Abstract

  Advances in cardiac imaging have resulted in greater recognition of light-chain (AL) cardiac amyloidosis in everyday clinical practice.

  AL amyloidosis is a rare and multisystem disease characterized by deposition of misfolded immunoglobulin light chain (LC) in the heart that causes a restrictive cardiomyopathy.

  We describe a 50-year-old melanodermic male that is followed at our Hospital with AL amyloidosis diagnosed since 2016, with renal and cardiac involvement. At the time of diagnosis, the echo showed left ventricular hypertrophy with moderately impaired function and typical apical sparing. A CMR demonstrated pleural and pericardial effusions; diffuse subendocardial staining pattern and remarkable difficult “nulling” of the myocardium of the left ventricle after gadolinium administration. An abdominal biopsy was positive for amyloidosis. In the last consultation, June/2018, the echo showed a moderately decreased of the left and the right ventricular function and blood and urine tests with NT-proBNP^b of 4048 ng/l, hsTnT^c 30 ng/L and albuminuria 0.58 g/day with MDRD^d 64ml/min.

  Improved medical recognition is necessary for earlier diagnosis and treatment and prompt outcomes4. Ventricular hypertrophy or heart failure without a known case maybe the first red flag for cardiac amyloid fibril infiltration and CMR should be considered.

  The CMR is useful in distinguishing ventricular wall thickening as a result of amyloid infiltration. Late gadolinium enhancement adds a unique advantage in evaluating myocardial tissue characterization. Although, CMR be a non-invasive exam that is similar to cardiac biopsy, delayed hyper-enhancement imaging may lead to the development of new classification and prognostic systems.

Keywords: Cardiac Magnetic Resonance; Cardiac Amyloidosis; Late Gadolinium Enhancement and Extracellular Volume

References

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Citation

Citation: F Lucas., et al. "Amyloidosis and Cardiac Magnetic Resonance".Acta Scientific Gastrointestinal Disorders 3.2 (2020): 07-08.

Copyright

Copyright: © 2020 F Lucas., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.