Myxoma

A myxoma is a noncancerous (benign) cardiac tumor that can form in your heart. Even though it isn’t cancer, a cardiac myxoma can pose serious risks by getting in the way of your heart’s function. For example, the tumor may disrupt blood flow through a heart valve. Or bits of the tumor may break off and travel through your blood, leading to life-threatening blockages in arteries or veins.

If you’re diagnosed with a myxoma, you’ll need surgery to remove it. This usually happens soon after your diagnosis. Taking the myxoma out lets your heart work normally and lowers your risk of complications.

Myxomas can develop in anyone at any age, but they usually affect people between 30 and 60. The average age at diagnosis is 50. They affect females more often than males.

While they’re rare overall, myxomas are the second most common type of noncancerous primary heart tumor. Experts used to think myxomas were the most common, but they now know that papillary fibroelastomas occur more often.

Where do myxomas usually occur?

Most myxomas develop in the heart. These are called cardiac myxomas. Within your heart, they can form in your:

• Upper left chamber (left atrium): This is the most common place.
• Upper right chamber (right atrium): This is the next most common place.
• Lower chambers (ventricles) or heart valves: These are the least common places.

You might hear the term “atrial myxoma.” This means the tumor is in either of your upper heart chambers. Atrial myxomas usually grow from the muscular wall (septum) that separates your left atrium and right atrium. The tumor may grow from a stalk (pedicle) that lets it sway as blood flows around it. This means the tumor can sometimes dangle into a heart valve, keeping it from closing as it should.

Rarely, myxomas can form in other parts of your body, including your:

• Jawbone (odontogenic myxoma)
• Muscle (intramuscular myxoma)
• Skin (cutaneous myxoma)

Symptoms of a myxoma

A myxoma can cause a wide range of symptoms depending on where it is and how it affects blood flow inside your heart. It’s possible to have no symptoms, meaning you don’t feel or notice anything. But most people have some symptoms. These are the most common:

• Chest pain or discomfort
• Dizziness or fainting
• Fatigue
• Fever
• Heart palpitations
• Shortness of breath
• Weight loss

Myxoma causes

Most myxomas have no clear cause. They occur sporadically, meaning they affect people without any known family history or genetic reason. But about 1 in 10 myxomas occur in people with Carney’s complex. This is a rare genetic condition that causes tumors in the heart and other locations, as well as other signs and symptoms.

With Carney’s complex, you’re more likely to have multiple myxomas in your heart. Those without this condition usually just have one (isolated myxoma).

Complications of myxomas

A cardiac myxoma can cause a dangerous condition called an embolism. This is a blockage in a blood vessel that severely reduces blood flow. It can happen if a piece of the tumor breaks off and travels through your bloodstream. It can also happen if blood clots form on the tumor and then break away.

What happens next depends on the blockage location and may include:

• Acute limb ischemia: Sudden and severe drop in blood flow to your leg or arm
• Heart attack: Lack of blood flow to part of your heart muscle
• Pulmonary embolism: Reduced blood flow to your lungs
• Eye stroke (retinal artery occlusion): Lack of blood flow to your retina
• Stroke: Lack of blood flow to part of your brain

These are all medical emergencies. Seek immediate medical attention if you think you or someone you’re with has one of these conditions.

Besides an embolism, other possible complications of a cardiac myxoma include:

• Damage to your heart valves (mitral or tricuspid)
• Heart failure
• Sudden cardiac death

How doctors diagnose this condition

Healthcare providers diagnose myxomas through physical exams and testing. Myxomas sometimes take a while to diagnose because their symptoms make them look like other conditions.

During an exam, your provider will talk with you about your symptoms. They’ll listen to your heart with a stethoscope. A myxoma may cause a heart murmur that has a distinct “tumor plop” sound. This is the sound of the myxoma temporarily blocking a valve.

Tests you may need to diagnose a myxoma include:

• Echocardiogram (echo): This is the main test providers use to diagnose myxomas. An echo that’s done on the outside of your chest (transthoracic) may be enough. But you may also need the type where providers guide a tiny device down your esophagus (transesophageal).
• Heart CT or MRI scans: These can help when an echo doesn’t show enough detail to confirm a diagnosis.
• Blood tests: Myxomas can cause certain changes in your blood, like low red blood cells, low platelets or increases in markers of inflammation. These findings alone can’t diagnose a myxoma. But they’re useful insights when combined with other test results.

How is a cardiac myxoma treated?

Heart surgery is the only way to treat a myxoma. Your surgeon will remove the tumor and repair any nearby heart tissues that are damaged. This may involve using tissue from your pericardium (the sac that surrounds your heart) to repair portions of your heart chamber walls. Your surgeon may also need to repair or replace your mitral valve if it’s severely damaged.

The exact method for your surgery will depend on your age and health history. Some people need open-heart surgery, which involves a long incision down the center of your chest. Others are candidates for minimally invasive surgery, including robotic-assisted techniques.

Your surgeon will explain the details of myxoma surgery in your case, including how long it’ll take and what you can expect for recovery. They’ll also explain any risks. In general, possible complications include:

• Bleeding
• Infection
• Abnormal heart rhythms (arrhythmias), which may need treatment with a permanent pacemaker

When should I see my healthcare provider?

Call your healthcare provider right away if you have any symptoms of a myxoma. These symptoms may indicate another heart problem, rather than a myxoma. But for any issue, early treatment can help you have a better outcome. You should also call your provider if you have a fever that lasts a long time and has no clear reason.

If you get a myxoma diagnosis, your provider will let you know how often you need to return for visits and when you need surgery.

After surgery, be sure to go to all your follow-up appointments. These are very important. Your provider will check your heart function and monitor for any signs that tumors are coming back.

What questions should I ask?

Talk with your provider about your diagnosis and treatment plan. You may want to ask:

• How large is the myxoma?
• How is the myxoma affecting my body?
• Is there more than one tumor?
• When will I need surgery?
• What will the surgery involve?
• Do I need any medications to manage symptoms or complications?
• How often should I come back for follow-ups or tests?

What can I expect if I have this condition?

Most people have an excellent prognosis (outlook) after myxoma surgery. Some research shows you can live just as long as people without a cardiac myxoma.

It’s possible for myxomas to come back (recur) after successful surgery. This is more likely to happen if you have Carney’s complex. Your healthcare provider will monitor your heart closely in the years following your surgery. If myxomas do return, you may need another surgery to remove them.