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Non-Compact Cardiomyopathy or Ventricular Non-Compact Syndrome?

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

Abstract

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

참고문헌 (66)

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