Madisons Foundation - Moms And Dads In Search Of Needed Support

What

Atrial myxomas are tumors of heart tissue, specifically the non-muscle tissues that make up the heart. While most cardiac tumors are caused by the spread of tumors from somewhere else, atrial myxomas actually originate in the heart. They are usually found in the upper chambers of the heart, or atria. The tumor starts from the wall that divides the top two chambers of the heart and grows outward. It is only when the tumor grows large enough to interfere with the valves inside the heart or impede blood flow into or out of the heart that it causes symptoms.

Who

Atrial myxomas are rare, occurring in about 0.007% of the population. In other words, there are about 75 myxomas discovered for every one million people. Roughly 3/4 of myxomas occur in the left atrium, while the remainder occur in the right atrium. Myxomas can occur sporadically or be inherited in families. Familial myxomas can occur as part of a variety of rare syndromes, such as the Carney syndrome, which has myxomas in the breast, skin, thyroid or neural tissues. These individuals can also have flat brown discoloration of the skin and increased levels of cortisone. The other syndromes associated with atrial myxomas are the NAME syndrome (standing for Nevi, Atrial myxoma, Myxoid neurofibroma and Ephelides) and the LAMB syndrome (Lentigines, Atrial Myxomas and Blue nevi). Of the sporadically occurring myxomas, 75% are found in women, while those that tend to occur in families usually are evenly divided between men and women. Myxomas can occur in anyone from age 3-83, but the average age for a new familial myxoma is age 25, while those that appear sporadically usually arise at age 56.

Signs and Symptoms

Symptoms of an atrial myxoma are variable. They are usually the result of obstruction of blood flow through the heart, leading to fainting, shortness of breath, dizziness, fatigue, fainting, swelling of the feet, legs, abdomen or neck, weight loss, pain in the joints or chest pain. Occasionally people will have fever, if the myxoma becomes infected, as can rarely occur. Sometimes the symptoms are more noticeable after changes in position. Most myxomas cause no symptoms whatsoever.

Possible Causes

Most cases of atrial myxomas are sporadic with the exact cause unknown. Familial cases, however, are transmitted in an autosomal dominant fashion. This means that one parent has the gene responsible for myxomas, and this parent has a 50% chance of passing this gene on to their offspring. The Carney syndrome, a form of familial myxoma, is thought to be due to defects in chromosome 2 and chromosome 12.

Diagnosis

The best test is an echocardiograph (ultrasound) of the heart which uses sound waves to look at the structure of the four different chambers of the heart. Because most myxomas are found in the back part of the heart, an ultrasound probe inserted down the throat while a person is given anesthesia is the best method to detect an atrial myxoma. The next best method is to place the ultrasound probe over the chest to look at the heart structures, and this is how most are detected. A chest x-ray can sometimes show buildups of calcium in the tumor, but is not the best method to detect myxomas. Sometimes a physician can detect a myxoma with their stethoscope when the tumor makes a unique sound (tumor plop) or causes a heart murmur when it shifts position inside the heart.

Treatment

Usually surgical removal of the tumor is the treatment of choice. The surgery is fairly safe, with a mortality rate of 2.2%, though occasionally part of the tumor can break off during the surgery and get stuck in other blood vessels, causing other problems such as a stroke or pulmonary embolism. Because the tumor grows from the heart tissue, it is important to make sure that the entire tumor is found and removed. This is done using cardiac bypass, a machine that circulates the blood in the body, thereby allowing the surgeons to work directly on the heart. After the tumor is removed, a special cloth patch is then placed to repair the hole left behind.

Prognosis

Most individuals do very well with no further symptoms. Recurrences are possible and depend on how the tumor was acquired. If the tumor is sporadic, surgery is usually curative and it rarely recurs. If the tumor is part of a familial syndrome, the recurrence rate is about 20%.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Medline Plus
http://www.nlm.nih.gov/medlineplus/ency/article/007273.htm
A well written, easy to read article on Atrial Myxoma with definitions of medial words.

Medline Plus
http://www.nlm.nih.gov/medlineplus/ency/imagepages/18079.htm
A great illustration of a right Atrial Myxoma

Medline Plus
http://www.nlm.nih.gov/medlineplus/ency/imagepages/18078.htm
A great illustration of a left Atrial Myxoma

Google Search for Atrial Myxoma

References and Sources

http://emedicine.com/med/topic186.htm

Bernstein D (2007) The cardiovascular system: Tumors of the heart, in Nelson Textbook of Pediatrics, Behrman RE, Kliegman RM, Jenson HB (eds) 18th Edition, WB Saunders. p 1581.

Puntila J, Hakala T, Salminen J, Pihkala J.(2006). Positive genetic test led to an early diagnosis of myxoma in a 4-year-old boy. Interact Cardiovasc Thorac Surg. 2006 Oct; 5(5):662-3. Epub 2006 Jul 28

Rahmanian PB, Castillo JG, et al (2007). Cardiac myxoma: preoperative diagnosis using a multimodal imaging approach and surgical outcome in a large contemporary series. Interact Cardiovasc Thorac Surg. 2007 Aug; 6(4):479-83. Epub 2007 May 30.