Atrial Myxoma Clinical Presentation

Updated: Jun 20, 2019
  • Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Richard A Lange, MD, MBA  more...
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Presentation

History

Symptoms of atrial myxomas range from nonspecific and constitutional to sudden cardiac death. [19] In about 20% of cases, myxomas 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. [20, 21]

  • 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 distention due to ascites is rare; however, it is more common in slowly growing right-sided tumors. [22]

  • 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.

  • Embolization to the central nervous system may result in transient ischemic attack, stroke, or seizure. [23] 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. [24] 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. [25]

  • 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. [26]

Atrial myxoma can become infected when vegetations may be seen attached to its surface. [27]

Cardiac myxoma in pregnancy is rare, and diagnosis and management may be challenging as fatigue and dyspnea are not uncommon in normal pregnancy. Transthoracic echocardiography can aid in the diagnosis. In most cases, the diagnosis is made in the second trimester and surgical resection performed in the third trimester. Maternal survival is generally excellent. [28]

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Physical Examination

The following findings may be noted on physical evaluation of patients with atrial myxomas:

  • 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 or tricuspid 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 (due to obstruction of the tricuspid valve) 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 known as syndrome myxoma or Carney syndrome, [29]  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. [30]

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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