Familial Mitral Arcade, Tricuspid Dysplasia, Left Ventricular Noncompaction and Short-Chain Acyl-CoA Reductase Deficiency

Adil, Amina; Samad, Fatima; Bush, Michelle; Galazka, Patrycja

doi:10.1016/j.amjcard.2019.11.005

Cited by 2 publications

(2 citation statements)

References 26 publications

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“…There are little information on the tricuspid valve in LVNC and most of them are case reports focusing on associated TV abnormalities ( 23 , 24 ). In this study, TA dilation could be demonstrated in a series of patients with isolated LVNC without significant TR (except 1 case), which was associated with increased RA volumes.…”

Section: Discussionmentioning

confidence: 99%

Tricuspid Annular Abnormalities in Isolated Left Ventricular Non-compaction—Insights From the Three-dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Nemes

Rácz

Kormányos

2022

Front. Cardiovasc. Med.

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IntroductionProminent trabecular left ventricular (LV) meshwork and deep intertrabecular LV recesses are featuring LV non-compaction (LVNC). The aim of this study was to evaluate tricuspid annular (TA) morphological and functional abnormalities by three-dimensional speckle-tracking echocardiography (3DSTE) in patients with LVNC without right ventricular (RV) involvement.Materials and MethodsThis study consisted of 21 patients with isolated LVNC, from which 6 cases were excluded due to inferior image quality. The remaining patient group consisted of 15 subjects with a mean age of 52.1 ± 11.4 years (9 males). LVNC was defined according to the Jenni's criteria. Their results were compared to 21 age- and gender-matched healthy controls (mean age: 52.4 ± 3.9 years, 14 males). Complete routine 2-dimensional Doppler echocardiographic examination was performed in all the patients with isolated LVNC and healthy controls. End-systolic and end-diastolic TA dimensions were assessed on selected planes derived from full-volume 3D echocardiographic datasets during 3DSTE.ResultsPatients with isolated LVNC showed significantly dilated end-systolic and end-diastolic TA diameter and area, which were accompanied with preserved TA functional properties and associated with right atrial (RA) volumes. TA plane systolic excursion (TAPSE) showed mild correlations with TA fractional area change (TAFAC) and TA fractional shortening (TAFS). No correlations could be demonstrated between TAPSE and TA morphological features. Extent of LVNC did not correlate with any echocardiographic parameters.ConclusionTA is dilated with preserved sphincter-like function in patients with isolated LVNC. Longitudinal (TAPSE) and sphincter-like (TAFAC and TAFS) TA movements correlate with each other. TA dilation is associated with an increased RA volumes respecting cardiac cycle.

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Section: Discussionmentioning

confidence: 99%

Tricuspid Annular Abnormalities in Isolated Left Ventricular Non-compaction—Insights From the Three-dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Nemes

Rácz

Kormányos

2022

Front. Cardiovasc. Med.

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“…In contrast, pathways involving acyl-CoA and ketone body metabolism were negatively correlated with LVNC. Diverse de ciencies of key enzymes through the metabolism of acyl-CoA were reported in association with LVNC, including malonyl coenzyme A decarboxylase [31], 3-hydroxy-3-methylglutarylcoenzyme A lyase (HMG-CoA) [32] and short-chain acyl-CoA reductase [33]. Meanwhile, HMG-CoA also participates in the last step of ketogenesis.…”

Section: Discussionmentioning

confidence: 99%

Construction of a ceRNA network reveals functional lncRNAs in left ventricular noncompaction through weighted gene co-expression network analysis

Kan¹,

Zhang

et al. 2022

Preprint

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Background Left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy. Lack of biomarkers and insights into epigenetic mechanism limits its screening, diagnosis and therapy. This study aimed to delineate the lncRNA-miRNA-mRNA interaction underlying LVNC through weighted gene co-expression network analysis (WGCNA) and construction of a competing endogenous RNA (ceRNA) network. Methods By analyzing microarray data from GSE178582, we acquired lncRNA and mRNA expression profiling in LVNC. Differentially expressed lncRNAs and mRNAs were identified using R package “limma”. Gene set enrichment analysis (GSEA) was conducted to detect biological function alteration in LVNC. We performed WGCNA to pick cardinal gene modules in LVNC and supplemented enrichmsent annotation based on mRNA modules. Interactions of lncRNA-miRNA and miRNA-mRNA were respectively predicted by LncBase and miRTarBase. In comprehensive consideration of expression change, gene clustering results and database prediction, we constructed a ceRNA network and visualized the lncRNA-miRNA-mRNA interaction. Then, a subnetwork centered on hub genes was extracted. Results 2452 mRNAs (1275 upregulated and 1177 downregulated) and 1415 lncRNAs (839 upregulated and 576 downregulated) were differentially expressed between LVNC patients and healthy controls. Enriched pathways involved acute myeloid leukemia, bile secretion, thyroid hormone synthesis etc. A ceRNA network with 3 lncRNAs, 5miRNAs and 106 mRNAs was constructed. Furthermore, we identified 9 hub mRNAs and visualized their lncRNA-miRNA-mRNA subnetwork. Conclusions The ceRNA regulatory network participates in the pathogenesis of LVNC, and identified hub genes may be potential targets for intervention and biomarkers to screen LVNC patients.

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Familial Mitral Arcade, Tricuspid Dysplasia, Left Ventricular Noncompaction and Short-Chain Acyl-CoA Reductase Deficiency

Cited by 2 publications

References 26 publications

Tricuspid Annular Abnormalities in Isolated Left Ventricular Non-compaction—Insights From the Three-dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Tricuspid Annular Abnormalities in Isolated Left Ventricular Non-compaction—Insights From the Three-dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Construction of a ceRNA network reveals functional lncRNAs in left ventricular noncompaction through weighted gene co-expression network analysis

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