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J Am Coll Cardiol. 2016 Aug 30;68(9):949-66. doi: 10.1016/j.jacc.2016.05.096.

Left Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy?

Author information

1
Centre for Inherited Cardiovascular Diseases, IRCCS Foundation, University Hospital Policlinico San Matteo, Pavia, Italy. Electronic address: e.arbustini@smatteo.pv.it.
2
Centre for Inherited Cardiovascular Diseases, IRCCS Foundation, University Hospital Policlinico San Matteo, Pavia, Italy.
3
Centre for Inherited Cardiovascular Diseases, IRCCS Foundation, University Hospital Policlinico San Matteo, Pavia, Italy; Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota.

Erratum in

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.

KEYWORDS:

congenital heart disease; genetic counseling; genetic testing; heart ventricles; mitochondrial myopathies; myocardium

PMID:
27561770
DOI:
10.1016/j.jacc.2016.05.096
[Indexed for MEDLINE]
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