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Published in: BMC Cardiovascular Disorders 1/2018

Open Access 01-12-2018 | Research article

Diagnosis of cardiac amyloidosis: a systematic review on the role of imaging and biomarkers

Authors: Panagiota Kyriakou, Dimitrios Mouselimis, Anastasios Tsarouchas, Angelos Rigopoulos, Constantinos Bakogiannis, Michel Noutsias, Vasileios Vassilikos

Published in: BMC Cardiovascular Disorders | Issue 1/2018

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Abstract

Background

Cardiac Amyloidosis (CA) pertains to the cardiac involvement of a group of diseases, in which misfolded proteins deposit in tissues and cause progressive organ damage. The vast majority of CA cases are caused by light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). The increased awareness of these diseases has led to an increment of newly diagnosed cases each year.

Methods

We performed multiple searches on MEDLINE, EMBASE and the Cochrane Database of Systematic Reviews. Several search terms were used, such as “cardiac amyloidosis”, “diagnostic modalities cardiac amyloidosis” and “staging cardiac amyloidosis”. Emphasis was given on original articles describing novel diagnostic and staging approaches to the disease.

Results

Imaging techniques are indispensable to diagnosing CA. Novel ultrasonographic techniques boast high sensitivity and specificity for the disease. Nuclear imaging has repeatedly proved its worth in the diagnostic procedure, with efforts now focusing on standardization and quantification of amyloid load. Because the latter would be invaluable for any staging system, those spearheading research in magnetic resonance imaging of the disease are also trying to come up with accurate tools to quantify amyloid burden. Staging tools are currently being developed and validated for ATTR CA, in the spirit of the acclaimed Mayo Staging System for AL.

Conclusion

Cardiac involvement confers significant morbidity and mortality in all types of amyloidosis. Great effort is made to reduce the time to diagnosis, as treatment in the initial stages of the disease is tied to better prognosis. The results of these efforts are highly sensitive and specific diagnostic modalities that are also reasonably cost effective.
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Metadata
Title
Diagnosis of cardiac amyloidosis: a systematic review on the role of imaging and biomarkers
Authors
Panagiota Kyriakou
Dimitrios Mouselimis
Anastasios Tsarouchas
Angelos Rigopoulos
Constantinos Bakogiannis
Michel Noutsias
Vasileios Vassilikos
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Cardiovascular Disorders / Issue 1/2018
Electronic ISSN: 1471-2261
DOI
https://doi.org/10.1186/s12872-018-0952-8

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