Atrial Myxoma 

  • Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Park W Willis IV, MD   more...
 
Updated: Aug 30, 2011
 

Background

Atrial myxomas are the most common primary heart tumors. Because of nonspecific symptoms, early diagnosis may be a challenge. Left atrial myxoma may or may not produce characteristic findings on auscultation. Two-dimensional echocardiography is the diagnostic procedure of choice. Most atrial myxomas are benign and can be removed by surgical resection.

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Pathophysiology

Myxomas account for 40-50% of primary cardiac tumors. Approximately 90% are solitary and pedunculated, and 75-85% occur in the left atrial cavity. Up to 25% of cases are found in the right atrium. Most cases are sporadic. Approximately 10% are familial and are transmitted in an autosomal dominant mode. Multiple tumors occur in approximately 50% of familial cases and are more frequently located in the ventricle (13% vs 2% in sporadic cases).

Myxomas are polypoid, round, or oval. They are gelatinous with a smooth or lobulated surface and usually are white, yellowish, or brown. The most common site of attachment is at the border of the fossa ovalis in the left atrium, although myxomas can also originate from the posterior atrial wall, the anterior atrial wall, or the atrial appendage. The mobility of the tumor depends upon the extent of attachment to the interatrial septum and the length of the stalk.

Although atrial myxomas are typically benign, local recurrence due to inadequate resection or malignant change has been reported. Occasionally, atrial myxomas recur at a distant site because of intravascular tumor embolization. The risk of recurrence is higher in the familial myxoma syndrome.[1]

Symptoms are produced by mechanical interference with cardiac function or embolization. Being intravascular and friable, myxomas account for most cases of tumor embolism. Embolism occurs in about 30-40% of patients. The site of embolism is dependent upon the location (left or right atrium) and the presence of an intracardiac shunt.

Jong-Won Ha and associates reported a more frequent occurrence of systemic embolism in polypoid tumors as compared to round (58% vs 0%).[2] Also, polypoid tumors more frequently prolapse into the ventricle. Prolapse of a tumor through the mitral or tricuspid valve may result in the destruction of the annulus or valve leaflets. In one study, 19% of the patients had atrial fibrillation associated with large atrial myxoma. Left atrial myxomas produce symptoms when they reach about 70 g. Right atrial myxomas grow to approximately twice this size before becoming symptomatic. Tumors vary widely in size, ranging from 1-15 cm in diameter. Rate of growth is not exactly known. In one case report, right atrial myxoma had a growth rate of 1.36 X 0.03 cm/mo. The myxomas are vascular tumors and may be neovascularized by a branch of a coronary artery.[3] Recently, a case of hemorrhage in a left atrial myxoma was reported.[4]

Myxomas have been demonstrated to produce numerous growth factors and cytokines, including vascular endothelial growth factor, resulting in angiogenesis and tumor growth and an increased expression of the inflammatory cytokine, interleukin-6.[5, 6, 7]

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Epidemiology

Frequency

United States

Based upon the data of 22 large autopsy series, the prevalence of primary cardiac tumors is approximately 0.02% (200 tumors per million autopsies). About 75% of primary tumors are benign, and 50% of benign tumors are myxomas, resulting in 75 cases of myxoma per million autopsies.

International

Surgical incidence in the Republic of Ireland from 1977-1991 was 0.50 atrial myxomas per million population per year.

Mortality/Morbidity

  • Sudden death may occur in 15% patients with atrial myxoma. Death is typically caused by coronary or systemic embolization or by obstruction of blood flow at the mitral or tricuspid valve.
  • Morbidity is related to symptoms produced by tumor embolism, heart failure, mechanical valvular obstruction, and various constitutional symptoms.

Sex

  • Approximately 75% of sporadic myxomas occur in females.
  • Female sex predominance is less pronounced in familial atrial myxomas.

Age

  • Myxomas have been reported in patients aged 3-83 years.
  • The mean age for sporadic cases is 56 years. In a retrospective review of 171 patients from India, the mean age of presentation was 37.1 years. Most of these patients were symptomatic; dyspnea was the most common symptom.[8]
  • The mean age for familial cases is 25 years.
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Contributor Information and Disclosures
Author

Gyanendra K Sharma, MD, FACC, FASE  Professor, Department of Medicine, Section of Cardiology, Medical College of Georgia, Georgia Health Sciences University

Gyanendra K Sharma, MD, FACC, FASE is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, Society for Cardiovascular Magnetic Resonance, and Society of Cardiovascular Computed Tomography

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert E Fowles, MD  Clinical Professor of Medicine, University of Utah College of Medicine; Consulting Staff, Intermountain Medical Center and LDS Hospital; Director and Consulting Staff, Department of Cardiology, Salt Lake Clinic

Robert E Fowles, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, and American Heart Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Steven J Compton, MD, FACC, FACP  Director of Cardiac Electrophysiology, Alaska Heart Institute, Providence and Alaska Regional Hospitals

Steven J Compton, MD, FACC, FACP is a member of the following medical societies: Alaska State Medical Association, American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, and Heart Rhythm Society

Disclosure: Nothing to disclose.

Amer Suleman, MD  Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

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