Biventricular Strain Imaging with Cardiac MRI in Genotyped and Histology Validated Amyloid Cardiomyopathy.

Leyi Jiang,Badri Karthikeyan,Abhinay Reddy,Ram Amuthan,Umesh C. Sharma,Vasvi Singh,Saraswati Pokharel,Silva Kristo,Sharma Kattel

doi:10.3390/cardiogenetics11030011

Leyi Jiang, Badri Karthikeyan

Open Access

https://doi.org/10.3390/cardiogenetics11030011

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Journal: Cardiogenetics	Publication Date: Jun 30, 2021
Citations: 5	License type: CC BY 4.0

Affiliation: Roswell Park Cancer Institute, Yale University

Abstract

Cardiac amyloidosis (CA) is a common and potentially fatal infiltrative cardiomyopathy. Contrast-enhanced cardiac MRI (CMR) is used as a diagnostic tool. However, utility of CMR for the comprehensive analysis of biventricular strains and strain rates is not reported as extensively as echocardiography. In addition, RV strain analysis using CMR has not been described previously.Objectives:We sought to study the global and regional indices of biventricular strain and strain rates in endomyocardial biopsy (EMB)-proven, genotyped cases of CA.Methods:A database of 80 EMBs was curated from 2012 to 2019 based on histology. A total of 19 EMBs positive for CA were subjected to further tissue-characterization with histology, and compared with four normal biopsy specimens. Samples were genotyped for ATTR- or AL-subtypes. Five patients, with both echocardiography and contrast-enhanced CMR performed 72-h apart, were subjected to comprehensive analysis of biventricular strain and strain-rates.Results:Histology confirmed that the selected samples were indeed positive for cardiac amyloid. Echocardiography showed reduced global and regional left-ventricular (LV) longitudinal strain indices. CMR with tissue-characterization of LV showed global reductions in circumferential, radial and longitudinal strains and strain-rates, following a general trend with the echocardiographic findings. The basal right-ventricular (RV) segments had reduced circumferential strains with no changes in longitudinal strain.Conclusions:In addition to providing a clinical diagnosis of CA based on contrast clearance-dynamics, CMR can be a potent tool for accurate functional assessment of global and regional changes in strain and strain-rates involving both LV and RV. Further studies are warranted to validate and curate the strain imaging capacity of CMR in CA.

Highlights

Cardiac amyloidosis (CA) presents in two distinct forms: ATTR amyloidosis, which is due to deposition of transthyretin in myocardium, and AL amyloidosis, which is a plasma cell dyscrasia distinct from multiple myeloma [2,3]
We aimed to demonstrate the feasibility of biventricular strain imaging using cardiac MRI (CMR) in endomyocardial biopsy (EMB) proven cardiac amyloid, illustrate the parallels of CMR strain imaging with echocardiographic strain imaging, and provide an initial foray into CMR based evaluation of right ventricular strain [13]
The global longitudinal strain (GLS) of the Left Ventricular (LV) with cardiac amyloidosis was −7.96 ± 1.45% when compared to the GLS of a LV with normal group, which was −19.44 ± 0.33% (p < 0.01)

Summary

Introduction

CA presents in two distinct forms: ATTR amyloidosis, which is due to deposition of transthyretin in myocardium, and AL amyloidosis, which is a plasma cell dyscrasia distinct from multiple myeloma [2,3]

Objectives

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Results

Conclusion