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Multimodality Imaging in Cardiac Amyloidosis: Diagnostic Criteria and Appropriate Utilization

Publication Date: July 1, 2021

Last Updated: March 14, 2022

Table 1. Expert Consensus Recommendations for Diagnosis of Cardiac Amyloidosis

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

Overview

Title

Multimodality Imaging in Cardiac Amyloidosis: Diagnostic Criteria and Appropriate Utilization

Authoring Organizations