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Review paper
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Left ventricular noncompaction cardiomyopathy: Recent advances

Javier Cuevas1, Raúl Ptaszynski1, Helena Cigarrán2, Juan Calvo2, María Martín1
DOI: 10.33963/KP.a2022.0089
·
Pubmed: 35366003
Affiliations
  1. Cardiology Department, Hospital Universitario Central de Asturias, Spain
  2. Radiology Department, Hospital Universitario Central de Asturias, Spain

open access

Online first
Review article
Published online: 2022-04-01

Abstract

From its initial description to the present day, left ventricle noncompaction cardiomyopathy has been the subject of numerous studies and publications. Questioned as a real cardiomyopathy, left ventricle noncompaction can appear isolated or associated with other cardiac malformations, genetic syndromes and neuromuscular disorders. It can be sporadic or familiar being a genetically heterogeneous disorder with an autosomic dominant pattern with variable penetrance the most frequently observed. Different diagnostic criteria have been described through the years, first by echocardiogram and later on and, more recently, by cardiac magnetic resonance. Lack of universally accepted diagnostic criteria have lead to overdiagnosed in the general population. Differential diagnosis between a real cardiomyopathy, epiphenomenon (phenocopy in the setting of loading conditions or even other cardiomyopathies) and physiological hypertrabeculation, like in the athlete`s heart must be considered. Clinically can present as heart failure, ventricular arrhythmias and even sudden death, but it can also be asymptomatic diagnoses during a familial screening. Main prognosis factors are left ventricular dilatation, dysfunction and fibrosis. There is no specific treatment. Familial screening is recommended and special recommendations in case of athletes must be take into account. In the present manuscript we review the main and more recent aspects about Left ventricular noncompaction: myth and reality.

Abstract

From its initial description to the present day, left ventricle noncompaction cardiomyopathy has been the subject of numerous studies and publications. Questioned as a real cardiomyopathy, left ventricle noncompaction can appear isolated or associated with other cardiac malformations, genetic syndromes and neuromuscular disorders. It can be sporadic or familiar being a genetically heterogeneous disorder with an autosomic dominant pattern with variable penetrance the most frequently observed. Different diagnostic criteria have been described through the years, first by echocardiogram and later on and, more recently, by cardiac magnetic resonance. Lack of universally accepted diagnostic criteria have lead to overdiagnosed in the general population. Differential diagnosis between a real cardiomyopathy, epiphenomenon (phenocopy in the setting of loading conditions or even other cardiomyopathies) and physiological hypertrabeculation, like in the athlete`s heart must be considered. Clinically can present as heart failure, ventricular arrhythmias and even sudden death, but it can also be asymptomatic diagnoses during a familial screening. Main prognosis factors are left ventricular dilatation, dysfunction and fibrosis. There is no specific treatment. Familial screening is recommended and special recommendations in case of athletes must be take into account. In the present manuscript we review the main and more recent aspects about Left ventricular noncompaction: myth and reality.

Get Citation

Keywords

cardiac magnetic resonance, cardiomyopathy, left ventricular noncompaction

About this article
Title

Left ventricular noncompaction cardiomyopathy: Recent advances

Journal

Kardiologia Polska (Polish Heart Journal)

Issue

Online first

Article type

Review paper

Published online

2022-04-01

Page views

91

Article views/downloads

112

DOI

10.33963/KP.a2022.0089

Pubmed

35366003

Keywords

cardiac magnetic resonance
cardiomyopathy
left ventricular noncompaction

Authors

Javier Cuevas
Raúl Ptaszynski
Helena Cigarrán
Juan Calvo
María Martín

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