A myxoma is a rare

benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

of the

heart The heart is a muscular organ in most animals. This organ pumps blood through the blood vessels of the circulatory system. The pumped blood carries oxygen and nutrients to the body, while carrying metabolic waste such as carbon dioxide to t ...

. Myxomata are the most common primary cardiac tumor in adults, and are most commonly found within the left atrium near the valve of the fossa ovalis. Myxomata may also develop in the other heart chambers. The tumor is derived from multipotent

mesenchymal Mesenchyme () is a type of loosely organized animal embryonic connective tissue of undifferentiated cells that give rise to most tissues, such as skin, blood or bone. The interactions between mesenchyme and epithelium help to form nearly every ...

cells. Cardiac myxoma can affect adults between 30 and 60 years of age.

Signs and symptoms

Symptoms may occur at any time, but most often they accompany a change of body position. Pedunculated myxomata can have a "wrecking ball effect", as they lead to stasis and may eventually embolize themselves. Symptoms may include: *

Shortness of breath Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing di ...

with activity *

Platypnea Platypnea or platypnoea is shortness of breath (dyspnea) that is relieved when lying down, and worsens when sitting or standing upright. It is the opposite of orthopnea. The condition was first described in 1949 and named in 1969. A related cond ...

– Difficulty breathing in the upright position with relief in the supine position *

Paroxysmal nocturnal dyspnea Paroxysmal nocturnal dyspnea or paroxysmal nocturnal dyspnoea (PND) is an attack of severe shortness of breath and coughing that generally occurs at night. It usually awakens the person from sleep, and may be quite frightening. PND, as well as simp ...

– Breathing difficulty when asleep *

Dizziness Dizziness is an imprecise term that can refer to a sense of disorientation in space, vertigo, or lightheadedness. It can also refer to disequilibrium or a non-specific feeling, such as giddiness or foolishness. Dizziness is a common medical c ...

Fainting Syncope, commonly known as fainting, or passing out, is a loss of consciousness and muscle strength characterized by a fast onset, short duration, and spontaneous recovery. It is caused by a decrease in blood flow to the brain, typically from ...

Palpitation Palpitations are perceived abnormalities of the heartbeat characterized by awareness of cardiac muscle contractions in the chest, which is further characterized by the hard, fast and/or irregular beatings of the heart. Symptoms include a rapi ...

s – Sensation of feeling your heart beat * Chest pain or tightness * Sudden Death (In which case the disease is an autopsy finding) The symptoms and signs of left atrial myxomata often mimic

mitral stenosis Mitral stenosis is a valvular heart disease characterized by the narrowing of the opening of the mitral valve of the heart. It is almost always caused by rheumatic valvular heart disease. Normally, the mitral valve is about 5 cm2 during d ...

. General symptoms may also be present, such as: *

Cough A cough is a sudden expulsion of air through the large breathing passages that can help clear them of fluids, irritants, foreign particles and microbes. As a protective reflex, coughing can be repetitive with the cough reflex following three ph ...

Pulmonary edema Pulmonary edema, also known as pulmonary congestion, is excessive liquid accumulation in the tissue and air spaces (usually alveoli) of the lungs. It leads to impaired gas exchange and may cause hypoxemia and respiratory failure. It is due t ...

– as blood backs up into the pulmonary artery, after increased pressures in the left atrium and atrial dilation *

Hemoptysis Hemoptysis is the coughing up of blood or blood-stained mucus from the bronchi, larynx, trachea, or lungs. In other words, it is the airway bleeding. This can occur with lung cancer, infections such as tuberculosis, bronchitis, or pneumonia, a ...

Fever Fever, also referred to as pyrexia, is defined as having a temperature above the normal range due to an increase in the body's temperature set point. There is not a single agreed-upon upper limit for normal temperature with sources using val ...

Cachexia Cachexia () is a complex syndrome associated with an underlying illness, causing ongoing muscle loss that is not entirely reversed with nutritional supplementation. A range of diseases can cause cachexia, most commonly cancer, congestive heart ...

– Involuntary weight loss * General discomfort ( malaise) *

Joint pain Arthralgia (from Greek ''arthro-'', joint + ''-algos'', pain) literally means ''joint pain''. Specifically, arthralgia is a symptom of injury, infection, illness (in particular arthritis), or an allergic reaction to medication. According to MeSH, ...

* Blue discoloration of the skin, especially the fingers change color upon pressure, cold, or stress (

Raynaud's phenomenon Raynaud syndrome, also known as Raynaud's phenomenon, eponymously named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862, is a medical condition in which the spasm of small arteries cau ...

) * Clubbing – Curvature of nails accompanied with soft tissue enlargement of the fingers * Swelling – any part of the body * Presystolic heart murmurEric J. Topol
The Topol Solution: Textbook of Cardiovascular Medicine
Third Edition with DVD, Plus Integrated Content Website, Volume 355. Lippincott Williams & Wilkins, Oct 19, 2006; page 223. These general symptoms may also mimic those of

infective endocarditis Infective endocarditis is an infection of the inner surface of the heart, usually the valves. Signs and symptoms may include fever, small areas of bleeding into the skin, heart murmur, feeling tired, and low red blood cell count. Complications ...

Complications

Arrhythmias Arrhythmias, also known as cardiac arrhythmias, heart arrhythmias, or dysrhythmias, are irregularities in the heartbeat, including when it is too fast or too slow. A resting heart rate that is too fast – above 100 beats per minute in adults ...

* Peripheral emboli * Spread (

metastasis Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then ...

) of the tumor * Blockage of the mitral heart valve * Stroke * Fusiform cerebral aneurysms

Causes

Myxomata are the most common type of adult primary heart tumor. Most myxomata arise sporadically (90%), and only about 10% are thought to arise due to inheritance. About 10% of myxomata are inherited, as in Carney syndrome. Such tumors are called familial myxomata. 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 myxomata. Other abnormalities are observed in people with Carney syndrome include skin myxomata, pigmentation, endocrine hyperactivity,

schwannoma A schwannoma (or neurilemmoma) is a usually benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves. Schwannomas are homogeneous tumors, consisting only of Schwann cells ...

s and epithelioid blue

nevi Nevus (plural nevi) is a nonspecific medical term for a visible, circumscribed, chronic lesion of the skin or mucosa. The term originates from ''nævus'', which is Latin for "birthmark"; however, a nevus can be either congenital (present at bir ...

. Myxomata are more common in women than men.

Diagnosis

A doctor will listen to the heart with a stethoscope. A "tumor plop" (a sound related to movement of the tumor), abnormal heart sounds, or a murmur similar to the mid-diastolic rumble of

may be heard. These sounds may change when the patient changes position. Right atrial myxomata rarely produce symptoms until they have grown to be at least 13 cm (about 5 inches) wide. Tests may include: *

Echocardiogram An echocardiography, echocardiogram, cardiac echo or simply an echo, is an ultrasound of the heart. It is a type of medical imaging of the heart, using standard ultrasound or Doppler ultrasound. Echocardiography has become routinely used in th ...

and Doppler study * Chest

x-ray An X-ray, or, much less commonly, X-radiation, is a penetrating form of high-energy electromagnetic radiation. Most X-rays have a wavelength ranging from 10 picometers to 10 nanometers, corresponding to frequencies in the range 30&nb ...

* CT scan of chest * Heart

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...

* Left heart

angiography Angiography or arteriography is a medical imaging technique used to visualize the inside, or lumen, of blood vessels and organs of the body, with particular interest in the arteries, veins, and the heart chambers. Modern angiography is perfor ...

* Right heart angiography *

ECG Electrocardiography is the process of producing an electrocardiogram (ECG or EKG), a recording of the heart's electrical activity. It is an electrogram of the heart which is a graph of voltage versus time of the electrical activity of the hear ...

—may show atrial fibrillation Blood tests: * Blood tests: An FBC may show anemia and increased WBCs (white blood cells). The

erythrocyte sedimentation rate The erythrocyte sedimentation rate (ESR or sed rate) is the rate at which red blood cells in anticoagulated whole blood descend in a standardized tube over a period of one hour. It is a common hematology test, and is a non-specific measure of ...

(ESR) is usually increased. * Blood tests: An FBC may show anemia and increased WBCs (white blood cells). The

(ESR) is usually increased. File:Atrial myxoma gsr.gif, Echocardiogram of atrial myxoma File:UOTW 31 - Ultrasound of the Week 1.webm, Echocardiogram showing atrial myxoma File:UOTW 31 - Ultrasound of the Week 2.jpg, Echocardiogram showing atrial myxoma Image:Atrial myxoma edge low mag.jpg, Atrial myxoma and myocardium. H&E stain. Image:Atrial myxoma low mag.jpg, Atrial myxoma. H&E stain. Image:Atrial myxoma intermed mag.jpg, Atrial myxoma. H&E stain. Image:Atrial myxoma edge high mag.jpg, Atrial myxoma covered by

endothelium The endothelium is a single layer of squamous endothelial cells that line the interior surface of blood vessels and lymphatic vessels. The endothelium forms an interface between circulating blood or lymph in the lumen and the rest of the vesse ...

. H&E stain.

Treatment

The surgery is treatment of choice, tumor must be surgically removed. Some patients will also need their mitral valve replaced. This can be done during the same surgery. Usually, inadequate excision of the tumor, development from a secondary focus, or intracardiac implantation from the primary tumor are the attributable explanation for recurrence, and it is more likely to occur in the first 10 postoperative years, especially in younger patients.

Prognosis

Although a myxoma is not malignant with risk of metastasis, complications are common. Untreated, a myxoma can lead to an

embolism An embolism is the lodging of an embolus, a blockage-causing piece of material, inside a blood vessel. The embolus may be a blood clot (thrombus), a fat globule (fat embolism), a bubble of air or other gas ( gas embolism), amniotic fluid (am ...

(tumor cells breaking off and traveling with the bloodstream). Myxoma fragments can move to the

brain A brain is an organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It is located in the head, usually close to the sensory organs for senses such as vision. It is the most complex organ in a ve ...

, eye, or limbs. If the tumor continues to enlarge inside the heart, it can block blood flow through the mitral valve and cause symptoms of

or mitral regurgitation. This may require emergency surgery to prevent sudden death.

References

External links

{{DEFAULTSORT:Atrial Myxoma Heart neoplasia