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基因分型和组织学验证的淀粉样心肌病的心脏磁共振双心室应变成像

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

作者信息

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

机构信息

Department of Medicine, Division of Cardiology, Jacob's School of Medicine and Biomedical Sciences, Buffalo, NY 14203, USA.

Department of Radiology, Division of Nuclear Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA.

出版信息

Cardiogenetics. 2021 Sep;11(3):98-110. doi: 10.3390/cardiogenetics11030011. Epub 2021 Jun 30.

Abstract

UNLABELLED

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.

摘要

未标注

心脏淀粉样变性(CA)是一种常见且可能致命的浸润性心肌病。对比增强心脏磁共振成像(CMR)用作诊断工具。然而,CMR用于双心室应变和应变率综合分析的效用尚未像超声心动图那样得到广泛报道。此外,此前尚未描述过使用CMR进行右心室应变分析。

目的

我们试图研究经心内膜心肌活检(EMB)证实且已进行基因分型的CA病例的双心室应变和应变率的整体及局部指标。

方法

基于组织学从2012年至2019年建立了一个包含80例EMB的数据库。总共19例CA阳性的EMB进行了进一步的组织学特征分析,并与4个正常活检标本进行比较。样本进行了ATTR或AL亚型的基因分型。5例患者在间隔72小时分别进行了超声心动图和对比增强CMR检查,对其双心室应变和应变率进行了综合分析。

结果

组织学证实所选样本确实为心脏淀粉样变性阳性。超声心动图显示左心室(LV)整体和局部纵向应变指标降低。对LV进行组织特征分析的CMR显示,圆周、径向和纵向应变及应变率整体降低,与超声心动图结果总体趋势一致。右心室(RV)基底节段圆周应变降低,纵向应变无变化。

结论

除了基于对比剂清除动力学提供CA的临床诊断外,CMR还可以成为准确评估涉及LV和RV的应变及应变率的整体和局部变化的有效功能评估工具。有必要进行进一步研究以验证和完善CMR在CA中的应变成像能力。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fb8/8318353/92fb859d2aac/nihms-1722379-f0001.jpg

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