UMEM Educational Pearls

Title: Cardiac Amyloidosis

Posted: 10/20/2012 by Semhar Tewelde, MD (Emailed: 10/21/2012) (Updated: 10/21/2012)
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Cardiac amyloidosis can present along a spectrum from asymptomatic to severe CHF w/conduction abnormalities

ECG with low voltage + echocardiogram with thickened myocardium should heighten suspicion

Definitive Dx. is myocardial biopsy identifying the infiltrative lesion (MRI w/gad is also supportive)

AL (light chain) amyloidosis is an acquired disease from improperly functioning plasma cells

¨ Rapidly progressive and life threatening

¨ Tx. w/chemotherapeutic agents (+/- BMT)

Transthyretin-related (TTR) amyloidosis is produced by the liver (2 types)

Familial transthyretin-related amyloidosis (ATTR)

Senile systemic amyloidosis (SSA)

¨ Both are slowly progressive

¨ Tx liver transplant (ATTR) and supportive care (SSA)

Quarta C, Kruger J, Falk R. Cardiac Amyloidosis. Circulation. Sept 2012;126(2)178-182