What Is Atrial Myxoma?
An atrial myxoma is defined as a noncancerous tumor that develops at the superior aspect, either on the left or right side of the heart. It most commonly starts growing on the wall that separates the heart into right and left halves. This wall is referred to as the atrial septum. It belongs to the category of primary heart tumors. Primary heart tumors are the ones that start to develop initially at the heart itself rather than developing from some other part of the body and migrating to the heart.
What Is the Epidemiology of Atrial Myxoma?
Atrial myxomas are the reason for 0.25 % of overall heart diseases. However, their annual incidence does not frequently exceed 0.5 to 1 cases per million people. Also, atrial myxomas are the most frequently occurring primary cardiac tumors in adults, representing more than 50 % to 85 % of benign tumors. It is more commonly noted in the female population.
What Are the Causes of Atrial Myxoma?
Most cases of atrial myxoma are very sporadic, and the exact cause tends to be unknown. Familial atrial myxomas have a genetic influence and show autosomal dominant transmission. Carney’s syndrome (an uncommon genetic disorder characterized by dark skin spots and tumors in the skin, nerves, endocrine glands, and heart) is identified to be genetically heterogeneous in nature, and it is caused due to defects present in more than one gene. Carney’s syndrome is estimated to account for almost seven percent of all atrial myxomas without any bias for age or sex. Abnormalities in the short arm of chromosome 2 known as Carney and chromosome 12 termed Ki-ras oncogene are known to be the cause. Atrial myxoma can affect more than one region of the heart at the same time.
What Are the Symptoms of Atrial Myxoma?
Most cases of atrial myxomas do not usually present with symptoms. They are very often accidentally discovered when an imaging study such as an echocardiogram (ECG), magnetic resonance imaging (MRI), or computed tomography (CT) is done for some other reason.
Symptoms can occur at any part of the time from when the tumor has started to develop, but often they start occurring with a change in body position.
The most common symptoms of a myxoma may include the following:
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The difficulty of breathing when a person is lying flat, on one side, or in other positions.
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Breathing difficulty when a person is asleep.
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Chest pain or a feeling of tightness in the left side of the chest.
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Feeling dizzy.
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Fainting.
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The sensation of feeling like the person’s heart is running too fast or is pounding in nature is known as palpitations.
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Shortness of breath while the person is involved in an activity such as exercise.
The symptoms and signs presented by left atrial myxomas very frequently mimic mitral stenosis symptoms that are caused due to the narrowing of the valve present between the left atrium and the left ventricle. Right atrial myxomas very rarely produce symptoms. In those cases, symptoms are seen only when the tumor has grown to be quite large, about five inches wide, or 13 centimeters by length.
The least common symptoms of atrial myxoma may include:
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Bluish skin and mucosal membranes known as cyanosis.
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Constriction of peripheral end blood vessels is known as Raynaud's phenomenon. It causes pain in the affected fingers.
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Non-productive cough.
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Fever.
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Weight loss
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Fingers change color when pressure is exerted or with cold or stress.
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The curvature of nails is accompanied by a swelling of soft tissue termed clubbing of the fingers.
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A general feeling of discomfort or not being well.
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Joint pain.
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Swelling in different body parts, commonly in the ankle regions.
What Are the Characteristics of Atrial Myxoma?
Atrial myxomas appear to be polypoid, round, or oval in nature. They look gelatinous with a smooth or lobulated surface. Their color usually ranges from white, yellowish, or brown. The most frequent site of attachment is at the border of the structure of the heart called fossa ovalis in the left atrium. However, myxomas are also noted to originate these days frequently from the posterior atrial wall, the anterior atrial wall, or from the atrial appendage. The movement of the tumor depends on the extent of attachment of the tumor to the interatrial septum and the stalk length.
How Is It Diagnosed?
The early diagnosis might be quite challenging as the symptoms are not specific. Left atrial myxoma may or may not show characteristic sounds on auscultation. Laboratory studies are also nonspecific for atrial myxoma. The usually noted laboratory findings include elevated levels of erythrocyte sedimentation rate (ESR), C-reactive protein, and serum gamma globulin levels. Leukocytosis can be present. Anemia is also noticed which may be normochromic or hypochromic. Hemolytic anemia also occurs because of the destruction of red blood cells by the growth of the tumor. Serum interleukin-6 levels are also noted to be raised and can be used as a good marker to check for recurrence. Two-dimensional echocardiography is the gold-standard diagnostic procedure of choice.
What Are the Treatment Options?
The treatment of choice for atrial myxoma is the surgical removal of the tumor by median sternotomy. The recent advancement of mini-thoracotomy by robotically assisted surgery has been reported to have a comparatively shorter duration of hospital stay, and it is presently a safe and effective method for excising atrial myxoma. Some patients will also need their mitral valve to be replaced. If the surgery did not remove the tumor cells completely it might recur.
What Is the Prognosis?
Untreated, atrial myxoma can cause an embolism when the tumor cells break off and travel to the bloodstream. This can result in blocks in blood flow or even cause the tumor to metastasize and grow in another part of the body. Cells of the tumor can move to organs like the brain, eye, or limbs. This might require emergency surgery to prevent sudden death.
What Are the Complications?
There are some complications seen in atrial myxoma, which can either be due to the lack of treatment or failure of proper treatment. That includes,
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Arrhythmias.
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Pulmonary edema.
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Peripheral emboli.
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The spread of the tumor.
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Blockage of the mitral valve.
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How Long Is the Hospital Stay for Atrial Myxoma Patients?
Cardiac myxomas are the most commonly found non-cancerous intracardiac tumors. The treatment option is the surgical removal of the lesion, the hospital stay on average ranges from around six to twenty days. Open-heart surgical options are used for removing atrial myxoma.
Usually, recurrence is not seen after the surgery, but in very few cases, recurrence may occur. Therefore, follow-ups are essential for a period of up to seven years following the surgery. Recurrence may occur due to the following reasons:
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The tumor became cancerous.
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Inadequate tumor removal during surgery.
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Multiple tumors that went unnoticed.
Conclusion
Atrial myxomas are non-cancerous tumor that develops on the upper chamber of the heart. The exact cause tends to be unknown in most cases. Most cases are asymptomatic and are accidentally discovered during imaging studies. The treatment option is the surgical removal of the lesion, follow-ups are needed as recurrence is seen in a few cases.
