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Clinical Article
Cardiac magnetic resonance T1 mapping manifestation of one patient with immunoglobin light-chain cardiac amyloidosis and literature review
ZHANG Min  CUI Ya-dong  LUO Xiao-jie  CHEN Min 

DOI:10.12015/issn.1674-8034.2017.08.003.


[Abstract] Objective: To discuss magnetic resonance T1 mapping of cardiac amyloidosis and its clinical features in order to achieve better understanding of this disease.Materials and Methods: A case from Beijing Hospital diagnosed with cardiac amyloidosis admitted was reported and the related literature was reviewed.Results: A 72 year-old man presented with chronic cardiac dysfunction, relapsing heart failure, poor self-care ability, paroxysmal nocturnal dyspnea and NYHA Grade III. Cardiac ultrasonography came to a diagnosis of non-obstructive hypertrophic cardiomyopathy. The patient was admitted to Beijing Hospital due to aggravating heart failure with left atrial thrombosis, pulmonary edema and bilateral plural effusion. Cardiac magnetic resonance (CMR) imaging revealed asymmetric hypertrophy in myocardium with ventricular interval predominant, and that left ventricular myocardial motion became weak with rapid chambers’ washout, dark blood pool in the chamber and diffused subendocardium enhancement in late gadolinium enhancement. T1 mapping was showing prolonged longitudinal relaxation time of myocardium and increased extracellular volume fraction. Biopsy of corpora linguae revealed thickened submucosal vessel wall and accumulation of homogeneous stained-pink amorphous substance with congo red positive combined with polarized light apple-green appearance on microscopy. The finding was consistent with cardiac amyloidosis.Conclusion: Cardiac amyloidosis usually manifests myocardial hypotrophy and left ventricular systolic dysfunction. CMR and the related advanced imaging techniques play an important role in diagnosis of cardiac amyloidosis.
[Keywords] Cardiomyopathies;Amyloidosis;Cardiac magnetic resonance;Immunoglobulin light chains;T1 mapping

ZHANG Min Graduate school of Peking Union Medical College, Beijing 100005, China; Department of Radiology, Beijing Hospital, Beijing 100730, China

CUI Ya-dong Graduate school of Peking Union Medical College, Beijing 100005, China

LUO Xiao-jie Graduate school of Peking Union Medical College, Beijing 100005, China

CHEN Min* Graduate school of Peking Union Medical College, Beijing 100005, China

*Correspondence to: Chen M, E-mail: cjr.chenmin@vip.163.com

Conflicts of interest   None.

ACKNOWLEDGMENTS  This work was part of National Key Specialty Construction of Clinical Project No. 2013-544
Received  2017-04-23
Accepted  2017-07-21
DOI: 10.12015/issn.1674-8034.2017.08.003
DOI:10.12015/issn.1674-8034.2017.08.003.

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