2 and 3: Your provider will treat the heart or lung issue that’s causing PH. This may involve things like medications, dietary changes, oxygen therapy, procedures or surgeries. 
• Group 4: The goal of treatment is to remove blockages in your pulmonary arteries. This may involve a procedure called balloon pulmonary angioplasty to widen your arteries. Or you may need surgery, called pulmonary endarterectomy, to remove blood clots and scarring.
• Group 5: Treatment depends on the condition that’s causing your PH. Your provider will work with you to find the best care plan.

A lung transplant is a last resort for some people with pulmonary hypertension.

Pulmonary vasodilators are often a first-line treatment for people with PAH (group 1) and CTEPH (group 4). But they’re currently not FDA-approved for other groups. Researchers continue to look into medications that can help more people with PH.

When should I see my healthcare provider?

Call your provider if you have:

• A fast heart rate (120 beats per minute)
• A respiratory infection or cough that’s getting worse
• Dizziness that keeps happening
• Episodes of chest pain or discomfort with physical activity
• Extreme fatigue or trouble doing your normal activities
• Nausea or lack of appetite
• Restlessness or confusion
• Shortness of breath that’s gotten worse, especially if you feel it when waking up 
• Swelling in your ankles, legs or belly that’s gotten worse
• Weight gain (2 pounds in one day or 5 pounds in one week)

Call 911 or your local emergency number if you experience:

• A fast heart rate that won’t go down
• Fainting or blacking out
• Issues with a catheter you’re using to take your medicine — like the catheter moving out of place, solution leaking or the pump not working right
• Shortness of breath that doesn’t go away when you rest
• Sudden and severe chest pain or headache
• Sudden weakness or paralysis in your arms or legs

What can I expect if I have this condition?

Pulmonary hypertension is a progressive disease. That means it gets worse over time. It does this more quickly in some people than in others. Treatment can improve your chances of surviving for many years. Without treatment, PH leads to right-sided heart failure and is ultimately fatal.

Because PH can happen for so many reasons, the outlook is different from person to person. In general, your outlook depends on the type of PH you have, how early it’s diagnosed and other health conditions you have. Talk with your provider to learn more about what you can expect and how to manage your condition.

Is there anything I can do to feel better?

There’s a lot you can do to manage PH, take care of yourself and lower your risk of complications. Talk with your provider about:

• Changes to what you eat: It’s important to reduce your salt intake. This means not adding salt and avoiding things like cured meats, smoked meats and fast food. Your provider may also recommend eating foods high in fiber, potassium and magnesium.
• Creating an emergency kit: When you’re living with PH, you need certain supplies and information with you at all times. Ask your provider what you should include in your kit.
• Exercise: Some types of exercise may help you. Others may be unsafe. Learn what’s best in your situation.
• Medication safety: You may have many medicines to take for PH and/or other health conditions. These can interact with some over-the-counter drugs and supplements. Always ask your provider before taking anything new. Also, ask what to do if you miss a dose.
• Pregnancy: PH makes pregnancy dangerous. Talk to your provider about the risks and your birth control options.
• Seasonal vaccines: Your provider may recommend vaccines against flu, pneumonia and other viruses that could make you very sick.
• Support groups or counseling: Adjusting to life with PH can be hard. You don’t have to carry the worries or anxiety alone.
• Travel: You may need to take certain precautions when flying or visiting places at high altitudes. Planning ahead can help you travel safely.

What is pulmonary venous hypertension?

This means the pressure in your pulmonary veins is too high. Despite its name, pulmonary venous hypertension isn’t a “type” of pulmonary hypertension (PH). Instead, it’s a common cause of PH — specifically, group 2 PH due to left-sided heart disease.

Your pulmonary veins carry blood from your lungs to the left side of your heart. But if there are problems on the left side of your heart, like heart failure or valve disease, blood can’t move through as easily. This causes blood to back up in your pulmonary veins as it waits its turn to enter your heart.

Over time, this can slow down the whole system of blood moving between your heart and lungs. Eventually, blood leaving your heart through your pulmonary arteries has to push harder to get through. This leads to raised pressure in those arteries and a PH diagnosis.