: Left ventricular non-compaction (LVNC) is a rare cardiomyopathy typically involving the left ventricle (LV); however, the right ventricle (RV) can also be affected. This case-control study aimed to assess the morphology and function of LV and RV in children with LVNC. : Sixteen children (13 ± 3 years, six girls) with LVNC were compared with 16 sex- and age-matched controls. LV and RV morphology and function were evaluated in cardiovascular magnetic resonance (CMR) studies. Additionally, LV and RV global radial (GRS), circumferential (GCS), and longitudinal strain (GLS) were assessed using tissue-tracking analysis. : Patients with LVNC did not differ from the healthy controls in terms of age, height, weight, and body surface area (BSA). In total, 4/16 subjects with LVNC had mid-wall late gadolinium enhancement (LGE). Compared to the control group, patients with LVNC had higher end-diastolic volume (EDV) indexed for body surface area (BSA), lower ejection fraction (EF), and lower LV strain parameters (all < 0.05). Children with LVNC also presented with thicker RV apical trabeculation, whereas there were no differences in RV EF and EDV/BSA between the groups. Nevertheless, children with LVNC had impaired RV GRS and GCS (both < 0.05). : LVNC in pediatric patients is associated with LV enlargement and impaired LV systolic function. Additionally, children with LVNC have increased RV trabeculations and subclinical impairment of RV myocardial deformation.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8875170PMC
http://dx.doi.org/10.3390/jcm11041104DOI Listing

Publication Analysis

Top Keywords

children lvnc
16
left ventricular
12
morphology function
12
lvnc
10
myocardial deformation
8
ventricular non-compaction
8
cardiovascular magnetic
8
magnetic resonance
8
patients lvnc
8
body surface
8

Similar Publications

Inherited cardiac channelopathies are major causes of sudden cardiac death (SCD) in young people. Genetic testing is focused on the identification of single-nucleotide variants (SNVs) by Next-Generation Sequencing (NGS). However, genetically elusive cases can carry copy number variants (CNVs), which need specific detection tools.

View Article and Find Full Text PDF
Article Synopsis
  • Left ventricular noncompaction (LVNC) is a hereditary heart condition marked by unusual heart muscle structure, and this study specifically focused on biventricular noncompaction (BiVNC) in children to understand its clinical characteristics and genetic factors.
  • The research involved 234 pediatric patients and revealed that BiVNC often leads to serious complications, including a higher incidence of congenital heart disease and reduced survival rates compared to other heart conditions.
  • Findings indicated that patients with BiVNC frequently exhibited left ventricular dysfunction and a notable percentage had genetic variants linked to mitochondrial and developmental issues, emphasizing the need for thorough genetic screening for better patient outcomes.
View Article and Find Full Text PDF

Background: To investigate the outcomes, clinical prognosticators, and genetic profiles of pediatric left ventricular non-compaction (LVNC).

Methods: All subjects were <18 years old, diagnosed with LVNC between January 2008 and December 2020. Whole-exome sequencing was undertaken.

View Article and Find Full Text PDF

What happened to the left ventricular non-compaction cardiomyopathy? to be or not to be: This is the question.

Curr Probl Cardiol

November 2024

Division of Cardiology, University Hospital Paolo Giaccone, Palermo, Italy; Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) "G. D'Alessandro", University of Palermo, Palermo, Italy.

For several years, left ventricular non-compaction (LVNC) was considered as a true cardiomyopathy and several definitions have followed one another. Particularly, LVNC was characterized by prominent left ventricular trabeculae separated from deep intertrabecular recesses. Several echocardiographic criteria and cardiac magnetic resonance imaging (CMR) criteria have been used to diagnose LVNC, leading to overestimate the diagnosis of LVNC in patients with other diseases and/or physiological conditions.

View Article and Find Full Text PDF

Ebstein Anomaly (EA) is a malformation of the right heart, but there is data to suggest that the left ventricle (LV) can suffer from intrinsic structural and functional abnormalities which affect surgical outcomes. The LV in patients with EA is hypertrabeculated with abnormalities in LV function and strain. In this retrospective single-center study, patients with EA who underwent pre-operative cardiac MRI (CMR) between the periods of 2014-2024 were included along with a group of healthy-age-matched controls.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!