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• Clinical Article •
Clinical and CMR manifestations in 4 cases with cardiac amyloidosis of middle-aged and elderly patients
ZHANG Min  LUO Xiao-jie  CUI Ya-dong  CHEN Min 

DOI:10.12015/issn.1674-8034.2017.10.006.


[Abstract] Objective: To retrospectively analyze the clinical data and cardiac magnetic resonance imaging features of 4 middle-aged and elderly cases with cardiac amyloidosis, in order to improve its diagnosis and treatment.Materials and Methods: The records of 4 patients who were diagnosed as cardiac amyloidosis by pathology in Beijing hospital from Aug 2010 to Dec 2016 were reviewed to identify the characteristics including age, gender, clinical manifestation, cardiac magnetic resonance imaging and pathological features, treatment and prognosis.Results: The mean age of patients was about 68 years (57y-72y). All 4 patients were with long course (9 months to 3 years) and found elevated serum NT-pro BNP and troponin with manifestation of varying degrees in systolic dysfunction of left ventricles. 3 patients presented arrhythmia. 2 cases were with low voltage and pathological q waves in ECG. And elevated light chain emerged in 3 patients' urine. Cardiac ultrasonography depicted thickened myocardium with ventricular interval predominant and came to a diagnosis of non-obstructive hypertrophic cardiomyopathy in 4 patients. However, cardiac magnetic resonance imaging (CMR) revealed asymmetric hypertrophy in left ventricles with increased myocardium mass and no chamber enlargement; Left ventricular myocardial motion became weak, and so were the systolic functions. Late Gadolinium Enhancement depicted diffused subendocardium or transmural enhancement, dark blood pool and myocardial nulling before blood pool. 4 cases turned out to be primary systemic amyloidosis light chain type (AL); 3 were proved by extro-myocardium biopsy and 1 by autopsy. Amyloidosis frequently involves kidneys, liver, intestines, peripheral nervous system and skin. 1 patient was treated with PD and MD chemo methods and maintains in good statue till now. 2 were with confirmed diagnosis, but without effective intervention. The last patient was confirmed amyloidosis late with no effective treatment, and he died.Conclusion: CMR features of cardiac amyloidosis include myocardium thickening, increasing myocardium mass, myocardial nulling before blood pool, dark blood pool in chambers and diffused subendocardial or transmural enhancement in LGE, all of which are characteristic, providing hints for etiological diagnosis of refractory heart failure. CMR plays a very important role in diagnosis and differential diagnosis of cardiac amyloidosis.
[Keywords] Aged；Cardiomyopathies；Amyloidosis；Cardiac magnetic resonance imaging；Magnetic resonance imaging

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

LUO Xiao-jie Department of Radiology, Beijing Hospital, Beijing 100730, China

CUI Ya-dong Department of Radiology, Beijing Hospital, Beijing 100730, China

CHEN Min* School of Graduate, Peking Union Medical College, Beijing 100005, China; Department of Radiology, Beijing Hospital, Beijing 100730, 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  2017-08-31
DOI: 10.12015/issn.1674-8034.2017.10.006
DOI:10.12015/issn.1674-8034.2017.10.006.

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