J Cardiovasc Ultrasound.  2014 Dec;22(4):165-172. 10.4250/jcu.2014.22.4.165.

Non-Compact Cardiomyopathy or Ventricular Non-Compact Syndrome?

• Yin L

• KMID: 1980418
• DOI: http://doi.org/10.4250/jcu.2014.22.4.165

Abstract

Ventricular myocardial non-compaction has been recognized and defined as a genetic cardiomyopathy by American Heart Association since 2006. The argument on the nomenclature and pathogenesis of this kind of ventricular myocardial non-compaction characterized by regional ventricular wall thickening and deep trabecular recesses often complicated with chronic heart failure, arrhythmia and thromboembolism and usually overlap the genetics and phenotypes of other kind of genetic or mixed cardiomyopathy still exist. The proper classification and correct nomenclature of the non-compact ventricles will contribute to the precisely and completely understanding of etiology and its related patho-physiological mechanism for a better risk stratification and more personalized therapy of the disease individually. All of the genetic heterogeneity and phenotypical overlap and the variety in histopathological, electromechanical and clinical presentation indicates that some of the cardiomyopathies might just be the different consequence of myocardial development variations related to gene mutation and phenotype of one or group genes induced by the interacted and disturbed process of gene modulation at different links of gene function expression and some other etiologies. This review aims to establish a new concept of "ventricular non-compaction syndrome" based on the demonstration of the current findings of etiology, epidemiology, histopathology and echocardiography related to the disorder of ventricular myocardial compaction and myocardial electromechanical function development.

Keyword

Non-compact; Ventricle; Cardiomyopathy; Syndrome

MeSH Terms

American Heart Association
Arrhythmias, Cardiac
Cardiomyopathies*
Classification
Echocardiography
Epidemiology
Genetic Heterogeneity
Genetics
Heart Failure
Humans
Phenotype
Thromboembolism

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