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Atrial Myxoma Clinical Presentation

  • Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Park W Willis IV, MD  more...
 
Updated: Dec 07, 2015
 

History

Symptoms range from nonspecific and constitutional to sudden cardiac death. In about 20% of cases, myxoma may be asymptomatic and discovered as an incidental finding. Signs and symptoms of mitral stenosis, endocarditis, mitral regurgitation, and collagen vascular disease can simulate those of atrial myxoma. A high index of suspicion aids in diagnosis.

Symptoms of left-sided heart failure include the following:

  • Dyspnea on exertion (75%) that may progress to orthopnea, paroxysmal nocturnal dyspnea, and pulmonary edema is observed. [14, 15]
  • Symptoms are caused by obstruction at the mitral valve orifice. Valve damage may result in mitral regurgitation.

Symptoms of right-sided heart failure include the following:

  • Patients experience fatigue and peripheral edema.
  • Abdominal distension due to ascites is rare; however, it is more common in slowly growing right-sided tumors. [16]
  • These symptoms are also observed in the later stage of progressive heart failure associated with left atrial myxomas.

Severe dizziness/syncope is experienced by approximately 20% of patients. The most frequent cause in patients with left atrial myxomas is obstruction of the mitral valve. Symptoms may change as the patient changes positions.

Symptoms related to embolization include the following:

  • Systemic or pulmonary embolization may occur from left- or right-sided tumors.
  • Left-sided symptoms are produced from the infarction or hemorrhage of viscera.
  • Embolization to the central nervous system may result in transient ischemic attack, stroke, or seizure. In an analysis of 113 cases of atrial myxoma with neurologic presentation, 83% of patients presented with ischemic stroke, most often in multiple sites (43%). Twelve percent of patients presented with seizures. In a retrospective review of 74 patients with atrial myxoma, 12% had neurologic manifestations. [17] Cerebral infarction was present in 89% of the cases and most myxomas (89%) demonstrated a mobile component on transesophageal echocardiography. Other complications include myxoma-induced cerebral aneurysm and myxomatous metastasis that can mimic vasculitis or endocarditis. [18]
  • Involvement of the retinal arteries may result in vision loss.
  • Systemic embolization that causes occlusion of any artery, including coronary, aortic, renal, visceral, or peripheral, may result in infarction or ischemia of the corresponding organ.
  • On the right side, embolization results in pulmonary embolism and infarction.
  • Multiple, recurrent small emboli may result in pulmonary hypertension and cor pulmonale.
  • Presence of an intracardiac shunt (atrial septal defect or patent foramen ovale) may result in a paradoxical embolism.

Constitutional symptoms that include fever, weight loss, arthralgias, and Raynaud phenomenon are observed in 50% of patients. These symptoms may be related to overproduction of interleukin-6.

Hemoptysis due to pulmonary edema or infarction is observed in up to 15% of patients.

Chest pain is infrequent. If it occurs, it may be due to coronary embolization.

A case report of 5-year history of visual loss, vertigo, ataxia, tinnitus, and bone lesions that resolved after diagnosis and resection of the atrial myxoma has raised a possibility of it causing a paraneoplastic syndrome.[19]

Atrial myxoma can become infected when vegetations may be seen attached to its surface.[20]

Next

Physical Examination

Note the following:

  • Jugular venous pressure may be elevated, and a prominent A wave may be present.
  • A loud S 1 is caused by a delay in mitral valve closure due to the prolapse of the tumor into the mitral valve orifice (mimicking mitral stenosis).
  • P 2 may be delayed. Its intensity may be normal or increased, depending on the presence of pulmonary hypertension.
  • In many cases, an early diastolic sound, called a tumor plop, is heard. This sound is produced by the impact of the tumor against the endocardial wall or when its excursion is halted.
  • An S 3 or S 4 may be audible.
  • A diastolic atrial rumble may be heard if the tumor is obstructing the mitral valve.
  • If there is valve damage from the tumor, mitral regurgitation may cause a systolic murmur at the apex.
  • A right atrial tumor may cause a diastolic rumble or holosystolic murmur due to tricuspid regurgitation.
  • General examination may reveal fever, cyanosis, digital clubbing, rash, or petechiae.

Patients with familial myxoma may have a variety of features called syndrome myxoma or Carney syndrome,[21]  as follows:

  • Myxomas in breast, skin, thyroid gland, or neural tissue
  • Spotty pigmentation such as lentigines (ie, flat brown discoloration of skin), pigmented nevi, or both
  • Endocrine hyperactivity such as Cushing syndrome
  • Multiple cerebral fusiform aneurysms may be seen in patients with Carney syndrome. [22]

Other described syndromes associated with atrial myxomas include the following:

  • NAME syndrome features nevi, atrial myxoma, myxoid neurofibroma, and ephelides (ie, freckles [tanned macules found on the skin]).
  • LAMB syndrome features lentigines, atrial myxoma, and blue nevi.
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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 Regents 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, Society of Cardiovascular Computed Tomography

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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

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

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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Transesophageal echocardiography.
 
 
 
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