CCS Amyloid 2020 Flashcards
What are the three types of Amyloid?
- AL Amyloid (Primary or Secondary)
- Hereditary (hTTR)
- Wild type (wtTTR)
Name 5 extracardiac manifestations of amyloid?
Peripheral neuropathy
Macroglossia
Carpal tunnel syndrome
Orthostatic Hypotension
Gastroparesis
How to determine whether hATTR or wtATTR?
TTR genetic testing
Name 4 MRI features of Amyloid?
Diffuse Subendocardial LGE
Left atrial LGE
Elevated native T1 relaxation time
Shortened post contrast T1 relaxation time
Elevated ECV fraction
Two criteria for diagnoses on Pyrophosphate scan?
uptake up to or greater than rib (Grade 2)
uptake > 1.5x contralateral lung
Indication for Tafamadis?
TTR CMO with NYHA I-III symptoms
How high did BNP have to be in ATTRACT trial?
> 600
What to monitor in Amyloid patients?
Echo, BNP +/- CMR
What are 4 autonomic features of Amyloid?
- Orthostatic Hypotension
- Gastroparesis
- Sweating abnormalities
- Sexual Dysfunction
What is the difference between renal dysfunction in AL vs. TTR
AL - Nephrotic syndrome/Severe renal dysfunction
TTR - Milder form of renal dysfunction
What are ocular manifestations in Amyloidosis?
Vitreous Opacities
What are 4 MSK manifestations in Amyloidosis?
- Biceps tendon rupture
- Muscle weakness
- Carpal tunnel syndrome
- Spinal stenosis
Name 4 populations to screened for cardiac amyloidosis
- Unexplained wall thickness
- Age > 60 with LFLG AS with LVEF > 50%
- Unexplained dysautonomia and neuropathy
- Established amyloid/AL
- Bilateral carpal tunnel syndrome
Should patients with aTTR amyloid be gene tested?
Yes
When to do EMB in Amyloid?
-Equivocal Pyrophosphate scan or uncertainty in AL work up
What is the process of Amyloid production to deposition in the heart
TTR mRNA transcribed -> TTR tetramer -> degrades to Dimers -> degrades to Monomers -> Misfolded Amyloid monomers break down to fibrils -> deposit in nerves and myocardium
