Pulmonary Hypertension: Complete Guide to Symptoms, Causes, Diagnosis, and Treatment
Updated: 4 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
TL;DR
Pulmonary hypertension is high blood pressure in the arteries of the lungs that forces the right side of the heart to work harder, and over time this can weaken and fail the heart. It develops slowly, often over months or years, with symptoms like shortness of breath, fatigue, and swelling. It is sorted into five cause groups, most commonly left-sided heart disease. There is no cure, but medications, oxygen therapy, and in some cases surgery or transplant can improve symptoms and extend life.
Quick Answer
Pulmonary hypertension is a type of high blood pressure affecting the lung arteries and the right side of the heart, occurring when lung blood vessels become thickened, narrowed or blocked. Symptoms develop slowly and include shortness of breath, fatigue, chest pressure and leg swelling. There is no cure, but medications, oxygen therapy and sometimes surgery improve symptoms and slow progression.
What the research says
Combining two drug classes cuts clinical worsening in pulmonary arterial hypertension (Group 1). A network meta-analysis of 53 randomized trials (10,670 patients) found that an endothelin receptor antagonist plus a PDE5 inhibitor reduced clinical worsening by 120.7 events per 1,000 patients (95% CI 93.4 to 136.8 fewer; high-certainty evidence) and worked better than either drug class alone (PubMed: Pitre et al., Eur Respir Rev, 2022).
A newer add-on drug, sotatercept, lowered the risk of clinical worsening further. In a network meta-analysis of 18 randomized trials (5,777 patients) with pulmonary arterial hypertension, adding sotatercept reduced clinical worsening compared with placebo (relative risk 0.21; 95% CI 0.11 to 0.41; high certainty) and probably reduced it more than add-on endothelin receptor antagonists (relative risk 0.28; 95% CI 0.14 to 0.55) (PubMed: Pitre et al., Ann Am Thorac Soc, 2024).
What Is Pulmonary Hypertension?
Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. It is not the same as the regular high blood pressure most people measure with an arm cuff.
The condition begins when blood vessels in the lungs become thickened, narrowed, blocked, or destroyed. These changes make it harder for blood to move through the lungs, so blood pressure in the lung arteries rises.
The heart must then work harder to pump blood through the lungs. Over time, this extra effort causes the heart muscle to become weak and fail.
One form, called pulmonary arterial hypertension (PAH), specifically involves narrowed, blocked, or destroyed blood vessels in the lungs. In some people, pulmonary hypertension slowly gets worse and can be life-threatening. There is no cure, but treatments are available to help you feel better, live longer, and improve your quality of life.
Fact | Detail |
What it is | High blood pressure in the lung arteries and right side of the heart |
How it starts | Lung blood vessels become thickened, narrowed, blocked, or destroyed |
Main consequence | Heart must pump harder; heart muscle eventually weakens and fails |
Progression | Develops slowly over months or years; can be life-threatening |
Cure | None currently exists |
Goal of treatment | Improve symptoms, extend life, improve quality of life |
What Are the Symptoms of Pulmonary Hypertension?
Symptoms develop slowly. You may not notice them for months or even years, and they get worse as the disease continues.
The recognized symptoms include:
Symptom | Notes |
Shortness of breath | May first start during exercise and eventually happen at rest |
Blue or gray skin | Depending on skin color, these changes may be harder or easier to see |
Chest pressure or pain | Pressure or pain in the chest area |
Dizziness or fainting | Feeling lightheaded or passing out |
Fast pulse or pounding heartbeat | Heartbeat feels rapid or forceful |
Fatigue | Ongoing tiredness |
Swelling | Occurs in the ankles, legs, and belly area |
These symptoms may be caused by many other health conditions, so seeing a healthcare professional is important for an accurate diagnosis.
When Should You See a Doctor?
If you experience new or worsening shortness of breath, chest pressure, dizziness, fainting, swelling in the legs or belly, or an unexplained pounding heartbeat, see a healthcare professional. Because pulmonary hypertension symptoms mimic many other heart and lung conditions, only a proper medical evaluation can confirm what is causing them.
People who are pregnant or planning pregnancy and have pulmonary hypertension should talk to a healthcare professional first, because the condition can cause serious complications for the mother and the developing baby.
What Causes Pulmonary Hypertension?
Pulmonary hypertension is caused by changes in the cells that line the lung arteries. These changes can make the artery walls narrow, stiff, swollen, and thick, which makes it harder for blood to flow through the lungs.
The condition is sorted into five groups depending on the cause.
Group | Cause | Examples |
Group 1 | Pulmonary arterial hypertension (PAH) | Unknown cause (idiopathic); inherited gene changes (heritable); some medicines or illicit drugs including methamphetamine; congenital heart defect; scleroderma, lupus, cirrhosis |
Group 2 | Left-sided heart disease (most common form) | Left heart failure; left-sided heart valve disease (mitral or aortic valve disease) |
Group 3 | Lung disease | Pulmonary fibrosis (lung scarring); COPD; sleep apnea; extended high altitude in people at high risk |
Group 4 | Blockages in the pulmonary artery | Long-lasting blood clots in the lungs; tumors that block the pulmonary artery |
Group 5 | Other health conditions | Blood disorders (polycythemia vera, essential thrombocythemia); sarcoidosis; glycogen storage disease; kidney disease |
What Is Eisenmenger Syndrome?
Eisenmenger syndrome can also lead to pulmonary hypertension. It is a long-term complication of an unrepaired heart condition present at birth, such as a large hole between the two lower heart chambers called a ventricular septal defect.
The unrepaired hole allows oxygen-rich blood to mix with oxygen-poor blood. The blood then goes to the lungs instead of traveling to the rest of the body, which increases pressure in the pulmonary arteries.
What Are the Risk Factors for Pulmonary Hypertension?
Pulmonary hypertension is usually seen in people ages 30 to 60. Growing older can increase the risk of developing Group 1 pulmonary hypertension (PAH). However, PAH from an unknown cause is more common in younger adults.
Risk Factor | Detail |
Age | Usually seen in people ages 30 to 60; older age raises Group 1 (PAH) risk |
Family history | A family history of the condition |
Weight | Being overweight |
Smoking | Tobacco use raises risk |
Clotting | Blood-clotting disorders or family history of blood clots in the lungs |
Asbestos | A history of being around asbestos |
Congenital heart defect | A heart condition present at birth |
Altitude | Living at 8,000 feet (2,438 meters) or higher |
Medicines | Use of some medicines, including weight-loss drugs |
Illicit drugs | Cocaine or methamphetamine |
What Are the Complications of Pulmonary Hypertension?
As the condition progresses, it can lead to serious complications.
Complication | What Happens |
Right-sided heart enlargement and failure (cor pulmonale) | The heart's right lower chamber enlarges and must pump harder through narrowed or blocked lung arteries; the walls thicken and the chamber stretches; eventually the chamber fails |
Blood clots | Higher risk of clots in the small arteries of the lungs |
Arrhythmias | Irregular heartbeats caused by the condition; can be life-threatening |
Bleeding in the lungs | Can be life-threatening and cause coughing up blood |
Pregnancy complications | Can be life-threatening for the mother and the developing baby |
How Is Pulmonary Hypertension Diagnosed?
Pulmonary hypertension is hard to diagnose early. It is not often found during a routine physical exam. Even in more advanced stages, its symptoms look like those of other heart and lung conditions.
Diagnosis begins with a physical exam and questions about your symptoms, medical history, and family history.
Core Diagnostic Tests
Test | What It Shows |
Blood tests | Can help find the cause of pulmonary hypertension and may detect complications |
Chest X-ray | A picture of the heart, lungs, and chest; checks for other lung conditions that can cause pulmonary hypertension |
Electrocardiogram (ECG/EKG) | A simple test that records the heart's electrical activity and shows how the heart beats |
Echocardiogram | Sound waves create pictures of the beating heart; shows blood flow through the heart and valves; may be done during exercise to see how activity affects the heart |
Right heart catheterization | A thin catheter is guided into the lower right heart chamber and pulmonary artery to measure blood pressure; used to confirm the diagnosis after an echocardiogram suggests it |
Additional Tests
Additional tests may be used to check the lungs and pulmonary arteries or find the underlying cause.
Test | What It Checks |
Exercise stress tests | Walking on a treadmill or riding a stationary bike while the heartbeat is watched, to see how the heart reacts to exercise |
CT scan | X-ray pictures of the body; contrast dye may help blood vessels show up more clearly; a cardiac CT shows heart size and pulmonary artery blockages |
MRI | Magnetic fields and radio waves create detailed heart pictures; shows blood flow in the pulmonary arteries and how well the right lower chamber works |
Lung function test | You blow into a device that measures how much air the lungs can hold and how air flows in and out |
Sleep study | Measures brain activity, heart rate, blood pressure, and oxygen levels during sleep; can diagnose sleep apnea |
V/Q scan | A radioactive tracer given through a vein shows blood flow; an inhaled tracer shows airflow; can tell whether blood clots are causing symptoms |
Lung biopsy | Rarely, a lung tissue sample is taken to check for a possible cause |
Genetic testing may also be recommended. If you carry gene changes that cause pulmonary hypertension, other family members may need to be screened too.
How Is Pulmonary Hypertension Classified?
Once the diagnosis is confirmed, the condition is classified by how symptoms affect your ability to do everyday tasks.
Class | What It Means |
Class I | Diagnosed, but no symptoms during rest or exercise |
Class II | No symptoms at rest; everyday activities like work or grocery shopping may cause shortness of breath or mild chest pain; slight limitation of physical activity |
Class III | Comfortable at rest, but simple tasks like bathing, dressing, or preparing meals cause fatigue, shortness of breath, and chest pain; physical activity very limited |
Class IV | Symptoms occur at rest and during any physical activity; any activity causes increasing discomfort |
Your healthcare team may also use a risk calculator that looks at symptoms and test results to decide what kind of treatment is needed. This process is called pulmonary hypertension risk stratification.
What Are the Treatment Options?
There is no cure for pulmonary hypertension. But treatments can improve symptoms, help you live longer, and keep the disease from getting worse. Finding the right treatment often takes time, because treatments are complex and require many health checkups.
Medications
Medication Type | How It Works | Examples |
Vasodilators | Relax blood vessels and improve blood flow; inhaled, taken by mouth, or given through a vein, sometimes through a continuous pump | Epoprostenol (Flolan, Veletri), treprostinil (Remodulin, Tyvaso, others), iloprost, selexipag (Uptravi) |
sGC stimulators | Relax the pulmonary arteries and lower pressure in the lungs; do not take if pregnant | Riociguat (Adempas) |
Endothelin receptor antagonists | Reverse the effect of a substance that narrows blood vessel walls; may improve energy and symptoms; do not take if pregnant | Bosentan (Tracleer), macitentan (Opsumit), ambrisentan (Letairis) |
PDE5 inhibitors | Increase blood flow through the lungs; also used to treat erectile dysfunction | Sildenafil (Revatio, Viagra), tadalafil (Adcirca, Alyq, Cialis) |
High-dose calcium channel blockers | Relax the muscles in blood vessel walls; only a small number of people with pulmonary hypertension improve while taking them | Amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others), nifedipine (Procardia) |
Blood thinners (anticoagulants) | Help prevent blood clots; can increase bleeding risk, especially around surgery | Warfarin (Jantoven) |
Digoxin | Helps the heart beat stronger and pump more blood; can help control irregular heartbeats | Digoxin (Lanoxin) |
Diuretics (water pills) | Help the kidneys remove excess fluid, reducing the heart's workload and fluid buildup in lungs, legs, and belly | Prescription diuretics |
Oxygen therapy | Breathing pure oxygen if you live at high altitude or have sleep apnea; some people need it all the time | Oxygen therapy |
Surgery and Procedures
If medicines do not control symptoms, surgery may be recommended.
Procedure | What It Involves |
Atrial septostomy | A doctor creates an opening between the upper left and right heart chambers to reduce pressure on the right side of the heart; possible complications include irregular heartbeats (arrhythmias) |
Lung or heart-lung transplant | Sometimes needed, especially for younger people with idiopathic pulmonary arterial hypertension; medicines must be taken for life afterward to prevent organ rejection |
What Lifestyle Changes Help Pulmonary Hypertension?
Lifestyle changes may help improve symptoms. Healthcare teams commonly recommend the following steps.
Lifestyle Step | Detail |
Eat healthy | Diet rich in whole grains, fruits, vegetables, lean meats, and low-fat dairy; avoid saturated fat, trans fat, and cholesterol; use less salt |
Stay active and manage weight | Even mild activity may exhaust some people; for others, moderate exercise like walking can help, especially during oxygen therapy; the team can help plan a program |
Don't smoke | Quit smoking and avoid secondhand smoke; treatment support is available |
Get plenty of rest | Resting can reduce tiredness related to pulmonary hypertension |
Avoid high altitudes | High altitude can make the condition worse; those living at 8,000 feet (2,438 meters) or higher may be advised to consider moving lower |
Avoid blood-pressure-lowering activities | Hot tubs, saunas, and long hot baths or showers can lower blood pressure and cause fainting; avoid heavy lifting or straining |
Review medicines | Tell your team about all medicines you take; some can worsen pulmonary hypertension or affect treatment |
Regular checkups | Report new or worsening symptoms or medicine side effects; ask about treatments that could help |
Get recommended vaccines | Respiratory infections can cause serious problems; ask which vaccines you need |
Plan pregnancy carefully | Talk to a professional before becoming pregnant; the condition can seriously affect mother and baby; birth control pills can raise blood clot risk |
Coping and Support
Talking with other people who have pulmonary hypertension can bring comfort and encouragement. Ask your healthcare team whether any support groups are available in your area.
Preparing for Your Appointment
If you think you are at risk of pulmonary hypertension or might have it, make an appointment for a health checkup. Because there is often a lot to discuss, preparation helps.
What You Can Do
Be aware of any preappointment restrictions, such as not eating or drinking before certain medical tests.
Write down any symptoms you are having, including ones that may not seem related, and note when they began.
Make a list of important personal information, including any family history of pulmonary hypertension, lung disease, heart disease, stroke, high blood pressure, or diabetes, plus major stresses or recent life changes.
List all medicines you take, including vitamins, herbal products, supplements, and over-the-counter medicines.
Take someone with you if possible, since a companion can help remember the information you receive.
Be prepared to discuss your diet and exercise habits.
Make a prioritized list of questions in case time runs out.
Questions to Ask Your Healthcare Team
Question | Why It Matters |
What is the likely cause of my symptoms or condition? | Identifies which of the five groups applies |
What are other possible causes? | Rules out look-alike conditions |
What tests do I need? | Clarifies the diagnostic plan |
What treatment do you recommend? | Establishes the starting treatment |
What are the other treatment options? | Helps you weigh alternatives |
Is there a generic form of the medicine you're prescribing? | Can reduce cost |
What's an appropriate level of physical activity? | Keeps activity safe |
Are there any restrictions I need to follow? | Avoids harmful activities |
How often do I need health checkups? | Sets the follow-up schedule |
I have other health conditions. How can I best manage them together? | Coordinates your care |
Should I see a specialist? | Determines whether specialist care is needed |
Is there information I can take home? What websites do you suggest? | Supports ongoing learning |
What to Expect From Your Doctor
Your healthcare team may ask many questions, and being ready can give you more time to discuss concerns. Expect questions about when your symptoms first began, whether they are constant or come and go, how bad they are on a scale of 1 to 10, and what seems to make them better or worse.
What You Can Do in the Meantime
It is never too late to make healthy lifestyle changes. Do not smoke, eat less salt, and choose nutritious foods. These changes may help prevent pulmonary hypertension from getting worse.
Conclusion
Pulmonary hypertension is a serious but treatable condition in which high pressure in the lung arteries forces the heart to work harder, gradually weakening it. Because symptoms such as shortness of breath, fatigue, and swelling develop slowly and mimic other conditions, early evaluation by a healthcare professional is essential. While no cure exists today, medications that relax and widen blood vessels, oxygen therapy, lifestyle changes, and in some cases surgery or transplant can meaningfully improve symptoms and extend life.
Talk to your healthcare team today if you are experiencing unexplained shortness of breath, chest pressure, or swelling. Early diagnosis opens the door to treatments that can help you live longer and feel better.
Frequently Asked Questions
1. What is pulmonary hypertension in simple terms?
Pulmonary hypertension is high blood pressure in the arteries of the lungs and the right side of the heart. Blood vessels in the lungs become thickened, narrowed, blocked, or destroyed, making it harder for blood to flow and raising pressure in the lung arteries.
2. What are the main symptoms of pulmonary hypertension?
The main symptoms are shortness of breath (starting during exercise and eventually at rest), blue or gray skin, chest pressure or pain, dizziness or fainting, fast pulse or pounding heartbeat, fatigue, and swelling in the ankles, legs, and belly area.
3. What causes pulmonary hypertension?
Pulmonary hypertension is caused by changes in the cells lining the lung arteries that make the walls narrow, stiff, swollen, and thick. It is sorted into five groups: pulmonary arterial hypertension, left-sided heart disease (most common), lung disease, pulmonary artery blockages, and other health conditions.
4. Is pulmonary hypertension curable?
No. There is no cure for pulmonary hypertension, but treatments including medications, oxygen therapy, and sometimes surgery or transplant can improve symptoms, help you live longer, and keep the disease from getting worse.
5. How is pulmonary hypertension different from regular high blood pressure?
Regular high blood pressure affects blood vessels throughout the body and is measured in the arm. Pulmonary hypertension specifically affects the arteries in the lungs and the right side of the heart.
6. Can pulmonary hypertension be genetic?
Yes. One form, heritable pulmonary arterial hypertension, is caused by changes in a gene passed down through families. If you have these gene changes, other family members may also need to be screened.
7. What tests diagnose pulmonary hypertension?
Tests include blood tests, chest X-ray, electrocardiogram, echocardiogram, and right heart catheterization, which confirms the diagnosis by measuring pressure in the pulmonary arteries. CT scans, MRI, sleep studies, lung function tests, V/Q scans, and occasionally lung biopsy may also be used.
8. What are the five groups of pulmonary hypertension?
Group 1 is pulmonary arterial hypertension. Group 2 is caused by left-sided heart disease, the most common form. Group 3 is caused by lung disease. Group 4 is caused by blockages in the pulmonary artery. Group 5 is triggered by other health conditions such as blood disorders or kidney disease.
9. What medications treat pulmonary hypertension?
Treatments include vasodilators, sGC stimulators, endothelin receptor antagonists, PDE5 inhibitors, high-dose calcium channel blockers, blood thinners, digoxin, and diuretics. Oxygen therapy is also used for some people, and some medicines must be avoided during pregnancy.
10. Can I exercise with pulmonary hypertension?
It depends. Even mild activity may be too exhausting for some people. For others, moderate exercise such as walking may be helpful, especially during oxygen therapy. Your healthcare team can help plan an appropriate exercise program.
11. Is pulmonary hypertension dangerous during pregnancy?
Yes. Pulmonary hypertension can be life-threatening for the mother and the developing baby. Anyone with the condition should talk to a healthcare professional before becoming pregnant, and birth control pills can increase blood clot risk, so other options should be discussed.
References
Mayo Clinic Staff. Pulmonary hypertension: Symptoms and causes. Mayo Clinic. April 11, 2025. https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697
Mayo Clinic Staff. Pulmonary hypertension: Diagnosis and treatment. Mayo Clinic. April 11, 2025. https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/diagnosis-treatment/drc-20350702
Pitre T, Su J, Cui S, et al. Medications for the treatment of pulmonary arterial hypertension: a systematic review and network meta-analysis. Eur Respir Rev. 2022;31(165):220036. doi:10.1183/16000617.0036-2022. PMID: 35948391.
Pitre T, Desai K, Mah J, et al. Comparative effectiveness of sotatercept and approved add-on pulmonary arterial hypertension therapies: a systematic review and network meta-analysis. Ann Am Thorac Soc. 2024;21(8):1194-1203. doi:10.1513/AnnalsATS.202311-942OC. PMID: 38820258.
American Heart Association. Pulmonary hypertension: High blood pressure in the heart-to-lung system. American Heart Association. https://www.heart.org/en/health-topics/high-blood-pressure/the-facts-about-high-blood-pressure/pulmonary-hypertension-high-blood-pressure-in-the-heart-to-lung-system
National Heart, Lung, and Blood Institute. Pulmonary hypertension. National Institutes of Health. https://www.nhlbi.nih.gov/health/pulmonary-hypertension
Pulmonary Hypertension Association. Pulmonary Hypertension Association. https://phassociation.org
Medical Disclaimer
This article is for informational purposes only and does not constitute medical advice. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read here.
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