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Cardiac Myxoma

Last updated March 23, 2018

Approved by: Krish Tangella MD, MBA, FCAP

DoveMed.com

Microscopic pathology image showing a myxoma of heart.


What are the other Names for this Condition? (Also known as/Synonyms)

  • Atrial Myxoma
  • Myxoma of Heart

What is Cardiac Myxoma? (Definition/Background Information)

  • Cardiac Myxoma is one of the most common tumors of the heart. These tumors are benign, can be single or multiple, and are commonly found in the left atrium (upper heart chamber). Hence, they are also called Atrial Myxomas
  • When multiple tumors are present, Cardiac Myxoma typically occurs against a background of Carney complex (which comprises a combination of medical conditions that includes multiple tumors, such as myxomas, schwannomas, endocrine disorders, and pigmented skin conditions)
  • Depending on their size and numbers, Cardiac Myxomas may cause heart dysfunction and cardiac arrhythmias. However, in some individuals with small-sized tumors, there may not be any signs and symptoms. Complications due to Cardiac Myxoma may be severe and may include infective endocarditis, thromboembolism affecting certain vital organs, and even sudden death
  • The diagnosis of Cardiac Myxomas might necessitate a thorough physical evaluation by a healthcare provider, imaging studies of the heart, blood tests, and other specialized tests to evaluate heart functioning
  • Generally, small-size tumors that are asymptomatic may require no treatment, apart from close observation. Tumors that cause significant signs and symptoms may be surgically removed
  • The prognosis of Cardiac Myxoma is linked to factors such as tumor size, numbers, and location. The overall outcome may also depend upon the successful treatment of the underlying Carney complex (if present)

Who gets Cardiac Myxoma? (Age and Sex Distribution)

  • Cardiac Myxoma is a common tumor that is observed in a wide age range of individuals including young and old children and adults
  • The observed age range is between 2-97 years, with an average age of diagnosis at 50 years. However, 90% of the cases are seen between the ages 30-60 years
  • Both males and females are affected, though females are affected more than males (male-female ratio is 3:1)
  • All races and ethnic groups are at risk for Myxoma of Heart

Note: When Cardiac Myxomas are seen in association with Carney’s complex, the affected individuals are much younger and the condition is more common in males.

What are the Risk Factors for Cardiac Myxoma? (Predisposing Factors)

  • Carney complex is a risk factor for Cardiac Myxoma in about 5% of the cases. Carney complex is an inherited genetic disorder
  • The risk factors for sporadic tumors are currently unidentified

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Cardiac Myxoma? (Etiology)

The cause of development of Cardiac Myxoma is generally unknown.

  • Single or solitary tumors are known to occur sporadically; in about 95% of the cases
  • The presence of multiple tumors is observed to be linked to Carney complex; a complex genetic disorder causing the formation of tumors in the body. About 5% of the tumors are seen to be associated with this disorder

What are the Sign and Symptoms of Cardiac Myxoma?

The signs and symptoms of Cardiac Myxoma depend on the location of the tumor, the number of tumors, and the size of the tumor. In about 1 in 5 individuals, the condition is asymptomatic. Also, smaller-sized tumors may not present any significant signs and symptoms when compared to larger-sized tumors.

The signs and symptoms of Cardiac Myxoma may include:

  • Sporadic tumors are usually single and syndromic tumors are usually multiple. Syndromic tumors are tumors that occur in individuals with a syndrome (such as Carney complex)
  • When Myxomas of Heart occur along with Carney complex, then presentations of the underlying disorder may be noted
  • Almost 75% of the tumors occur in the left atrium, 20% in the right atrium and 5% in the right or left ventricle. Atrium is the upper chamber and ventricle the lower chamber of the heart respectively
  • Some individuals can have signs and symptoms such as fever, joint pain, muscle aches, general discomfort, and weight loss
  • Some individuals may have heart failure signs and symptoms that include cyanosis (bluish skin), breathing difficulties, fluid in the legs, and abnormal heart rate
  • Both small and large tumors can cause arrhythmia (irregular heartbeat)

How is Cardiac Myxoma Diagnosed?

In order to accurately diagnose Cardiac Myxoma, a healthcare professional may need information from the following tests and exams:

  • Complete evaluation of medical history, along with a thorough physical examination; including examination of the heart (auscultation can show heart murmurs or abnormal heart sounds)
  • Blood tests such as complete blood count (CBC) and erythrocyte sedimentation rate (ESR)
  • Electrocardiogram (EKG or ECG): It is used to measure the electrical activity of the heart, in case of arrhythmias
  • CT or MRI scan of the heart
  • Echocardiography: This procedure uses sound waves to create a motion picture of the heart movement
    • It can help show the size and shape of the heart and how well the chambers and valves are working
    • It can also help visualize the tumors, especially the large tumors; 1 mm and lesser-sized tumors may not be visible though
  • Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
  • Electrophysiological studies to determine where arrhythmia is getting generated in the heart, is often helpful
  • Electron microscopic studies on a tissue biopsy of the tumor are helpful for treatment purposes
  • Genetic mutational analysis to determine the presence of Carney complex

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Cardiac Myxoma?

Some potential complications of Cardiac Myxoma include:

  • Large tumors can compress the heart and severely affect its function
  • Heart failure
  • Severe arrhythmias
  • Thromboembolism is common in many cases; it may affect a variety of organs including the brain and kidney
  • Infection of tumor can result in infective endocarditis
  • Rarely, tumors can undergo malignant transformations to cancer
  • Recurrence: Cardiac Myxomas are known to recur in 1 in 3 individuals in sporadic cases (over 30%). In syndromic cases, the recurrence rate is about 10%
  • Sudden death
  • Complications that arises due to underlying Carney complex

How is Cardiac Myxoma Treated?

Cardiac Myxoma may be treated through the following measures:

  • For individuals with arrhythmias, anti-arrhythmic medication can be given
  • If the arrhythmia persists for a long period of time, then surgical treatment measures may be considered
  • In sporadic cases and for large tumors, a surgical removal of Cardiac Myxoma is a good option
  • Treatment of underlying Carney complex may have to be undertaken (if present)
  • Observation and periodic checkups to monitor the condition is recommended

How can Cardiac Myxoma be Prevented?

Presently, the sporadic cases of Cardiac Myxoma cannot be prevented. Also, there are no specific methods or guidelines to prevent tumor formation, if it occurs in association with Carney complex, which is a genetic condition.

  • Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders such as Carney complex

What is the Prognosis of Cardiac Myxoma?

  • The prognosis of Cardiac Myxoma depends upon the location, size, and number of tumors
  • It is also dependent upon the severity of underlying signs and symptoms associated with the syndrome (Carney complex)
  • Sporadic tumors often have good prognosis upon their surgical removal

Additional and Relevant Useful Information for Cardiac Myxoma:

The following article link will help you understand other heart conditions:

http://www.dovemed.com/healthy-living/heart-center/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 15, 2016
Last updated: March 23, 2018