Ventricular Noncompaction Research Articles

Entropy as a novel predictor of cardiovascular events in patients with left ventricular noncompaction

BackgroundThe risk stratification of left ventricular noncompaction (LVNC) remains ambiguous. LV entropy derived from late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR) as a novel measurement of myocardial heterogeneity may serve as the substrate of major adverse cardiovascular events (MACEs). This retrospective study aimed to investigate the value of LV entropy for predicting MACEs in LVNC patients. MethodsConsecutive patients who underwent CMR and met the diagnosis criteria of LVNC were included. All patients were follow-up for MACEs (cardiac death, ventricular arrhythmia requiring therapy or heart failure hospitalization), and their LV entropy derived from the distribution of pixel signal intensities in the LGE of the LV myocardium was analyzed. ResultsOne hundred and forty-three patients (mean age 40 years, 64.3% male) were followed for a median of 3.2 years, and forty-two (29.4%) experienced MACEs. Presenting of symptoms, left ventricular end-diastolic diameter (LVEDD), LV end-diastolic volume (LVEDV) index, LV end-systolic volume (LVESV) index, LV ejection fraction (LVEF), LGE extent, and LV entropy showed association with MACEs. LV entropy maintained independent association with MACEs (HR: 4.76, 95%CI 3.68–5.15, p < 0.001) in multivariable analysis. Entropy was also strong independent predictor of MACEs in patients with and without LGE (HR: 5.89, 95% CI4.18–7.73, p < 0.001; HR: 3.06, 95% CI:1.53–4.80, p = 0.013, respectively). ConclusionsLV entropy can predict MACEs in LVNC patients and provide incremental prognostic value on top of LVEF and LGE. Also, LV entropy may help risk stratification in LGE-negative LVNC patients.

Establishment and identification of cardiomyocyte arhGEF18 gene conditional knockout mice

AbstractLeft ventricular noncompaction (LVNC) is a heterogeneous disorder with unclear genetic causes. The arhGEF18 gene is a guanine nucleotide exchange factor related to the Rho pathway and a possible predisposing gene for LVNC. In this study, a mouse model of arhGEF18 gene conditional knockout (cKO) in cardiomyocytes was established using the Cre‐LoxP system to provide an animal model for the genetic study of LVNC. ArhGEF18 cKO mice were obtained by crossing arhGEF18f/+/myh6‐Cre+ and arhGEF18f/f mice. The mRNA and protein expression levels of arhGEF18 in different tissues were verified. The myocardial cytoskeleton and polar protein expression, the survival rate, cardiac function, and cardiac histology of model mice were determined. ArhGEF18 cKO mice were successfully established. ArhGEF18 was confirmed to be specifically knocked out in the myocardial tissue of arhGEF18f/f/myh6‐Cre+ mice at the mRNA and protein levels, and the knockout rate was approximately 75%. The relative expression levels of cytoskeleton and cell polarity proteins such as α/β‐tubulin, Scribble, Crb2, and PAR3 in the cKO group were significantly lower than those in the control group (p &lt; 0.05). During monitoring, the survival rate, cardiac systolic function, and myocardial structure of arhGEF18f/f/myh6‐Cre+ mice were not significantly different from those of control mice. A mouse model of cardiomyocyte arhGEF18 gene cKO was successfully established, and it was confirmed that knocking out arhGEF18 changed cytoskeleton and cell polar protein expression levels in the myocardium. However, there was no obvious LVNC phenotype in the constructed arhGEF18 cKO mice.

Left ventricular noncompaction in Ibadan, Nigeria

BackgroundThere has been an increase in the reporting of cases of left ventricular noncompaction (LVNC) cardiomyopathy in medical literature due to advances in medical imaging. Patients with LVNC may be asymptomatic or may present with arrhythmias, heart failure, thromboembolism or sudden death. LVNC is typically diagnosed by echocardiography, although there are higher-resolution cardiac imaging techniques such as cardiac magnetic resonance imaging (MRI) to make the diagnosis. The objective of the study is to report on a series of 9 cases of LVNC cardiomyopathy seen at the University College Hospital, Ibadan. Cases of LVNC seen between September 1, 2015 and July 31, 2022 in our echocardiography service is being reported.ResultsThere were a total of 6 men and 3 women. Mean age at presentation was 52.89 ± 15.02 years. The most common mode of presentation was heart failure (6 patients). Hypertension was the most common comorbidity (6 patients). Three patients had an ejection fraction of less than 40% and the mean ratio of noncompacted to compacted segment at end-systole was 2.80 ± 0.48. The most common areas of trabecular localization were the LV lateral wall and the apex. Beta blockers were highly useful in the management of the patients.ConclusionsLVNC cardiomyopathy is not uncommon in our environment and a high index of suspicion is often required.

Abstract P1182: The Genetic Dissection Of Rpl3l As A Causal Gene For Neonatal Dilated Cardiomyopathy And Left Ventricular Noncompaction

Background: Cardiomyopathies are responsible for nearly 10% of cardiac deaths in neonates. Recently, biallelic variants in ribosomal protein L3-like ( RPL3L , NM_005061.2) have been reported in neonates with dilated cardiomyopathy (DCM) and heart failure (HF). This study is aimed to study RPL3L induced genetic pathways and mechanisms involved in the development of DCM. Methods: Whole exome and RNA sequencing, histology, immunohistochemistry, and Western blotting were performed in a human explanted heart. Genetic correlation, expression quantitative trait loci (eQTL) mapping and functional analysis were performed using cardiac gene expression data from the patient as well as human and murine genetic reference populations (GRPs). Results: Two-month-old patient with DCM and HF carrying compound heterozygous RPL3L variants, p.A51T and p.V231F, has been managed on an INTERMACS profile 2 with continuous-flow ventricular assist device and successfully bridged to heart transplantation at the age of 4 months. Morphology of the explanted heart displayed apical deep intertrabecular recesses consistent with left ventricular noncompaction (LVNC). Histology revealed interstitial fibrosis and myocardial hypertrophy. No signs of myocarditis and glycogen or mucopolysaccharides deposits were detected. Immunohistochemistry revealed abnormal clusters of RPL3L protein in the cytosol of cardiomyocytes and disruption of F-actin, Z-disk proteins, MYL4 and dystrophin was evident. In a mouse heart, the Rpl3l level was of 33.45 RPKM, while levels of RPL3L appeared to be 32 RPKM in a human heart compared to its mean expression in other adult tissues of 2.54 (mouse) and 2.36 (human). Systems genetics analysis identified high expression values ranged from 11.31 to 12.16 across murine GRPs of BXD mice with the ~1.8-fold difference. Pathways such as “thermogenesis”, “diabetic cardiomyopathy” and “DCM” significantly associated with RPL3L. e QTL mapping suggested Myl4 (Chr 11) and Sdha (Chr 13) as the upstream regulators of Rpl3l . Conclusions: Compound RPL3L heterozygosity is causal for neonatal DCM and LVNC. The RPL3L is highly expressed in the heart tissue of humans and mice. Genes, Myl4 and Sdha, are strong candidates that regulate expression of Rpl3l in murine heart.

Left Ventricular Noncompaction Masquerading as Hypertrophic Nonobstructive Cardiomyopathy

A 61-year-old male with no significant medical history presented with dyspnea on exertion for the past 3 months. Clinical examination findings and electrocardiogram showed sinus rhythm and left ventricular hypertrophy. The chest X-ray and basic laboratory tests were within the normal limits. A transthoracic echocardiogram was performed which showed left ventricular wall thickening involving the anterior wall, lateral wall, septum, and inferior wall in the basal, mid, and apical segments. The left ventricular apex showed disproportionately increased thickness. There was no left ventricular outflow tract (LVOT) turbulence or obstruction. Left ventricular angiogram showed prominent trabeculations with deep intertrabecular recesses. LVOT obstruction was ruled out. The patient then underwent cardiac magnetic resonance imaging, which showed features suggestive of left ventricular noncompaction.

A novel mutation in the TTN gene resulted in left ventricular noncompaction: a case report and literature review

BackgroundLeft ventricular noncompaction (LVNC) is a specific type of cardiomyopathy characterized by coarse trabeculae and interspersed trabecular crypts within the ventricles. Clinical presentation varies widely and may be nonsignificant or may present with progressive heart failure, malignant arrhythmias, and multiorgan embolism. The mode of inheritance is highly heterogeneous but is most commonly autosomal dominant. The TTN gene encodes titin, which is not only an elastic component of muscle contraction but also mediates multiple signalling pathways in striated muscle cells. In recent years, mutations in the TTN gene have been found to be associated with LVNC, but the exact pathogenesis is still not fully clarified.Case presentationIn this article, we report a case of an adult LVNC patient with a TTN gene variant, c.87857G > A (p. Trp29286*), that has not been reported previously. This 43-year-old adult male was hospitalized repeatedly for heart failure. Echocardiography showed reduced myocardial contractility, dilated left ventricle with many prominent trabeculae, and a loose texture of the left ventricular layer of myocardium with crypt-like changes. During the out-of-hospital follow-up, the patient had no significant signs or symptoms of discomfort.ConclusionThis case report enriches the mutational spectrum of the TTN gene in LVNC and provides a basis for genetic counselling and treatment of this patient. Clinicians should improve their understanding of LVNC, focusing on exploring its pathogenesis and genetic characteristics to provide new directions for future diagnosis and treatment.

Variants in ACTC1 underlie distal arthrogryposis accompanied by congenital heart defects.

Contraction of the human sarcomere is the result of interactions between myosin cross-bridges and actin filaments. Pathogenic variants in genes such as MYH7, TPM1, and TNNI3 that encode parts of the cardiac sarcomere cause muscle diseases that affect the heart, such as dilated cardiomyopathy and hypertrophic cardiomyopathy. In contrast, pathogenic variants in homologous genes such as MYH2, TPM2, and TNNI2 that encode parts of the skeletal muscle sarcomere cause muscle diseases affecting skeletal muscle, such as distal arthrogryposis (DA) syndromes and skeletal myopathies. To date, there have been few reports of genes (e.g., MYH7) encoding sarcomeric proteins in which the same pathogenic variant affects skeletal and cardiac muscle. Moreover, none of the known genes underlying DA have been found to contain pathogenic variants that also cause cardiac abnormalities. We report five families with DA because of heterozygous missense variants in the gene actin, alpha, cardiac muscle 1 (ACTC1). ACTC1 encodes a highly conserved actin that binds to myosin in cardiac and skeletal muscle. Pathogenic variants in ACTC1 have been found previously to underlie atrial septal defect, dilated cardiomyopathy, hypertrophic cardiomyopathy, and left ventricular noncompaction. Our discovery delineates a new DA condition because of variants in ACTC1 and suggests that some functions of ACTC1 are shared in cardiac and skeletal muscle.

Meet the First Authors.

Meet the First Authors.

Increased Ca2+ Transient Underlies RyR2-Related Left Ventricular Noncompaction.

A loss-of-function cardiac ryanodine receptor (RyR2) mutation, I4855M+/-, has recently been linked to a new cardiac disorder termed RyR2 Ca2+ release deficiency syndrome (CRDS) as well as left ventricular noncompaction (LVNC). The mechanism by which RyR2 loss-of-function causes CRDS has been extensively studied, but the mechanism underlying RyR2 loss-of-function-associated LVNC is unknown. Here, we determined the impact of a CRDS-LVNC-associated RyR2-I4855M+/- loss-of-function mutation on cardiac structure and function. We generated a mouse model expressing the CRDS-LVNC-associated RyR2-I4855M+/- mutation. Histological analysis, echocardiography, ECG recording, and intact heart Ca2+ imaging were performed to characterize the structural and functional consequences of the RyR2-I4855M+/- mutation. As in humans, RyR2-I4855M+/- mice displayed LVNC characterized by cardiac hypertrabeculation and noncompaction. RyR2-I4855M+/- mice were highly susceptible to electrical stimulation-induced ventricular arrhythmias but protected from stress-induced ventricular arrhythmias. Unexpectedly, the RyR2-I4855M+/- mutation increased the peak Ca2+ transient but did not alter the L-type Ca2+ current, suggesting an increase in Ca2+-induced Ca2+ release gain. The RyR2-I4855M+/- mutation abolished sarcoplasmic reticulum store overload-induced Ca2+ release or Ca2+ leak, elevated sarcoplasmic reticulum Ca2+ load, prolonged Ca2+ transient decay, and elevated end-diastolic Ca2+ level upon rapid pacing. Immunoblotting revealed increased level of phosphorylated CaMKII (Ca2+-calmodulin dependent protein kinases II) but unchanged levels of CaMKII, calcineurin, and other Ca2+ handling proteins in the RyR2-I4855M+/- mutant compared with wild type. The RyR2-I4855M+/- mutant mice represent the first RyR2-associated LVNC animal model that recapitulates the CRDS-LVNC overlapping phenotype in humans. The RyR2-I4855M+/- mutation increases the peak Ca2+ transient by increasing the Ca2+-induced Ca2+ release gain and the end-diastolic Ca2+ level by prolonging Ca2+ transient decay. Our data suggest that the increased peak-systolic and end-diastolic Ca2+ levels may underlie RyR2-associated LVNC.

The Rare Condition of Left Ventricular Non-Compaction and Reverse Remodeling.

Left ventricular non-compaction (LVNC) is a rare disease defined by morphological criteria, consisting of a two-layered ventricular wall, a thin compacted epicardial layer, and a thick hyper-trabeculated myocardium layer with deep recesses. Controversies still exist regarding whether it is a distinct cardiomyopathy (CM) or a morphological trait of different conditions. This review analyzes data from the literature regarding diagnosis, treatment, and prognosis in LVNC and the current knowledge regarding reverse remodeling in this form of CM. Furthermore, for clear exemplification, we report a case of a 41-year-old male who presented symptoms of heart failure (HF). LVNC CM was suspected at the time of transthoracic echocardiography and was subsequently confirmed upon cardiac magnetic resonance imaging. A favorable remodeling and clinical outcome were registered after including an angiotensin receptor neprilysin inhibitor in the HF treatment. LVNC remains a heterogenous CM, and although a favorable outcome is not commonly encountered, some patients respond well to therapy.

Missense Mutation in Human CHD4 Causes Ventricular Noncompaction by Repressing ADAMTS1.

Left ventricular noncompaction (LVNC) is a prevalent cardiomyopathy associated with excessive trabeculation and thin compact myocardium. Patients with LVNC are vulnerable to cardiac dysfunction and at high risk of sudden death. Although sporadic and inherited mutations in cardiac genes are implicated in LVNC, understanding of the mechanisms responsible for human LVNC is limited. We screened the complete exome sequence database of the Pediatrics Cardiac Genomics Consortium and identified a cohort with a de novo CHD4 (chromodomain helicase DNA-binding protein 4) proband, CHD4M202I, with congenital heart defects. We engineered a humanized mouse model of CHD4M202I (mouse CHD4M195I). Histological analysis, immunohistochemistry, flow cytometry, transmission electron microscopy, and echocardiography were used to analyze cardiac anatomy and function. Ex vivo culture, immunopurification coupled with mass spectrometry, transcriptional profiling, and chromatin immunoprecipitation were performed to deduce the mechanism of CHD4M195I-mediated ventricular wall defects. CHD4M195I/M195I mice developed biventricular hypertrabeculation and noncompaction and died at birth. Proliferation of cardiomyocytes was significantly increased in CHD4M195I hearts, and the excessive trabeculation was associated with accumulation of ECM (extracellular matrix) proteins and a reduction of ADAMTS1 (ADAM metallopeptidase with thrombospondin type 1 motif 1), an ECM protease. We rescued the hyperproliferation and hypertrabeculation defects in CHD4M195I hearts by administration of ADAMTS1. Mechanistically, the CHD4M195I protein showed augmented affinity to endocardial BRG1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4). This enhanced affinity resulted in the failure of derepression of Adamts1 transcription such that ADAMTS1-mediated trabeculation termination was impaired. Our study reveals how a single mutation in the chromatin remodeler CHD4, in mice or humans, modulates ventricular chamber maturation and that cardiac defects associated with the missense mutation CHD4M195I can be attenuated by the administration of ADAMTS1.

Genetic landscape in Russian patients with familial left ventricular noncompaction.

Left ventricular noncompaction (LVNC) cardiomyopathy is a disorder that can be complicated by heart failure, arrhythmias, thromboembolism, and sudden cardiac death. The aim of this study is to clarify the genetic landscape of LVNC in a large cohort of well-phenotyped Russian patients with LVNC, including 48 families (n=214). All index patients underwent clinical examination and genetic analysis, as well as family members who agreed to participate in the clinical study and/or in the genetic testing. The genetic testing included next generation sequencing and genetic classification according to ACMG guidelines. A total of 55 alleles of 54 pathogenic and likely pathogenic variants in 24 genes were identified, with the largest number in the MYH7 and TTN genes. A significant proportion of variants -8 of 54 (14.8%) -have not been described earlier in other populations and may be specific to LVNC patients in Russia. In LVNC patients, the presence of each subsequent variant is associated with increased odds of having more severe LVNC subtypes than isolated LVNC with preserved ejection fraction. The corresponding odds ratio is 2.77 (1.37 -7.37; p <0.001) per variant after adjustment for sex, age, and family. Overall, the genetic analysis of LVNC patients, accompanied by cardiomyopathy-related family history analysis, resulted in a high diagnostic yield of 89.6%. These results suggest that genetic screening should be applied to the diagnosis and prognosis of LVNC patients.

Multimodality Imaging and Biomarker Approach to Characterize the Pathophysiology of Heart Failure in Left Ventricular Non-Compaction with Preserved Ejection Fraction.

We enrolled 21 patients with LVNC and HFpEF and 21 HFpEF controls. For all patients, we performed CMR, speckle tracking echocardiography (STE), and biomarker assessment for HFpEF (NT-proBNP), for myocardial fibrosis (Galectin-3), and for endothelial dysfunction [ADAMTS13, von Willebrand factor, and their ratio]. By CMR, we assessed native T1 and extracellular volume (ECV) for each LV level (basal, mid, and apical). By STE, we assessed longitudinal strain (LS), globally and at each LV level, base-to-apex gradient, LS layer by layer, from epicardium to endocardium, and transmural deformation gradient. In the LVNC group, mean NC/C ratio was 2.9 ± 0.4 and the percentage of NC myocardium mass was 24.4 ± 8.7%. LVNC patients, by comparison with controls, had higher apical native T1 (1061 ± 72 vs. 1008 ± 40 ms), diffusely increased ECV (27.2 ± 2.9 vs. 24.4 ± 2.5%), with higher values at the apical level (29.6 ± 3.8 vs. 25.2 ± 2.8%) (all p < 0.01); they had a lower LS only at the apical level (-21.4 ± 4.4 vs. -24.3 ± 3.2%), with decreased base-to-apex gradient (3.8 ± 4.7 vs. 6.9 ± 3.4%) and transmural deformation gradient (3.9 ± 0.8 vs. 4.8 ± 1.0%). LVNC patients had higher NT-proBNP [237 (156-489) vs. 156 (139-257) pg/mL] and Galectin-3 [7.3 (6.0-11.5) vs. 5.6 (4.8-8.3) ng/mL], and lower ADAMTS13 (767.3 ± 335.5 vs. 962.3 ± 253.7 ng/mL) and ADAMTS13/vWF ratio (all p < 0.05). LVNC patients with HFpEF have diffuse fibrosis, which is more extensive at the apical level, explaining the decrease in apical deformation and overexpression of Galectin-3. Lower transmural and base-to-apex deformation gradients underpin the sequence of myocardial maturation failure. Endothelial dysfunction, expressed by the lower ADAMTS13 and ADAMTS13/vWF ratio, may play an important role in the mechanism of HFpEF in patients with LVNC.

Left Ventricular Non-Compaction Cardiomyopathy: Case Report and Review of Literature

Non-compaction cardiomyopathy (NCC) is characterized by trabeculations in either one or both ventricles. Clinical presentation is highly variable: dyspnea, palpitation, thromboembolic events, arrhythmia, or sudden cardiac death. There are currently no universally-accepted criteria for classifying and diagnosing left ventricular non-compaction (LVNC) cardiomyopathy. Transthoracic echocardiography (TTE) is the diagnostic exam of choice. The diagnosis is often missed or delayed because of a lack of knowledge about this uncommon disease. Progression of LVNC is highly variable and prognosis is very difficult to predict.&#x0D; We report a case of a 50-year-old female patient with a history of total thyroidectomy under hormonal supplementation who consults for dyspnea and paroxysmal palpitations revealing an isolated LVNC.&#x0D; This case emphasizes the importance of imaging techniques, which are, TTE and cardiac magnetic resonance imaging (MRI) in early diagnosis, management, and follow-up.

Altered contractility, Ca2+ transients, and cell morphology seen in a patient-specific iPSC-CM model of Ebstein's anomaly with left ventricular noncompaction.

Patients with two congenital heart diseases (CHDs), Ebstein's anomaly (EA) and left ventricular noncompaction (LVNC), suffer higher morbidity than either CHD alone. The genetic etiology and pathogenesis of combined EA/LVNC remain largely unknown. We investigated a familial EA/LVNC case associated with a variant (p.R237C) in the gene encoding Kelch-like protein 26 (KLHL26) by differentiating induced pluripotent stem cells (iPSCs) generated from affected and unaffected family members into cardiomyocytes (iPSC-CMs) and assessing iPSC-CM morphology, function, gene expression, and protein abundance. Compared with unaffected iPSC-CMs, CMs containing the KLHL26 (p.R237C) variant exhibited aberrant morphology including distended endo(sarco)plasmic reticulum (ER/SR) and dysmorphic mitochondria and aberrant function that included decreased contractions per minute, altered calcium transients, and increased proliferation. Pathway enrichment analyses based on RNASeq data indicated that the "structural constituent of muscle" pathway was suppressed, whereas the "ER lumen" pathway was activated. Taken together, these findings suggest that iPSC-CMs containing this KLHL26 (p.R237C) variant develop dysregulated ER/SR, calcium signaling, contractility, and proliferation.NEW & NOTEWORTHY We demonstrate here that iPSCs derived from patients with Ebstein's anomaly and left ventricular noncompaction, when differentiated into cardiomyocytes, display significant structural and functional changes that offer insight into disease pathogenesis, including altered ER/SR and mitochondrial morphology, contractility, and calcium signaling.

A case report of isolated right ventricular noncompaction with mutation of ACVRL1: a new cause of noncompaction of ventricular myocardium?

BackgroundNoncompaction of ventricular myocardium(NVM) is a rare kind of cardiomyopathy associated with genetic mutations and nongenetic factors, among which the isolated right ventricular noncompaction (iRVNC) is the most rare type. ACVRL1 is the pathogenic gene of type 2 hereditary hemorrhagic telangiectasia (HHT2), and there’s no NVM reported to be associated with ACVRL1 mutation.Case presentationThis is a rare case diagnosed as iRVNC and pulmonary hypertention with ACVRL1 mutation detected.ConclusioniRVNC in this case may be due to ACVRL1 mutation, secondary to pulmonary hypertention and right ventricular failure caused by ACVRL1 mutation, or they happened in the same case coincidently.

(783) LVAD as a Bridge to Candidacy in a Patient with Left Ventricular Noncompaction Cardiomyopathy Complicated by RHF

(783) LVAD as a Bridge to Candidacy in a Patient with Left Ventricular Noncompaction Cardiomyopathy Complicated by RHF

Endothelial deletion of PTBP1 disrupts ventricular chamber development

The growth and maturation of the ventricular chamber require spatiotemporally precise synergy between diverse cell types. Alternative splicing deeply affects the processes. However, the functional properties of alternative splicing in cardiac development are largely unknown. Our study reveals that an alternative splicing factor polypyrimidine tract-binding protein 1 (PTBP1) plays a key role in ventricular chamber morphogenesis. During heart development, PTBP1 colocalizes with endothelial cells but is almost undetectable in cardiomyocytes. The endothelial-specific knockout of Ptbp1, in either endocardial cells or pan-endothelial cells, leads to a typical phenotype of left ventricular noncompaction (LVNC). Mechanistically, the deletion of Ptbp1 reduces the migration of endothelial cells, disrupting cardiomyocyte proliferation and ultimately leading to the LVNC. Further study shows that Ptbp1 deficiency changes the alternative splicing of β-arrestin-1 (Arrb1), which affects endothelial cell migration. In conclusion, as an alternative splicing factor, PTBP1 is essential during ventricular chamber development, and its deficiency can lead to congenital heart disease.

Left ventricular non-compaction: Underdiagnosed disease with fatal outcomes

Left ventricular non compaction (LVNC) is a type of congenital cardiomyopathy characterized by the presence of prominent intertrabecular recesses that typically affect the apical portion of the left ventricular cavity. These recesses give a distinctive phenotype to the affected myocardium that has been described as “sponge-like”. Devastating complications have been observed in those patients with LVNC, including heart failure requiring heart transplantation in selected cases, life-threatening arrhythmias, thromboembolic events including stroke and transient ischemic attack and sudden cardiac death. This report follows the case of a 62-year-old female patient with recurrent chest pain for several years and extensive negative work and recent echocardiography showed increased left ventricle trabeculations, suspicious for LVNC, later confirmed by cardiac magnetic resonance (CMR).

Value of cardiac magnetic resonance feature tracking technology in the differential diagnosis of isolated left ventricular noncompaction and dilated cardiomyopathy.

This study explored the value of myocardial strain in the differential diagnosis of isolated left ventricular myocardial noncompaction (ILVNC) and dilated cardiomyopathy (DCM) using cardiac magnetic resonance (CMR) feature tracking technology. This retrospective analysis was performed on consecutive patients (25 with ILVNC, 30 with DCM, and 30 healthy controls) presenting to Shanxi Cardiovascular Hospital. All ILVNC patients met echocardiographic and CMR criteria for ventricular non-compaction. All patients with DCM met the 2016 American Heart Association and 2018 Chinese Medical Association Cardiovascular Branch diagnostic criteria. cvi42 software (Circle Cardiovascular Imaging) was used to measure radial, circumferential, and longitudinal strain (LS) globally and in segments of the left ventricle. Analysis of variance was used to compare strains among groups and among different segments within the same group. Receiver operating characteristic (ROC) curves were used to evaluate the diagnostic efficacy of different parameters in ILVNC and DCM. Basal circumferential strain was lower in the DCM than in the ILVNC group (P=0.05). Both median and apical LS were lower in the ILVNC than in DCM group (P=0.02 and P=0.01, respectively). ROC curves showed that apical LS was the most effective in distinguishing ILVNC from DCM [area under the curve (AUC) =0.883; P<0.001; 95% CI: 0.850-0.977]. Comparing strains among different segments within the same group revealed that in DCM, the circumferential and LS of the apex were higher than those of the basal segment, which is consistent with the pattern in healthy controls; however, has no such regular pattern was seen in ILVNC. Myocardial strain parameters are of considerable value in the differential diagnosis of ILVNC and DCM. Differences in patterns between ILVNC and DCM can be sensitively identified, providing more comprehensive information for early clinical diagnosis.