Early on, pulmonary arterial hypertension (PAH) doesn’t typically cause symptoms. However, many people experience symptoms as the disease worsens. One common symptom is edema, or swelling.

It’s important for people with PAH to learn about swelling and understand what it means. Experiencing this symptom may signal that your condition is progressing or getting worse. Certain dietary changes and medications can prevent edema, reduce swelling, and improve your quality of life.

Read on for 10 facts to know about swelling and PAH.

1. Swelling With PAH Can Affect Different Parts of the Body

There are several possible ways edema can show up if you have PAH:

• Ascites (fluid buildup in the abdomen)
• Puffy skin in the face, arms, or legs (swollen legs are often because of the gravity effect)
• Skin that looks shiny or stretched out
• Skin that pits (creates a dimple when you press it with your finger)
• Weight gain

Left untreated, swelling could lead to more serious problems like pain, stiffness, walking difficulties, scarring, infection, or skin ulcers.

Sometimes, people with PAH wonder, “Is facial swelling a sign of heart problems?” While swelling may not be limited to your face, it can certainly show up there. Symptoms such as facial swelling and foot swelling may indicate that your doctor needs to take a look at your heart.

Many members of myPHteam, the social network for people with pulmonary hypertension, have discussed swelling. “Whenever I have swelling in my feet or lower legs, they start to itch,” wrote one member. “The itching is so intense that it drives me insane.”

“Whenever I have swelling in my feet or lower legs, they start to itch.”
— A myPHteam member

Another said, “Although I have a mild case of PAH, my symptoms at times are pretty bad. I battle frequently with my legs and feet swelling from retaining fluids.”

2. Swelling With PAH May Signal Heart Failure

Swelling may be a sign of PAH or another type of heart and lung disease. As the disease progresses, your heart may have a harder time pumping blood, which can affect levels of blood flow around the body.

At some point, the right ventricle (the chamber on the bottom right side of the heart) stops working correctly. The heart muscle can’t pump enough blood to the kidneys — the organs responsible for controlling fluid levels in your body. As a result, fluid builds up in different tissues and areas of the body.

Other problems that can develop due to swelling caused by heart failure include:

• Shortness of breath
• Difficulty moving around
• Skin infections
• Blood clots
• Damage to the kidneys, intestines, and liver (like liver enlargement)

“The PAH led to heart failure, and my heart doesn’t pump enough to handle all of the fluid,” explained one member of myPHteam. “This overload worked against my kidneys and liver, leading to both being damaged. It’s a domino effect.”

3. If You Have Swelling, Your Doctor Can Diagnose PAH With Several Tests

If you experience swelling, your doctor may perform a physical exam, ask about your family history and risk factors, and run blood tests to look for potential problems.

Some tests can help your doctor get a better look at your heart:

• An echocardiogram helps your doctor visualize how your heart works and see if it’s under too much strain.
• An electrocardiogram (ECG) shows your heart’s electrical activity.
• Right heart catheterization is a procedure that allows your doctor to directly measure blood pressure in your lungs’ blood vessels.

Other tests can help evaluate your lung function:

• Breathing tests check for asthma, chronic obstructive pulmonary disease (COPD), and other diseases that affect lung function.
• A 6-minute walk test is an exercise test that checks your oxygen levels during physical activity and measures how far you can walk.

These tests can also help a doctor diagnose any underlying conditions that may be causing the swelling.

There are several types of PAH, which is itself a type of pulmonary hypertension:

• Idiopathic PAH — PAH of unknown cause
• Heritable PAH — Caused by a family history of PAH
• Associated PAH — Caused by another condition like congenital heart or lung disease, blood clots, HIV, or connective tissue diseases such as scleroderma
• Drug-induced PAH — Caused by drug use (methamphetamines, for example)

PAH can be challenging to diagnose. You may need to work with a team of PAH specialists to get an accurate diagnosis and effective treatment plan.

4. Some Racial Groups Have a Higher Risk for PAH and Swelling

Fluid buildup is a potential PAH symptom for all groups of people, and it shouldn’t be ignored. However, this symptom may be especially important for certain racial groups — some research has found that PAH is more prevalent in African Americans and Hispanic people than in white people.

Researchers and doctors are increasingly recognizing that there are inequalities in healthcare, in general and for people with pulmonary hypertension, that make it harder for certain groups to get diagnosed and access treatment. As this issue becomes more widely understood, healthcare professionals are working to develop solutions that lessen these inequalities.

In the meantime, be aware that swelling can be a sign of a serious problem, especially for people who are more susceptible to PAH.

5. Certain Medications Make Swelling Worse

Some drugs lead to swelling as a side effect. Stopping these medications may help reduce or prevent fluid retention. Ask your doctor before starting or stopping any medications to make sure that it’s safe to do so.

Some drugs may cause swelling as a side effect. Stopping these medications may help reduce or prevent fluid retention.

Medications that may contribute to edema include:

• Calcium channel blockers (drugs for high blood pressure)
• Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen
• Corticosteroids
• Estrogens
• Thiazolidinediones (medications to treat diabetes)
• Certain IV drugs that contain high levels of salt

Some PAH treatment options may also cause swelling. Endothelin receptor antagonists — medications that widen blood vessels and keep PAH from getting worse too quickly — often cause fluid to build up. Other PAH treatments like phosphodiesterase-5 inhibitors or soluble guanylate cyclase stimulators are less likely to cause swelling.

6. Treating Underlying Conditions May Help With Swelling

In some cases, swelling is caused by other health conditions such as:

• Anemia (low levels of red blood cells)
• Kidney disease
• Liver disease
• Heart disease
• Chronic venous insufficiency (damaged blood vessels)
• Arrhythmia (irregular heartbeats)
• Blood clots in the legs
• Autoimmune disease (when your immune system attacks your body) or connective tissue disease

Managing these medical conditions may help reduce swelling. Since edema is a symptom of PAH, treating your PAH may also help reduce edema and other symptoms of pulmonary hypertension, such as chest pain, tiredness, or a fast heart rate.

7. Drinking Less Fluid Can Help Prevent or Reduce Swelling

If you’re experiencing edema, your body is not getting rid of extra fluid quickly enough. Taking in less fluid can help with this issue.

Health experts recommend that people with PAH and heart failure drink between 1.5 and 2 liters per day to prevent edema. This is about the same as 6.5 to 8 servings of fluids per day, where one serving is 1 cup or 8 ounces of liquid. If you have already been diagnosed with edema, you may need to further reduce your fluid intake, but always talk to your doctor about what is best for you.

Health experts recommend that people with PAH and heart failure drink between 1.5 and 2 liters of water per day to prevent edema.

“I am supposed to be limiting my fluid intake because I am in heart failure,” commented one member. “When you have been in the habit of ‘the more water the better,’ it is hard to break that cycle.”

Any liquids you drink, including water, coffee, tea, soda, and juice, count toward your daily total. Other items like ice, soup, and Jell-O should also be counted as fluids.

8. A Low-Salt Diet Can Help Treat Edema

The more salt (sodium) you eat, the more fluid your body holds on to. High-salt foods can worsen fluid buildup in your body.

Health experts say that people with PAH and heart failure should eat less salt. Some guidelines suggest getting less than 2 grams of sodium per day (less than 2,000 milligrams), although your doctor may make other recommendations.

Check the labels of the foods you eat to determine how much sodium is in one serving. To eat less salt, you may want to try these tips:

• Use less salt when cooking. For more flavor, add pepper, garlic, herbs, spices, or salt-free seasoning blends.
• Cook meals from scratch rather than going out to restaurants or eating processed or instant meals.
• Watch out for foods that can be surprisingly high in sodium, such as cheese, baking mixes, canned foods, and some desserts.

Members of myPHteam have also offered tips. “MrsDash.com has a meal-planning section for a no-salt diet,” suggested a member.

“I am going to meet with a dietitian next week,” said another.

9. Diuretics Help Your Body Get Rid of Extra Water

When diet changes don’t help, your doctor may recommend trying medications called diuretics or water pills. These drugs help your body get rid of extra water, so they are a common option for treating edema.

Loop diuretics such as furosemide, thiazide diuretics like metolazone, or potassium-sparing aldosterone antagonists like spironolactone may all be options.

Some myPHteam members report taking these medications for swelling. “I am on spironolactone to help rid my body of liquid, and I must say it works well,” one member commented. Another wrote, “The furosemide seems to be keeping the swelling at bay.”

When taking diuretics, watch for signs of dehydration, which can include:

• Thirst
• Dry skin
• Chapped lips
• Tiredness
• Dizziness
• Itchiness

Your doctor may want you to take a smaller dose if diuretics make you too dehydrated. The medications could also lead to other side effects like headaches, muscle cramps, or high blood sugar levels.

10. You Should Always Tell Your Doctor About Signs of Edema

Edema can be serious or even life-threatening, so it’s essential to address it right away. Tell your doctor if you gain a few pounds within a single day or if you gain weight a couple of days in a row.

“My cardiologist told me to weigh myself each day at the same time and keep a chart,” mentioned one member.

“Keep an eye on your swelling,” another member recommended. “That and other issues put me in the hospital for five days with congestive heart failure. Please be aware if the swelling worsens, call your pulmonologist.”

Talk With Others Who Understand

On myPHteam, more than 56,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Have you been struggling with swelling while living with PAH? What have you done to manage it? Share your experience in the comments below, or start a conversation by posting on your Activities page.