Atrial Myxoma

I recently found out that I have a right atrial myxoma. In my case, it seems to cause mainly hematological and immunological alterations (deranged blood coagulation, GI bleeding, proliferation of  B -lymphocytes and vasvulitic-like symptoms/ dermatological signs and poor acral microcirculation) rather than classic heart symptoms. However, some echoes in the past did suggest increased RA pressure (dilated/congested proximal vena cava inf. And hepatic veins) - but at that time, the cause could not be figured out, and the finding was not constant, but recurred regularly.Due to above mentioned symptoms someone finally suggested doing a transesophageal echo and this clearly showed a small myxoma in the right atrium, nearby the entrance of the vena cava into the RA.Now, I have some questions (I havent had consultation with heart surgery yet):1. How long does surgery for myxoma take?2. Is there an increased risk for complications due to resternotomy? (i had a median sternotomy in the past for a mediastinal mass)3. How could the finding by transthoracic echo be explained when it did not show the tumor? 4. I am 26, and have already severe health issues unrelated to the myxoma. However, I read that cafe au lait spots and adrenal overactivity (manifest or subclinical) can go together - i do have 2big and 4 small cafe au lait spots and some unexplained subclinical adrenal issues. Could there be a connection?5. Have you ever had a patient with myxoma that presented with immunological/rheumatological symptoms? If yes, did surgery cure the symptoms?Thank you very much.Sarah
Hi Sarah,
I best answer you point by point:
  1. Several hours.
  2. A little, but an experienced surgeon should have no trouble getting back in, or even suggest a different approach, depending on the exact location - even with your information I would have to see the TransEsophageal Echo (TEE) for that kind of decision.

(The images show a transesophageal echocardiography probe in the esophagus, which is located behind the heart. Sound waves from the probe create high-quality pictures of the heart. B shows an echocardiogram of the heart's lower and upper chambers (ventricles and atrium, respectively).

  1. With a TransThoracic Echo (TTE) there "blind spots", one of the reasons to recommend a TEE. The increased RA pressure (dilated/congested proximal vena cava inferior and hepatic veins) are indications however, that something was out of order, hence the TEE. I don't know why this wasn't recommended earlier.
  2. I don't know. I am a cardiac surgeon!
  3. A myxoma is usually found on the left side of the heart and may involve other structures such a heart valve. As in your case, most often a myxoma causes a local obstruction, or problems with a valve function (either obstruction or leakage). Myxomas can have a broad base or just a narrow stalk and need to be completely resected for a cure. 
Because of the nature of the (otherwise benign) tumor, pieces may break off and get in the circulation (embolize) and cause a stroke as well as other complications. Myxomas are also occasionally associated with arrhythmias such as atrial fibrillation.
Most of the symptoms are related to complications due a local obstruction, valve malfunction or embolization to a different part of the body. However, myxomas have also been associated with some inflammatory diseases. Thus, symptoms can range from nonspecific to sudden cardiac death. A myxoma may be asymptomatic and discovered as an incidental finding. 

In summary, depending on where the myxoma is located it may cause the following symptoms:

  • Symptoms of left-sided heart failure:
    • Shortness of breath on exertion (75%) that may worsen with a change of position, and even pulmonary edema is observed.
    • Symptoms are caused by obstruction at the mitral valve orifice. Valve damage may result in mitral regurgitation.
    • Severe dizziness/syncope.
    • The most frequent cause in patients with left atrial myxomas is obstruction of the mitral valve.
    • Symptoms may change as the patient changes position.
  • Symptoms of right-sided heart failure:
    • Fatigue and peripheral edema.
  • Symptoms related to embolization:
    • Embolization to the brain may result in a stroke or seizure. A stroke often occurs in multiple sites.
    • Involvement of the arteries to your eyes 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.
      • Multiple, recurrent small lung emboli may result in pulmonary hypertension.
    • Presence of an atrial septal defect may result in embolization from the right side of the heart to the “left-sided circulation” and cause a stroke.
  • Constitutional symptoms that include fever, weight loss, arthralgias, and other inflammatory symptoms. I assume successful resection would alleviate these symptoms as well.

Hope this helps,

Dr T



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