45
7.8.1. Diagnosis of Cardiac Amyloidosis
COR LOE
Recommendations
1 B-NR 1. Patients for whom there is a clinical suspicion for cardiac
amyloidosis* should have screening for serum and
urine monoclonal light chains with serum and urine
immunofixation electrophoresis and serum free light chains.
1 B-NR 2. In patients with high clinical suspicion for cardiac
amyloidosis, without evidence of serum or urine monoclonal
light chains, bone scintigraphy should be performed to
confirm the presence of transthyretin cardiac amyloidosis.
1 B-NR 3. In patients for whom a diagnosis of transthyretin cardiac
amyloidosis is made, genetic testing with TTR gene
sequencing is recommended to differentiate hereditary variant
from wild-type transthyretin cardiac amyloidosis.
* LV wall thickness ≥14 mm in conjunction with fatigue, dyspnea, or edema, especially in
the context of discordance between wall thickness on echocardiogram and QRS voltage on
ECG, and in the context of aortic stenosis, HFpEF, carpal tunnel syndrome, spinal stenosis,
and autonomic or sensory polyneuropathy.
7.8. Cardiac Amyloidosis
7.8.2. Treatment of Cardiac Amyloidosis
COR LOE
Recommendations
1 B-R 1. In select patients with wild-type or variant transthyretin
cardiac amyloidosis and NYHA class I to III HF symptoms,
transthyretin tetramer stabilizer therapy (tafamidis) is
indicated to reduce cardiovascular morbidity and mortality.
Value Statement:
Low Value (B-NR)
2. At 2020 list prices, tafamidis provides low economic value
(>$180,000 per QALY gained) in patients with HF with
wild-type or variant transthyretin cardiac amyloidosis.
2a C-LD 3. In patients with cardiac amyloidosis and AF, anticoagulation
is reasonable to reduce the risk of stroke regardless of the
CHA
2
DS
2
-VASc (congestive heart failure, hypertension,
age ≥75 years, diabetes mellitus, stroke or transient ischemic
attack [TIA], vascular disease, age 65 to 74 years, sex category)
score.
