Publication: Non-compaction of the ventricular myocardium, a cardiomyopathy in search of a pathoanatomical definition
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Date
2010
Authors
Val-Bernal, José Fernando ; Garijo, M.F. ; Rodriguez-Villar, Diana ; Val, D.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Ventricular non-compaction is a rare
cardiomyopathy characterized by numerous, excessively
prominent ventricular trabeculations and deep
intertrabecular recesses communicating with the
ventricular cavity. The lesion is postulated to result from
an intrauterine developmental arrest that stops
compaction of the myocardial fiber meshwork. This
cardiomyopathy affects the left ventricle, with or without
concomitant right ventricular involvement. The disease
is now seen with increasing frequency and it is clinically
diagnosed by imaging techniques such as
echocardiography or cardiac magnetic resonance.
Current diagnostic criteria are considered too sensitive,
particularly in black individuals. Therefore, this
condition has generated considerable controversy and
demands a new definition. Non-compaction
cardiomyopathy shows variability of hereditary patterns,
genetic heterogeneity, diversity in associated phenotypes
and a wide spectrum of clinical presentation and
pathophysiological findings. Non-compaction can be
simply a variant of normal maturation of the ventricular
myocardium with only the most severe forms producing
a distinct clinical-pathological entity. Ventricular noncompaction
most probably is a secondary consequence
of an underlying molecular derangement produced by a
pathogenetic mutation. It is likely that surgical
pathologists will find this entity more frequently due to
involvement in transplantation teams.
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