Keywords

Case Presentation

An 59-year-old man with a history of hypertension, hyperlipidemia, presented to the emergency department with progressive swelling of feet. Physical examination demonstrated normal and regular heart sounds, and normal respiratory rate and oxygen saturation. Echocardiogram described a well-circumscribed echo-dense pedunculated mass in the right atrial cavity, which was attached to the interatrial septum with short stalk and also with obstructing the tricuspid valve annulus, measuring 3.7 × 5.2 cm at its widest diameter (Figs. 5.1, 5.2, 5.3, 5.4, 5.5 and 5.6).

Fig. 5.1
figure 1

Large well-circumscribed echo-dense mass in the right atrial cavity

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Fig. 5.2
figure 2

Large well-circumscribed echo-dense mass that filled right atrial cavity

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Fig. 5.3
figure 3

Large well-circumscribed echo-dense mass that obstructing the tricuspid valve annulus

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Fig. 5.4
figure 4

Significant Doppler flow gradient due to large well-circumscribed echo-dense mass that obstructing the tricuspid valve annulus (Velocity: 2 m/s)

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Fig. 5.5
figure 5

3D view. Large well-circumscribed echo-dense mass in the right atrial cavity

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Fig. 5.6
figure 6

Large extracted mass from RA in operating room

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Discussion and Management

Myxomas are the most common type of adult primary heart tumor. Most myxomas arise sporadically (90%), and only about 10% are thought to arise due to inheritance.

About 10% of myxomas are inherited, as in Carney syndrome. 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. Other abnormalities are observed in people with Carney syndrome include skin myxomas, pigmentation, endocrine hyperactivity, schwannomas, and epithelioid blue nevi. Myxomas are more common in women than in men.

General symptoms of myxoma may also be present, such as:

  • Cough

  • Fever

  • Cachexia—Involuntary weight loss

  • General discomfort (malaise)

  • Joint pain

  • Blue discoloration of the skin, especially the fingers change color upon pressure, cold, or stress (Raynaud’s phenomenon)

  • Clubbing—Curvature of nails accompanied with soft tissue enlargement of the fingers

  • Swelling—any part of the body

The symptoms and signs of left atrial myxomas probably mimic the mitral stenosis. Right atrial myxomas rarely produce symptoms until they have grown to be obstructive on TV.

The CMR of our patient confirmed the myxoma too and tumor surgically removed there is no need for TV repair. He discharged very well.

Some patients will also need their mitral or tricuspid valves to repair or replaced. This can be done during the same surgery, also myxomas may come back if the surgery did not remove all of the tumor cells [1,2,3,4,5,6].