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Left Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy?

Abstract：

:Left ventricular noncompaction (LVNC) describes a ventricular wall anatomy characterized by prominent left ventricular (LV) trabeculae, a thin compacted layer, and deep intertrabecular recesses. Individual variability is extreme, and trabeculae represent a sort of individual "cardioprinting." By itself, the diagnosis of LVNC does not coincide with that of a "cardiomyopathy" because it can be observed in healthy subjects with normal LV size and function, and it can be acquired and is reversible. Rarely, LVNC is intrinsically part of a cardiomyopathy; the paradigmatic examples are infantile tafazzinopathies. When associated with LV dilation and dysfunction, hypertrophy, or congenital heart disease, the genetic cause may overlap. The prevalence of LVNC in healthy athletes, its possible reversibility, and increasing diagnosis in healthy subjects suggests cautious use of the term LVNC cardiomyopathy, which describes the morphology but not the functional profile of the cardiomyopathy.

journal_name

J Am Coll Cardiol

journal_title

Journal of the American College of Cardiology

authors

Arbustini E,Favalli V,Narula N,Serio A,Grasso M

doi

10.1016/j.jacc.2016.05.096

subject

Has Abstract

pub_date

2016-08-30 00:00:00

pages

949-66

issue

9

eissn

0735-1097

issn

1558-3597

pii

S0735-1097(16)34481-3

journal_volume

68

pub_type

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