Pulmonary arterial hypertension (PAH) is a serious condition that affects the arteries in your lungs. Changes in these blood vessels can limit blood flow. And as blood flow slows, your oxygen levels drop. Simple tasks – like walking up stairs or carrying groceries – can leave you gasping for air.
But PAH is more than just high blood pressure in the lungs. It strains the heart and affects the whole body. Understanding how it works can help you spot symptoms early, manage complications, and work with your doctor on the best treatment plan.
What Is PAH?
PAH is a long-term condition that gets worse over time. It narrows the lung’s blood vessels, making your heart work extra hard to pump blood through a tight space. Over time, this weakens the right side of the heart, leading to fatigue, breathlessness, and eventually heart failure.
PAH vs. Pulmonary Hypertension
PAH is a specific type of pulmonary hypertension (PH), which is the general term for high blood pressure in the lungs. Heart problems, lung diseases, or blood clots are some of the causes. PAH can also happen with no cause.
How Common Is PAH?
PAH is rare, with only around 500 to 1,000 new cases diagnosed in the U.S. each year. That’s about one to two people per 1 million. Women, or people assigned female at birth, are three to five times more likely to get it than men. Hormones like estrogen probably play a role, but more research is needed.
What Causes PAH?
Something changes or damages the cells that line the lung’s blood vessels. Doctors don't fully understand this process, but certain things make it more likely.
Risk factors for PAH include:
- Being a woman between 30 and 60
- Genetic changes (PAH you inherit)
- Connective tissue disorders, like scleroderma
- Advanced liver disease (cirrhosis)
- Stimulant drugs, like methamphetamines
- Heart problems you’re born with
What Is Heritable PAH?
Around 15% to 20% of PAH cases run in families. The BMPR2 gene is the most common culprit. Other genes likely play a role, but scientists are still studying them. Not everyone with the gene gets PAH. This means something else, like environment or lifestyle, must be involved.
In about 20% of families with PAH, the disease clearly runs in the family, but the exact gene remains a mystery.
How PAH Affects Your Body
PAH raises pressure in your lungs, which puts extra strain on your heart, blood vessels, and other organs.
PAH and your blood vessels
Your pulmonary arteries carry blood away from the right side of your heart through your lungs. That’s where carbon dioxide is taken out and oxygen is put back in. It’s like a filtering system. Your lungs clean up the blood before sending it back through the left side of your heart to the rest of the body.
But with PAH, the blood vessels in your lungs get less flexible and tighten. Here’s what happens:
Vessel walls harden. When the arteries in your lungs can’t widen, this leaves less room for blood.
Pressure builds. Blood moves through a smaller space, raising pressure in your lungs.
Slower circulation. Less oxygen-rich blood gets to the rest of your body.
Think of it as if you're trying to drink a milkshake with a tiny straw. Your heart pushes harder, but the blood can’t move easily.
PAH and your heart
Your heart and lungs work together, but PAH throws them out of sync. The right side of your heart, which normally pumps blood at low pressure, now has to push against more force to get blood through your lungs.
How PAH overworks your heart:
Ventricle strain. The right side of the heart works overtime and stretches.
Left ventricle squeeze. The right side crowds the left side, making it smaller and weaker.
Less oxygen flow. A shrunken left ventricle can’t pump enough oxygen-rich blood to the lungs.
Arrhythmias. PAH can disrupt electrical signals, leading to dangerous irregular heartbeats.
Over time, this stress can lead to heart failure, meaning your heart muscle can’t keep up with your body’s needs.
PAH and your lungs
How PAH can impact the lungs:
Blood clots. PAH raises the risk of tiny clots forming in the lung arteries.
Bleeding in your lungs. You may cough up blood if the weakened, damaged vessels leak.
PAH and the rest of the body
PAH limits blood flow, setting off a chain reaction in your body. Some symptoms come and go – like when you're feeling breathless during activity but fine at rest – while others stick around or get worse.
Here’s what can happen:
Low oxygen (hypoxemia). Your blood doesn’t carry enough oxygen, making physical activity tough. Over time, muscles – including those that help you breathe – get weaker. Your lips or skin may turn blue. That’s called cyanosis.
Swollen veins (congestion). Blood backs up in your veins, triggering edema. That’s when fluid builds up in your legs, belly, and even your face.
Organ stress. Fluid buildup can strain other organs like your kidneys, liver, and spleen. Long-term damage can lead to liver disease.
Low blood count levels. PAH can lower platelet levels, making it harder for your blood to clot, especially if your liver and spleen are swollen.
Sleep problems. Oxygen levels may drop more at night. That’s called nocturnal hypoxemia. Many people with PAH also have sleep apnea, which makes it even harder to breathe.
Brain fog. Low oxygen may affect memory and focus. Research is ongoing in this area.
What Are the Symptoms of PAH?
At first, PAH symptoms might only show up with activity, like exercising. Eventually, everyday tasks like taking out the trash might wear you out. PAH can also look like other lung and heart conditions, such as asthma or chronic obstructive pulmonary disease (COPD). This makes it hard to know what’s going on.
Symptoms start mild but worsen over time. Here’s what to expect:
Early symptoms:
- Shortness of breath (especially with activity)
- Unusual tiredness
- Chest pain or tightness
- Swelling in your legs or ankles
Moderate symptoms:
- Fast heartbeat
- Pain in the upper right side of your belly (from liver stress)
- Not feeling hungry
- Feeling dizzy or fainting
- Serious weakness or fatigue
Advanced symptoms:
- Severe shortness of breath
- Coughing up blood
- Blue lips or nails
- Very fast heart rate and dizziness
- Loss of consciousness
PAH can be a medical emergency. It’s important to have a plan in case your symptoms suddenly get worse. Keep a kit with the following:
- Backup pump and catheter supplies
- Medical alert bracelet or necklace
- Extra oxygen mask
- Thermometer to check for infections
- Blood pressure monitor
- Phone numbers for doctors, family, and close friends
Make sure your family and friends know what to do if your condition takes a turn.
How Do You Treat PAH?
The right care plan can help you feel better and slow the disease. Most people take PAH-specific medications to improve blood flow, but other treatments may be needed based on your symptoms and overall health.
Doctors often prescribe:
- Vasodilators to widen blood vessels and make it easier for the heart to pump
- Endothelin receptor antagonists to block proteins that tighten blood vessels
- Soluble guanylate cyclase stimulators to boost blood flow
- Diuretics(water pills) to help flush out extra fluid and reduce swelling
- Blood thinners to lower the risk of clots
An activin signaling inhibitor is a newer drug that blocks the protein that fuels cell growth in the muscles around your blood vessels and the cells that line the inner wall. When cell and tissue growth slow down, the walls of the blood vessels may thin and loosen up. This can help blood flow and lower blood pressure in the lung arteries.
Your doctor may suggest oxygen therapy if your blood oxygen levels drop too low, especially at high altitudes or if you also have sleep apnea.
For serious cases, you may need a special surgery called an atrial septostomy to lower pressure on the right side of your heart. In advanced PAH, a lung or heart-lung transplant may be the only option when other treatments no longer work.
Living Well With PAH
PAH may limit some of your activities, but small changes can help you feel stronger and more in control. Healthy habits can boost your energy, ease symptoms, and lower your risk of other health problems.
These lifestyle changes may help:
Avoid smoke. If you smoke, stop. Try to stay away from secondhand smoke, too.
Stay active. Light exercise, like walking, is usually fine. Ask your doctor what’s safe.
Eat well. Focus on fruits, veggies, whole grains, and lean proteins. Cut back on salty foods.
Travel smart. Ask your doctor if you need extra oxygen when flying or visiting high altitudes.
Manage stress. Meditation, breathing exercises, and support groups can help.
You’ll also need to stay on top of your heart and lung health. Your doctor will help keep an eye on your PAH and manage related conditions, like COPD, heart disease, or sleep apnea.
Keeping up with routine visits ensures your treatment plan is working and helps catch any new concerns early. While you’re there, ask your doctor for more tips on how to manage symptoms and improve daily life.
Takeaways
Pulmonary arterial hypertension (PAH) is a serious condition that causes high blood pressure in the lungs. Over time, PAH strains your heart and other organs. Early treatment is key to slowing the disease and boosting your quality of life. If you notice symptoms like shortness of breath or unusual tiredness, talk to your doctor as soon as possible.
Pulmonary arterial hypertension FAQs
Can PAH be cured?
No, PAH can’t be cured. But treatment can help you live longer and feel better.
Can PAH be prevented?
There’s no surefire way to prevent PAH. But there are things you can do to protect your heart and lungs. Exercise as much as you can (within safety limits), don’t smoke, take your medication, and eat nutritious foods low in salt, sugar, and saturated fat.
Can I exercise if I have PAH?
Yes, but it’s best to talk to your doctor about what’s safe for you. Light exercises, such as walking or yoga, are usually fine. But things like heavy weightlifting might put too much strain on your heart and lungs. Ask your care team if pulmonary rehabilitation is right for you.
Can pregnancy make PAH worse?
Yes, pregnancy can be dangerous for people with PAH. That’s because carrying a baby strains your heart and lungs. If you have PAH and are pregnant or want to be, it’s important that you talk to your lung doctor about how to protect yourself and your baby.
Can PAH affect mental health?
Yes, living with a condition like PAH raises your chances of anxiety and depression. Consider joining a support group, meeting with a mental health professional, or practicing stress management techniques.
Show Sources
Photo Credit: E+/Getty Images
SOURCES:
National Organization for Rare Disorders (NORD): “Pulmonary Arterial Hypertension.”
Diseases: “Pulmonary Arterial Hypertension: Pathophysiology and Treatment.”
Pulmonary Hypertension Association: “Day-To-Day Living,” “Treatments,” “Diet and Nutrition,” “When Is It An Emergency?"
National Heart, Lung, and Blood Institute: “Pulmonary Hypertension.”
Mayo Clinic: “Pulmonary hypertension.”
Yale Medicine: “What if the Diagnosis is Pulmonary Hypertension?”
Frontiers in Cardiovascular Medicine: “Sex Differences, Estrogen Metabolism and Signaling in the Development of Pulmonary Arterial Hypertension.”
American Heart Association: “Pulmonary Hypertension – High Blood Pressure in the Heart-to-Lung System,” “Types of Heart Failure.”
Circulation: “Systemic Consequences of Pulmonary Hypertension and Right-Sided Heart Failure.”
Cleveland Clinic: “Pulmonary Arteries.”
American Lung Association: “Learn About Pulmonary Arterial Hypertension,” “Treating and Managing Pulmonary Arterial Hypertension.”
Circulation Research: “Pulmonary Arterial Hypertension: The Clinical Syndrome.”
Cell Metabolism: “The Metabolic Basis of Pulmonary Arterial Hypertension.”
Frontiers in Physiology: “Left-Ventricular Energetics in Pulmonary Arterial Hypertension-Induced Right-Ventricular Hypertrophic Failure.”
Frontiers in Medicine: “Arrhythmic Burden and Outcomes in Pulmonary Arterial Hypertension.”
Respiratory Research: “Cognitive, emotional, and quality of life outcomes in patients with pulmonary arterial hypertension.”
Advanced Emergency Nursing Journal: “Pulmonary Arterial Hypertension Emergency Complications and Evaluation.”
