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Left ventricular noncompaction in children: the role of genetics, morphology, and function for outcome

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Item Type:Review
Title:Left ventricular noncompaction in children: the role of genetics, morphology, and function for outcome
Creators Name:Klaassen, S., Kühnisch, J., Schultze-Berndt, A. and Seidel, F.
Abstract:Left ventricular noncompaction (LVNC) is a ventricular wall anomaly morphologically characterized by numerous, excessively prominent trabeculations and deep intertrabecular recesses. Accumulating data now suggest that LVNC is a distinct phenotype but must not constitute a pathological phenotype. Some individuals fulfill the morphologic criteria of LVNC and are without clinical manifestations. Most importantly, morphologic criteria for LVNC are insufficient to diagnose patients with an associated cardiomyopathy (CMP). Genetic testing has become relevant to establish a diagnosis associated with CMP, congenital heart disease, neuromuscular disease, inborn error of metabolism, or syndromic disorder. Genetic factors play a more decisive role in children than in adults and severe courses of LVNC tend to occur in childhood. We reviewed the current literature and highlight the difficulties in establishing the correct diagnosis for children with LVNC. Novel insights show that the interplay of genetics, morphology, and function determine the outcome in pediatric LVNC.
Keywords:Noncompaction, Cardiomyopathy, Congenital Heart Disease, Genetics, Children
Source:Journal of Cardiovascular Development and Disease
ISSN:2308-3425
Publisher:MDPI
Volume:9
Number:7
Page Range:206
Date:30 June 2022
Official Publication:https://doi.org/10.3390/jcdd9070206
PubMed:View item in PubMed

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