What is Atrial Myxoma?
Myxomas are the most common type of heart tumor. It is estimated that over 75% of myxomas start in the left side of the heart, either at the ring surrounding the mitral valve or the wall dividing the two atria. 20% develop in the right side of the heart, while 5% grow in both chambers and the ventricles. Atrial myxomas – those in the heart’s chambers – can lead to three main complications: blockages, clots, and general symptoms like fever and weight loss.
What Causes Atrial Myxoma?
Studies that examine tissue using special dyes, known as immunohistochemical studies, hint that the cells in myxomas (a type of noncancerous tumor) come from a kind of cell that can turn into different types of cells, including nerve and blood vessel cells. About 10% of myxomas are part of an inherited condition called Carney’s complex, which passes through families. However, the majority of myxoma cases seem to happen at random with no clear inherited pattern. The precise cause of atrial myxoma, a tumor that develops in the heart, is still being researched.
Risk Factors and Frequency for Atrial Myxoma
Atrial myxomas, a type of heart tumor, mostly affect women, particularly between their 40s and 60s. Some studies show that the ratio of women to men with these tumors in the left and right parts of the heart are 2.05:1 and 0.75:1, respectively. Atrial myxoma is rarely seen in children.
Signs and Symptoms of Atrial Myxoma
An atrial myxoma is a type of heart tumor that can show a variety of symptoms. The kind of symptoms can depend on which side of the heart the myxoma is situated in, and they can cause a range of heart-related issues.
Patients with myxomas on the left side of the heart may experience signs and symptoms related to heart problems like valve obstruction and heart failure. These include difficulties in breathing, especially during physical activity, difficulty in breathing while lying down, and swelling of the lungs. They might also experience a characteristic sound called a “tumor plop” when the doctor listens to their heart.
On the right side of the heart, myxomas can also cause heart problems that result in symptoms like difficulty in breathing during physical activity, swelling of the lower legs, enlargement of the liver and fluid accumulation in the abdomen. Physicians might pick up a particular sound during a check-up and some may observe a noticeable pulse in the neck’s veins.
Both left and right-sided myxomas can penetrate the heart muscle, causing irregular heart rhythms.
- Difficulties in breathing while doing physical activity
- Difficulty in breathing while lying flat
- Swelling of the lungs
- Tumor plop sound when the doctor listens to their heart
- Swelling of the lower legs
- Enlargement of the liver
- Fluid accumulation in the abdomen
- Physician hearing a different sound during a check-up
- Noticeable pulse on patient’s neck veins
- Irregular heart rhythms
Myxomas can also lead to clotting problems. Left atrial myxomas can increase the risk of blood clots that can reach other body parts, like the brain, eyes, and major organs, potentially causing stroke, loss of vision, chest pain, and difficulty in breathing. On the other hand, right atrial myxomas can cause obstruction of the lung’s arteries or systemic clotting problems if there’s also a hole in the heart’s septum.
Patients with atrial myxoma can also experience general symptoms like fever, tiredness, loss of appetite, joint pain, and weight loss due to the release of a protein called IL-6. This protein can cause myxoma cells to multiply and trigger an inflammatory response.
Lastly, atrial myxoma can also be a part of a complex condition called Carney’s complex. People with Carney’s complex have unusual skin pigmentation and develop tumors in the hormone-making tissues like adrenal glands, thyroid, testes, and ovaries. They can also develop a pituitary tumor that can cause a condition called acromegaly. These patients often experience recurring atrial myxomas even after removal.
Testing for Atrial Myxoma
Basic blood tests can reveal a low red blood cell count or anemia, an increase in white blood cells or leukocytosis, a decrease in blood platelets or thrombocytopenia, a high rate at which red blood cells fall to the bottom of a test tube or elevated erythrocyte sedimentation rate, and high levels of a protein in the blood called gamma globulin.
Heart imaging techniques like echocardiography, cardiac MRI, cardiac CT, PET scans, and coronary angiography can aid in evaluating a patient’s condition. Usually, these non-invasive imaging techniques offer enough information about the tumor, like its type and location, and whether surgery is necessary.
1) Echocardiography is frequently the first choice for diagnosis. It can provide information about the size, location, way of attaching, and mobility of any masses in the heart, as well as if the tumor obstructs circulation or causes clots. Transesophageal echocardiography, which involves inserting a probe down a patient’s throat, is superior to transthoracic echocardiography, which scans the heart through the chest wall, in detailing heart masses. Nevertheless, the latter is usually the initial test done due to its simplicity and if a doctor suspects any heart problems.
2) Cardiac MRI is another top choice for diagnosis. It does not only provide detailed images of the anatomy but also gives insight into the microenvironment within a tumor. In situations where cardiac MRI is not available or not advisable, the use of cardiac CT is an effective alternative.
3) Though not commonly used, a PET scan can also help describe atrial myxomas, which are benign tumors in the upper chambers of the heart.
4) Coronary angiography is crucial in assessing the blood supply of the tumor. If patients need to have heart surgery to remove a tumor, coronary angiography is part of the standard pre-surgery assessment.
5) On a case-by-case basis, physicians determine the necessity for a transvenous biopsy, which is a procedure to take a small tissue sample from a vein. Usually, non-invasive imaging is enough to identify and describe a heart tumor. This procedure carries a risk of dislodging and mobilizing emboli, or abnormal particles in the bloodstream, associated with the tumor.
Treatment Options for Atrial Myxoma
When someone has an atrial myxoma, which is a type of heart tumor, immediate surgical removal is recommended. This is particularly important to prevent serious heart-related complications and the risk of the tumor spreading to other parts of the body. To fix any defects that can’t be addressed with the primary closure of the heart, a patch made from a tissue lining of the heart (pericardium) may be used.
After removing the tumor, it’s sent to a lab for testing to make sure there aren’t other dangerous types of tumors or signs that the cancer has spread elsewhere. If the atrial myxoma comes back repeatedly, there is a potential for a treatment called cardiac autotransplantation that doctors might consider.
What else can Atrial Myxoma be?
When doctors are diagnosing atrial myxomas, which is a type of heart tumor, they must also consider whether a patient instead has a condition known as mural thrombi with myxoid stroma. This condition resembles atrial myxomas quite closely when doctors examine tissue samples under the microscope, which can make it a tough call. In such situations, a special test called the calretinin marker can help doctors tell the two apart, as this marker is specifically found in myxomas.
Besides this, there are other types of malignant or cancerous tumors, such as primary sarcoma, primary cardiac lymphoma, and large B-cell lymphoma, that can also deceive doctors into thinking they’re dealing with an atrial myxoma. Therefore, doctors have to consider these conditions in their diagnosis too.
What to expect with Atrial Myxoma
The outlook for patients having surgery to remove atrial myxomas, which are tumours of the heart, is very good. The rate of deaths from the operation is under 5 percent, with patients recovering quickly after the procedure. There is only a 1% to 3% chance of the tumor coming back in random cases, a 12% chance in cases that run in families, and a 22% chance in more complicated cases. One study suggests that to prevent the tumor from coming back, it’s crucial that the surgeon is careful in operating the tumor, removes it completely with enough margin, and inspects all parts of the heart carefully.
Possible Complications When Diagnosed with Atrial Myxoma
Heart failure caused by overworking of the heart can be a result of Atrial myxomas, a type of heart tumor. Diuretics, medicines that help reduce fluid buildup, are often used to alleviate symptoms. Other typical treatments for heart failure, like beta-blockers and angiotensin-converting enzyme inhibitors, are usually not needed because the left ventricular function (the heart’s ability to pump blood) is often still intact.
Unusual heart rhythm or cardiac arrhythmia can occur due to the tumor itself or after its surgical removal. Also, valve defects such as mitral and tricuspid regurgitation or stenosis can happen due to atrial myxomas.
Atrial myxomas can also lead to the formation of blood clots. Patients with these tumors can experience a stroke as a result of these blood clots traveling to the brain. When this happens, treatments to prevent further clotting, such as anticoagulant or antiplatelet drugs, are needed. Moreover, cerebral aneurysm and myxomatous metastasis are some delayed brain-related complications and can look similar to infectious inflammation of the heart’s inner lining or inflammation of the blood vessels. Pulmonary embolism, which is a sudden blockage in a lung artery, can occur when the tumour is in the right atrium of the heart. Anticoagulation treatment or removal of the blood clot is required for severe cases. Further, pulmonary artery aneurysm, the bulging or ballooning of the wall of the lung artery, is also a delayed complication of atrial myxomas.
Atrial myxomas can be a source of infection as well. Patients may experience symptoms similar to those of infectious endocarditis, sepsis, or a condition that causes abnormal blood clotting throughout the body (Disseminated Intravascular Coagulation). Those particularly at risk are individuals who undergo dental or invasive procedures, and those with weakened immune systems. Common bacteria causing this infection include Streptococcus viridans, staphylococci, and Enterococcus faecalis. It is compulsory to undergo extensive antibiotic treatment and surgical removal of the heart tumor to survive.
Preventing Atrial Myxoma
If a person is diagnosed with atrial myxoma, it’s essential that they understand how the condition works and what complications can occur. They should be taught to look out for signs that require urgent medical attention, including:
- A feeling of weakness or numbness, particularly on one side of the body, which could signal a stroke.
- Chest pain, difficulty catching breath, or swelling of the legs, which may indicate congestive heart failure or a blood clot in the lungs.
- Symptoms like fever, chills, sweating at night, or unusual changes to the skin, which could mean an infection of the heart lining.
People need to know that the recommended treatment for atrial myxoma is to surgically remove the tumor, and this generally has very good results. It’s necessary for their main healthcare team to assist them in forming a multi-specialist team to manage their treatment effectively.
