Cardiac myxoma

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 with activity
• Platypnea – Difficulty breathing in the upright position with relief in the supine position
• Paroxysmal nocturnal dyspnea – Breathing difficulty when asleep
• Dizziness
• Fainting
• Palpitations – 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. General symptoms may also be present, such as:

• Cough
• Pulmonary edema – as blood backs up into the pulmonary artery, after increased pressures in the left atrium and atrial dilation
• Hemoptysis
• Fever
• Cachexia – Involuntary weight loss
• General discomfort (malaise)
• Joint pain
• Blue discoloration of the skin, especially the fingers change color upon pressure, cold, or stress (Raynaud's phenomenon)
• Clubbing – Curvature of nails accompanied with soft tissue enlargement of the fingers
• Swelling – any part of the body
• Presystolic heart murmur

These general symptoms may also mimic those of infective endocarditis.

Complications

• Arrhythmias
• Pulmonary edema
• Peripheral emboli
• Spread (metastasis) 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, schwannomas and epithelioid blue nevi. 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 mitral stenosis may be heard. These sounds may change when the patient changes position.{{cite web |url=https://www.lecturio.com/concepts/cardiac-myxoma/ | title= Cardiac Myxoma

Right atrial myxomata rarely produce symptoms until they have grown to be at least 13 cm (about 5 inches) wide.

Tests may include:{{cite web |url=https://www.lecturio.com/concepts/cardiac-myxoma/ | title= Cardiac Myxoma

• Echocardiogram and Doppler study
• Chest x-ray
• CT scan of chest
• Heart MRI
• Left heart angiography
• Right heart angiography
• ECG—may show atrial fibrillation

Blood tests:

• Blood tests: An FBC may show anemia and increased WBCs (white blood cells). The erythrocyte sedimentation rate (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. 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 (tumor cells breaking off and traveling with the bloodstream). Myxoma fragments can move to the brain, 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 mitral stenosis or mitral regurgitation. This may require emergency surgery to prevent sudden death.

References

References

1. Hecht, Sisalee M.. (2009-10-27). "A Review of: "Current Diagnosis & Treatment: Cardiology. 3rd ed. Crawford, Michael H., ed."". Medical Reference Services Quarterly.
2. (November 2020). "Cardiac Myxoma: Review and Update of Contemporary Immunohistochemical Markers and Molecular Pathology". Advances in Anatomic Pathology.
3. 0781770122
4. (Feb 2008). "Benign cardiac tumors of the pluripotent mesenchyme.". Semin Diagn Pathol.
5. Masters, Barry R.. (2012-05-25). "Harrisons's Principles of Internal Medicine, 18th Edition, two volumes and DVD. Eds: Dan L. Longo, Anthony S. Fauci, Dennis L. Kasper, Stephen L. Hauser, J. Larry Jameson and Joseph Loscalzo, ISBN 9780071748896 McGraw Hill". Graefe's Archive for Clinical and Experimental Ophthalmology.
6. (2016). "Cardiac Myxoma". Autopsy & Case Reports.
7. (30 December 2014). "UOTW #31 - Ultrasound of the Week".
8. (2008). "Atrial myxoma: Trends in management". International Journal of Health Sciences.
9. (2012). "Risk factors for postoperative recurrence of cardiac myxoma and the clinical managements: A report of 5 cases in one center and review of literature". Chinese Medical Journal.
10. (2015). "Cardiac Myxomas: A 50-Year Experience with Resection and Analysis of Risk Factors for Recurrence". The Annals of Thoracic Surgery.
11. "A Biatrial Myxoma with Triple Ripples".