Myocardial fatty foci (tuberous sclerosis)

Case contributed by Tarun Ramayya

Presentation

Patient has a history of tuberous sclerosis and end stage renal failure on dialysis. Patient underwent a transthoracic echocardiogram.

Patient Data

Age: 40
Gender: Male
Ultrasound

Transthoracic echocardiogram... can you spot the abnormality?

Abnormal hyperechogenicity at the left ventricle apex
 

CT

Can you spot the abnormality?

Multiple focal areas of fat densities within the left and right ventricular myocardium, specifically in the anterior wall, lateral wall, and apex on the left side and on the free wall and apex on the right side on cardiac-gated CTA.

http://thoracicrad.org/assets/index/2015/Case_of_the_Month/cardio/10-2015/index.htm

Case Discussion

Tuberous Sclerosis

Autosomal dominant neurocutaneous disorder associated with development of benign tumors of the embryonic ectoderm

Myocardial Fatty Foci (MFF)

  • MFF are benign fatty deposits in myocardial tissue and have been described as well-circumscribed lesions with no invasion, no mass effect, and no signs of enhancement on CT and MR
  • prior studies by Adriaensen et al. and Tresoldi et al. report a prevalence for MFF of 64% and 50% in TSC patients, respectively
  • MFF have been reported in interventricular septum, left ventricular wall, right ventricular wall and papillary muscles with shapes ranging from ovoid to linear
    • Clinical significance of MFF
      • the prevalence of MFF is higher in TSC patients when compared to non-TSC patients and may serve as a useful clinical finding in patients with undiagnosed TS
      • Tresoldi et al. have shown  a positive correlation between fatty foci number and degree of extra cardiac involvement; indicating that MFF may shed light on disease severity
      • on echocardiogram, MFF has been characterized by hyper-echogenicity and must be distinguished from Cardiac rhabdomyoma, which is also hyperechogenic on echocardiogram
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Case information

rID: 39836
Case created: 22nd Sep 2015
Last edited: 3rd Oct 2015
Systems: Chest, Cardiac
Inclusion in quiz mode: Included

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