Cardiac amyloidosis – Cardiology MCQ

Pick the wrong statement:
a) Light chain cardiac amyloidosis has a better survival than transthyretin cardiac amyloid amyloidosis
b) Light chain cardiac amyloidosis responds to chemotherapy
c) Transthyretin cardiac amyloidosis is associated with sensorimotor polyneuropathy
d) Transthyretin cardiac amyloidosis can be detected by bone scintigraphy
Correct answer: a) Light chain cardiac amyloidosis has a better survival than transthyretin cardiac amyloid amyloidosis
Light chain amyloidosis with left ventricular systolic failure has median survival of 4 months while transthyretin amyloidosis has a median survival of 3-6 years [1].
Patients with less than 20% light chain amyloidosis load who respond to chemotherapy has been shown to have a better survival than those who does not respond. Light chain amyloidosis involves almost all organs, with predominant involvement being in the heart and kidney.
Transthyretin (TTR), the precursor protein in senile systemic amyloidosis and familial TTR-related amyloidosis is a tetrameric plasma transport protein synthesized in the liver.  TTR-derived fibrils show avidity for bone tracers, especially 99mTechnetium-3,3-diphosphono-1,2-propano-di-carboxylic acid (99mTc-DPD).
Reference

1. Kristen AV et al. Cardiac Amyloid Load. Prognostic and Predictive Biomarker in Patients With Light-Chain Amyloidosis. J Am Coll Cardiol. 2016;68(1):13-24.