Amyloidosis

Question 1

Types of Amyloisis

Answer 1

Light chain Amyloisis (AL)
Wild type Transthyretin Amyloisis (wt-ATTR)
Hereditary - variant Transthyretin Amyloisis (v-ATTR)

Question 2

Echo changes in Amyloisis

Answer 2

Thickening of all heart chambers - - >
Increased cardiac stiffness
Diastolic heart failure
Arrhythmias

Question 3

Type of heart failure associated with wt ATTR

Answer 3

HFpEF

Question 4

Which drug groups Amyloidosis patients are intolerant to?

Answer 4

Beta blockers and ACE/ARB

Question 5

What is Transthyretin?

Answer 5

Tetramer protein produced in the liver, works as a transporter for vitamin A and Thyroid hormone

Question 6

ATTR Therapie

Answer 6

Neurology
TTR silencers - - >patisiran/inotersen
TTR depleters
Cardiology
TTR stabilizer - - > tafamidis/acoramidis

Question 7

Cause of wt ATTR

Answer 7

Misfolded Protein

Question 8

Cause of v ATTR

Answer 8

Mutation in the Transthyretin

Question 9

Ecg changes in cardiac amyloidosis

Answer 9

1 - low voltage
2 - LVH /sometimes mismatch with imaging
3 - Arrhythmia

Question 10

Echo changes

Answer 10

Bilatrial enlargement
Ventricular hypertrophy >= 14mm
Diastolic dysfunction
Abnormal longitudinal strain

Question 11

Confirm diagnosis

Answer 11

biopsy of affected organs

Question 12

Non invasive testing

Answer 12

ECG
Echo
MRI
Bone scintigraphy

Question 13

Red flags preceding Cardiac amyloidosis

Answer 13

Bilateral carpal tunnel syndrome
Rotator cuff tear
Biceps tendon tear
Lumbar spinal stenosis