Multimodality Imaging in Cardiac Amyloidosis: Diagnostic Criteria and Appropriate Utilization
Publication Date: June 30, 2021
Last Updated: March 14, 2022
Table 1. Expert Consensus Recommendations for Diagnosis of Cardiac Amyloidosis
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| Criteria for Diagnosis | Subtype |
| Histological Diagnosis of Cardiac Amyloidosis: Endomyocardial Biopsy* | |
| 1. Endomyocardial biopsy positive for cardiac amyloidosis with Congo red staining with apple-green birefringence under polarized light; typing by immunohistochemistry and/or mass spectrometry at specialized centers | AL, ATTR, Other subtypes |
| Histological Diagnosis of Cardiac Amyloidosis: Extracardiac Biopsy | |
| 1. ATTR cardiac amyloidosis is diagnosed when below criteria ATTR are met:
a. Extracardiac biopsy proven ATTR amyloidosis AND
b. Typical cardiac imaging features (as defined below) |
ATTR |
| 2. AL cardiac amyloidosis is diagnosed when below criteria are met:
a. Extracardiac biopsy proven AL amyloidosis AND
b. Typical cardiac imaging features (as defined below) OR c. Abnormal cardiac biomarkers: abnormal age-adjusted NT-pro BNP or abnormal Troponin T/I/Hs-Troponin with all other causes for these changes excluded. |
AL |
| Clinical Diagnosis of ATTR Cardiac Amyloidosis: 99mTc-PYP, DPD, HMDP | |
| 1. ATTR cardiac amyloidosis is diagnosed when below criteria ATTR are met:
a. 99mTc-PYP, DPD, HMDP Grade 2 or 3 myocardial uptake
of radiotracer AND b. Absence of a clonal plasma cell process as assessed by serum FLCs and serum and urine immunofixation AND c. Typical cardiac imaging features (as defined below) |
ATTR |
| Typical Imaging Features of Cardiac Amyloidosis | |
| Typical cardiac echo or CMR or PET features: ANY of the below imaging features with all other causes for these cardiac manifestations, including hyper-tension, reasonably excluded. | |
| 2. CMR
a. LV wall thickness >U LN for sex on SSFP cine CM R
b. Global ECV >0.40 c. Diffuse LG E d. Abnormal gadolinium kinetics typical for amyloidosis, myocardial nulling prior to blood pool nulling |
ATTR/AL |
| 3. PET: 18F-florbetapir or 18F-florbetaben PET
a. Target to background (LV myocardium to blood pool) ratio >1.5
b. Retention index >0.030 min·-1 |
ATTR/AL |
Recommendation Grading
Overview
Title
Multimodality Imaging in Cardiac Amyloidosis: Diagnostic Criteria and Appropriate Utilization
Authoring Organizations
American Heart Association
American Society of Echocardiography
American Society of Nuclear Cardiology
Heart Failure Society of America
Society of Nuclear Medicine and Molecular Imaging
Publication Month/Year
June 30, 2021
Last Updated Month/Year
April 1, 2024
Document Type
Consensus
External Publication Status
Published
Country of Publication
US
Inclusion Criteria
Female, Male, Adult, Older adult
Health Care Settings
Ambulatory, Hospital
Intended Users
Physician, nurse practitioner, nurse, physician assistant
Scope
Diagnosis, Management
Diseases/Conditions (MeSH)
D000686 - Amyloidosis
Keywords
diagnosis, cardiac amyloidosis, multimodality, AHA Scientific Statements, expert consensus, appropriate use