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Key Questions Relating to Left Ventricular Noncompaction Cardiomyopathy: Is the Emperor Still Wearing Any Clothes?

Lookup NU author(s): Professor Bob Anderson

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Abstract

© 2017 Canadian Cardiovascular Society. The evidence is increasing that left ventricular noncompaction cardiomyopathy as it is currently defined does not represent a failure of compaction of pre-existing trabecular myocardium found during embryonic development to form the compact component of the ventricular walls. Neither is there evidence of which we are aware to favour the notion that the entity is a return to a phenotype seen in cold-blooded animals. It is also known that when seen in adults, the presence of excessive ventricular trabeculations does not portend a poor prognosis when the ejection fraction is normal, with the risks of complications such as arrhythmia and stroke being rare in this setting. It is also the case that images of "noncompaction" as provided from children or autopsy studies are quite different from the features observed clinically in asymptomatic adults with excessive trabeculation. Our review suggests that the presence of an excessively trabeculated left ventricular wall is not in itself a clinical entity. It is equally possible that the excessive trabeculation is no more than a bystander in the presence of additional lesions such as dilated cardiomyopathy, with the additional lesions being responsible for the reduced ejection fraction bringing a given patient to clinical attention. We, therefore, argue that the term "noncompaction cardiomyopathy" is misleading, because there is neither failure of compaction nor a cardiomyopathic process in most individuals that fulfill widely used diagnostic criteria.

Publication metadata

Author(s): Anderson RH, Jensen B, Mohun TJ, Petersen SE, Aung N, Zemrak F, Planken RN, MacIver DH

Publication type: Article

Publication status: Published

Journal: Canadian Journal of Cardiology

Year: 2017

Volume: 33

Issue: 6

Pages: 747-757

Print publication date: 01/06/2017

Online publication date: 01/02/2017

Acceptance date: 20/01/2017

ISSN (print): 0828-282X

ISSN (electronic): 1916-7075

Publisher: Elsevier Inc.

URL: http://doi.org/10.1016/j.cjca.2017.01.017

DOI: 10.1016/j.cjca.2017.01.017

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