Medical Disease: Atrial myxoma

Atrial myxoma

An atrial myxoma is a noncancerous tumor in the upper left or right side of the heart. It grows on the wall (atrial septum) that separates the two sides of the heart.

Causes

A myxoma is a primary heart (cardiac) tumor. This means that the tumor started within the heart. Most heart tumors start somewhere else.

Primary cardiac tumors are rare. Myxomas are the most common type of these rare tumors. About 75% of myxomas occur in the left atrium of the heart, usually beginning in the wall that divides the two upper chambers of the heart. The rest are in the right atrium. Right atrial myxomas are sometimes associated with tricuspid stenosis and atrial fibrillation.

Myxomas are more common in women. About 10% of myxomas are passed down through families (inherited). Such tumors are called familial myxomas. They tend to occur in more than one part of the heart at a time, and often cause symptoms at a younger age than other myxomas.

Symptoms

Symptoms may occur at any time, but most often they accompany a change of body position. Symptoms may include:

Breathing difficulty when lying flat
Breathing difficulty when asleep
Chest pain or tightness
Dizziness
Fainting
Sensation of feeling your heart beat (palpitations)
Shortness of breath with activity

The symptoms and signs of left atrial myxomas often mimic mitral stenosis.

General symptoms may also be present, such as:

Blueness of skin, especially the fingers (Raynaud's phenomenon)
Cough
Curvature of nails accompanied with soft tissue enlargement (clubbing) of the fingers
Fever
Fingers that change color upon pressure or with cold or stress
General discomfort (malaise)
Involuntary weight loss
Joint pain
Swelling - any part of the body

These general symptoms may also mimic those of infective endocarditis.

Exams and Tests

The health care provider will listen to the heart with stethoscope. A "tumor plop" (a sound related to movement of the tumor), abnormal heart sounds, or murmur may be heard. These sounds may change when the patient changes position.

Right atrial myxomas rarely produce symptoms until they have grown to be at least 13 cm (about 5 inches) wide.

Imaging tests may include:

Blood tests:

A complete blood count may show anemia and increased white blood cells. The erythrocyte sedimentation rate (ESR) is increased.

Treatment

The tumor must be surgically removed. Some patients will also need their mitral valve replaced. This can be done during the same surgery.

Myxomas may come back if surgery did not remove all of the tumor cells.

Outlook (Prognosis)

Although a myxoma is not cancer, complications are common. Untreated, a myxoma can lead to an embolism (tumor cells breaking off and traveling with the bloodstream), which can block blood flow or cause the myxoma to grow in another part of the body. Myxoma fragments can move to the brain, eye, or limbs.

If the tumor grows inside the heart, it can block blood flow through the mitral valve and cause symptoms of mitral stenosis. This may require emergency surgery to prevent sudden death.

Possible Complications

Arrhythmias
Pulmonary edema
Peripheral emboli
Spread (metastasis) of the tumor
Blockage of the mitral heart valve

When to Contact a Medical Professional

Tell your health care provider if there is any family history of myxomas or if you have symptoms of atrial myxoma.

Update Date: 5/12/2008

Updated by: Larry A. Weinrauch, MD, Assistant Professor of Medicine, Harvard Medical School, and Private practice specializing in Cardiovascular Disease, Watertown, MA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.