Atrial myxoma- familial

Synonyms

1

Overview

An atrial myxoma is a benign tumor of the heart, most commonly found within the left and then the right atria on the interatrial septum. It grows on the wall (atrial septum) that separates the two sides of the heart.

Symptoms

Symptoms may occur at any time, but most often they accompany a change of body position. Pedunculated myxomas can have a "wrecking ball effect", as they lead to stasis and may eventually embolize themselves. Symptoms may include:

  • Shortness of breath with activity
  • Platypnea - Difficulty breathing in the upright position with relief in the supine position
  • Paroxysmal nocturnal dyspnea - Breathing difficulty when asleep
  • Dizziness
  • Fainting
  • Palpitations - Sensation of feeling your heart beat
  • Chest pain or tightness
  • Sudden Death (In which case the disease is an autopsy finding)

The symptoms and signs of left atrial myxomas often mimic mitral stenosis. General symptoms may also be present, such as:

  • Cough
  • Pulmonary edema, as blood backs up into the pulmonary artery , after increased pressures in the left atrium and atrial dilation
  • Hemoptysis
  • Fever
  • Cachexia - Involuntary weight loss
  • General discomfort (malaise)
  • Joint pain
  • Blue discoloration of the skin, especially the fingers (Raynaud's phenomenon)
  • Fingers that change color upon pressure or with cold or stress
  • Clubbing - Curvature of nails accompanied with soft tissue enlargement of the fingers
  • Swelling - any part of the body
  • Presystolic heart murmur
  • Embolism
  • Asymptomatic
  • Breathlessness on exertion
  • Orthopnea

These general symptoms may also mimic those of infective endocarditis.

Causes

Myxomas are the most common type of primary heart tumor. The tumor is derived from multipotential mesenchymal cells and may cause a ball valve-type obstruction.

Diagnosis

A "tumor plop" (a sound related to movement of the tumor), abnormal heart sounds, or a murmur similar to the mid-diastolic rumble of mitral stenosis 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.

Tests may include:

  • Echocardiogram and Doppler study
  • Chest x-ray
  • CT scan of chest
  • Heart MRI
  • Left heart angiography
  • Right heart angiography
  • ECG—may show atrial fibrillation

Blood tests: A FBC may show anemia and increased WBCs (white blood cells). The erythrocyte sedimentation rate (ESR) is usually increased.

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. 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.

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.

Resources

  • NIH