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How Are the Lungs and Heart Connected in PAH?

Medically reviewed by Vedran Radonić, M.D., Ph.D.
Written by Emily Wagner, M.S.
Updated on January 2, 2026

Key Takeaways

  • Pulmonary arterial hypertension is a condition that affects how your heart and lungs work together by making the blood vessels between them stiff and narrow.
  • View full summary

Some members of myPHteam have asked about how their pulmonary arterial hypertension (PAH) relates to their hearts and lungs. “What does PAH mean or do to your heart? The shortness of breath is very scary,” one member posted.

Your heart and lungs help move oxygen-rich blood throughout your body. A problem with either of these vital organs can spell trouble for your cardiovascular system and the rest of your body. When you’re living with PAH — one of the five types of pulmonary hypertension (PH) — problems usually develop in the lungs, the heart, or both.

This article provides a general overview of the connection between your heart and lungs and how they function together. To understand one system, you’ll need to understand the other. Armed with this knowledge, you’ll have a better idea of what’s happening in your body with PAH.

How Do Your Heart and Lungs Work Together?

Your lungs and heart are interconnected systems that rely on one another to do their jobs. Blood is constantly moving in and out of these organs to supply your body with the oxygen and nutrients it needs. According to the Cleveland Clinic, your heart and lungs circulate about 2,000 gallons of blood every day.

To understand how your heart and lungs work together, we’ll look at how your blood moves through them. It’s different from your systemic circulation, which moves oxygenated blood from your heart throughout your body to your other organs.

Comparison of a heart and lungs with pulmonary hypertension showing an enlarged right ventricle and thickened pulmonary arteries, versus a healthy heart and normal pulmonary vessels.
Pulmonary hypertension affects the heart and lungs because it increases pressure in the pulmonary arteries. The condition makes it harder for your blood to get enough oxygen and puts extra stress on the right side of your heart. (Adobe Stock)

From Your Body to the Right Side of the Heart

Your pulmonary circuit begins in your right atrium. This upper right part of your heart takes in oxygen-poor blood from two big veins — the superior vena cava and the inferior vena cava. The blood travels through your right atrium and passes through the tricuspid valve. These flaps open and close to make sure blood moves the right way through your heart.

The tricuspid valve controls blood from your right atrium into your right ventricle. This lower chamber then pushes blood through the pulmonary valve and into your pulmonary arteries. They’re the only arteries in your body that carry oxygen-poor blood. They branch off into a left artery and a right artery that carry blood to your left lung and right lung.

From Your Lungs to the Left Side of the Heart

Inside the lungs, carbon dioxide from your body is exchanged for oxygen. The blood is then moved into your pulmonary veins and left atrium. This chamber pumps blood through the mitral valve and into your left ventricle. This is your blood’s last stop in the journey through your lungs and heart.

The left ventricle is the part of the heart that pumps oxygen-rich blood to the rest of your body. Blood travels through the aortic valve and into the aorta — the largest artery in your body — and then flows to your other organs, tissues, and cells.

After the blood gives away its oxygen and nutrients, it travels back to your heart through your veins. The cycle then starts over again. Your heart is a powerful muscle that works fast — it takes less than a minute for blood to travel to a body part and return to the heart.

It takes less than a minute for blood to travel around your body and return to the heart.

What Factors Have an Impact on Lung and Heart Function?

Your lungs and heart are closely connected — if a problem arises with one organ, chances are that the other will be affected. Studies show that your lung function is related to your heart health. For example, cardiovascular factors that play a role in lung health include:

  • Blood pressure levels
  • Cholesterol levels
  • Blood glucose (blood sugar) levels
  • Smoking status
  • Exercise frequency

Your lungs and heart are closely connected — if a problem arises with one organ, chances are that the other will be affected.

You can take extra steps to take care of your lungs and heart, especially while living with PAH. Your doctor will likely encourage you to eat a healthy diet, try to limit stress, quit smoking, and exercise regularly.

How Does Pulmonary Arterial Hypertension Affect the Lungs and Heart?

Now that you know how the lungs and heart work together, it’s easier to understand how PAH can affect them. PAH is a type of pulmonary hypertension that happens when you have high blood pressure in your pulmonary arteries, making them stiff and narrow. Again, these blood vessels move deoxygenated blood from your heart to your lungs. PAH makes it harder for these blood vessels to move blood from your heart to your lungs, which can cause symptoms like:

  • Chest pain
  • Fatigue
  • Cough
  • Edema (swelling of the feet, face, or stomach from fluid buildup)

PAH makes it harder for the pulmonary arteries to move blood from your heart to your lungs.

You may have high blood pressure in your pulmonary arteries for several reasons. The World Health Organization (WHO) classifies PH into five distinct groups based on the specific cause. Some types of PH are caused by heart problems. Others are caused by lung diseases that can also hurt the heart.

Group 1 PH refers to pulmonary arterial hypertension. This condition makes your pulmonary arteries stiff, thick, and narrow. The right side of your heart has to work harder to pump blood to your lungs. Eventually, PAH can lead to right-sided heart failure.

Many cases of PAH are idiopathic, meaning they have no known cause. Other causes of PAH include:

  • Genetics — About 15 percent to 20 percent of PAH cases are heritable (passed down from one generation to the next).
  • Use of certain drugs — PAH can develop from taking diet pills, amphetamines, or certain cancer medications.
  • Other diseases — Conditions linked with PAH include heart disease present at birth, lupus, and scleroderma (a disease that hardens and tightens the skin and can also affect internal organs).

Although PAH is a progressive disease, meaning it gets worse over time, PAH medications can help.

How Do PAH Medications Affect the Lungs and Heart?

The goal of PAH medications is to slow disease progression and improve PAH symptoms. Just as PAH can affect the lungs and heart, PAH medications can affect these vital organs, too.

There are several types of PAH medications, and they work in different ways.

Activin Signaling Inhibitors

Activin signaling inhibitors are a newer type of drug for treating PAH. The first drug in this class has been found to help improve PAH symptoms, and it potentially helps slow disease progression by stopping the chain of events leading to increased pressure on the heart.

Anticoagulants

Anticoagulants can help prevent blood clots, which may contribute to disease progression in PAH.

Diuretics

Also known as water pills, diuretics help widen the blood vessels, making it easier for the kidneys to eliminate excess fluid and salt. For people with PAH, diuretics can help reduce swelling.

Oxygen Therapy

You may experience hypoxia (low oxygen levels) with PAH. Oxygen therapy can increase oxygen levels in the blood and reduce blood pressure by widening the blood vessels. Some people with PAH need oxygen all the time, whereas others require it only during physical activity, while on an airplane, or when at high altitudes.

Pulmonary Vasodilators

Pulmonary vasodilators can help improve blood flow by opening narrowed blood vessels. This can reduce the pressure on the heart and help ease PAH symptoms.

Calcium Channel Blockers

Calcium channel blockers change the way the body uses calcium. By relaxing the blood vessel walls to encourage more blood flow, these drugs can help lower blood pressure in the pulmonary arteries. Very few individuals with PAH are treated with calcium channel blockers.

Phosphodiesterase 5 Inhibitors

Phosphodiesterase 5 inhibitors help increase blood flow through the lungs and lower blood pressure.

Prostacyclin

People with PAH may be low in prostacyclin, a hormone released by the lungs that affects how blood vessels dilate. Taking a version of this hormone through a medication can help blood vessels relax, lowering blood pressure.

Endothelin Receptor Antagonists

Endothelin receptor antagonists help decrease blood pressure in the lungs and help the heart work better.

Soluble Guanylate Cyclase Stimulators

Soluble guanylate cyclase stimulators can help lower the pressure in the lungs by relaxing the pulmonary arteries.

Talk to Your Doctor

These are just some of the types of PAH medications your doctor might recommend. According to the Pulmonary Hypertension Association, new PAH treatments continue to improve outcomes and quality of life. Ask your healthcare provider which PAH treatments they recommend and why.

Over time, your healthcare provider will monitor your condition through blood tests, echocardiograms, and other tests and tools. They’ll consider your risk factors, family history, health history, and other medical conditions when deciding your treatment plan. They may also recommend lifestyle changes, like eating less salt and getting regular physical activity.

If your PAH gets worse, your doctor might suggest new or different treatments. Don’t hesitate to ask your doctor any questions about their recommendations or the potential side effects of PAH medications.

The lungs and heart work together to keep the body healthy. Knowing how PAH and PAH medications affect these essential organs can help you make informed decisions about your health, manage symptoms, and work with your healthcare providers to improve your quality of life.

Join the Conversation

On myPHteam, people share their experiences with pulmonary hypertension, get advice, and find support from others who understand.

If you have been diagnosed with pulmonary arterial hypertension, what steps do you take to protect your heart and lungs? Let others know in the comments below.

References
  1. Your Heart and Lungs: The Ultimate Relationship — American Lung Association
  2. Circulatory System — Cleveland Clinic
  3. Heart Chambers — Cleveland Clinic
  4. Pulmonary Arteries — Cleveland Clinic
  5. Correlation Between Heart Rate Variability and Pulmonary Function Adjusted by Confounding Factors in Healthy Adults — Brazilian Journal of Medical and Biological Research
  6. Pulmonary Arterial Hypertension — National Organization for Rare Disorders
  7. What Is Pulmonary Hypertension? — National Heart, Lung, and Blood Institute
  8. Pulmonary Hypertension — Mayo Clinic
  9. Learn About Pulmonary Arterial Hypertension — American Lung Association
  10. Medications for PAH — Lung Foundation Australia
  11. FDA Approves Merck’s Winrevair (Sotatercept-Csrk), a First-in-Class Treatment for Adults With Pulmonary Arterial Hypertension (PAH, WHO* Group 1) — Merck
  12. Sotatercept — UC Health
  13. Treating and Managing PAH — American Lung Association
  14. Is Anticoagulation Beneficial in Pulmonary Arterial Hypertension?: A Systematic Review and Meta-Analysis — Circulation: Cardiovascular Quality and Outcomes
  15. Diuretics — Cleveland Clinic
  16. Pulmonary Arterial Hypertension — Cleveland Clinic
  17. Oxygen Has Potential Benefits as a Therapy for Pulmonary Arterial Hypertension — AJMC
  18. Calcium Channel Blockers — Cleveland Clinic
  19. Tyvaso (Oral Inhaled Treprostinil) — Pulmonary Hypertension Association
  20. PAH Medication and Treatment Guide — American Lung Association
  21. New PAH Treatments Increase Patient Options — Pulmonary Hypertension Association

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