Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular Disease

Left ventricular non-compaction (LVNC) is an extremely heterogeneous disorder with a highly variable clinical presentation, morphologic appearance at imaging testing, and prognosis. It is still unclear whether LVNC should be classified as a separate cardiomyopathy or if it is a mere morphological tr...

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Main Authors: Flavia Fusco, Nunzia Borrelli, Rosaria Barracano, Giovanni Domenico Ciriello, Federica Verrillo, Giancarlo Scognamiglio, Berardo Sarubbi
Format: Article
Language:English
Published: MDPI AG 2022-04-01
Series:Cardiogenetics
Subjects:
Online Access:https://www.mdpi.com/2035-8148/12/2/16
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author Flavia Fusco
Nunzia Borrelli
Rosaria Barracano
Giovanni Domenico Ciriello
Federica Verrillo
Giancarlo Scognamiglio
Berardo Sarubbi
author_facet Flavia Fusco
Nunzia Borrelli
Rosaria Barracano
Giovanni Domenico Ciriello
Federica Verrillo
Giancarlo Scognamiglio
Berardo Sarubbi
author_sort Flavia Fusco
collection DOAJ
description Left ventricular non-compaction (LVNC) is an extremely heterogeneous disorder with a highly variable clinical presentation, morphologic appearance at imaging testing, and prognosis. It is still unclear whether LVNC should be classified as a separate cardiomyopathy or if it is a mere morphological trait shared by many phenotypically distinct cardiomyopathies. Moreover, the hypertrabeculated phenotype may be reversible in some cases, possibly reflecting the left ventricular physiological response of the cardiac muscle to chronic overload. The current diagnostic criteria have several limitations, leaving many patients in a grey area. Here, we review the available literature on LVNC in order to provide an overview of the current knowledge on this complex disorder.
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spelling doaj.art-c721133f872e4b5c917148ee65ec7e692023-11-23T15:58:37ZengMDPI AGCardiogenetics2035-82532035-81482022-04-0112217018410.3390/cardiogenetics12020016Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular DiseaseFlavia Fusco0Nunzia Borrelli1Rosaria Barracano2Giovanni Domenico Ciriello3Federica Verrillo4Giancarlo Scognamiglio5Berardo Sarubbi6Adult Congenital Heart Disease Unit, AO dei Colli—Monaldi Hospital, 80131 Naples, ItalyAdult Congenital Heart Disease Unit, AO dei Colli—Monaldi Hospital, 80131 Naples, ItalyAdult Congenital Heart Disease Unit, AO dei Colli—Monaldi Hospital, 80131 Naples, ItalyAdult Congenital Heart Disease Unit, AO dei Colli—Monaldi Hospital, 80131 Naples, ItalyInherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania Luigi Vanvitelli, Monaldi Hospital, 80131 Naples, ItalyAdult Congenital Heart Disease Unit, AO dei Colli—Monaldi Hospital, 80131 Naples, ItalyAdult Congenital Heart Disease Unit, AO dei Colli—Monaldi Hospital, 80131 Naples, ItalyLeft ventricular non-compaction (LVNC) is an extremely heterogeneous disorder with a highly variable clinical presentation, morphologic appearance at imaging testing, and prognosis. It is still unclear whether LVNC should be classified as a separate cardiomyopathy or if it is a mere morphological trait shared by many phenotypically distinct cardiomyopathies. Moreover, the hypertrabeculated phenotype may be reversible in some cases, possibly reflecting the left ventricular physiological response of the cardiac muscle to chronic overload. The current diagnostic criteria have several limitations, leaving many patients in a grey area. Here, we review the available literature on LVNC in order to provide an overview of the current knowledge on this complex disorder.https://www.mdpi.com/2035-8148/12/2/16left ventricular non-compactionLVNChypertrabeculated left ventriclecardiomyopathyreversible
spellingShingle Flavia Fusco
Nunzia Borrelli
Rosaria Barracano
Giovanni Domenico Ciriello
Federica Verrillo
Giancarlo Scognamiglio
Berardo Sarubbi
Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular Disease
Cardiogenetics
left ventricular non-compaction
LVNC
hypertrabeculated left ventricle
cardiomyopathy
reversible
title Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular Disease
title_full Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular Disease
title_fullStr Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular Disease
title_full_unstemmed Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular Disease
title_short Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular Disease
title_sort left ventricular non compaction spectrum in adults and children from a morphological trait to a structural muscular disease
topic left ventricular non-compaction
LVNC
hypertrabeculated left ventricle
cardiomyopathy
reversible
url https://www.mdpi.com/2035-8148/12/2/16
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