MYH7 mutation in a pedigree with familial dilated hypertrophic cardiomyopathy:A case report and review of literature

BACKGROUND Hypertrophic cardiomyopathy(HCM)is one of the most prevalent inherited myocardial disorders and is charac-terized by considerable genetic and phenotypic heterogeneity.A subset of patients with HCM progress to a dilated phase of HCM(DPHCM),which is associated with a poor prognosis;however,the underlying pathogenesis remains inadequately understood.CASE SUMMARY In this study,we present a case involving a pedigree with familial DPHCM and conduct a retrospective review of patients with DPHCM with identified gene mutations.Through panel sequencing targeting the coding regions of 312 genes associated with inherited cardiomyopathy,a heterozygous missense mutation(c.746G>A,p.Arg249Glu)in the MYH7 gene was identified in the proband(III-5).Sanger sequencing subsequently confirmed this pathogenic mutation in three additional family members(II-4,III-4,and IV-3).A total of 26 well-documented patients with DPHCM were identified in the literature.Patients with DPHCM are commonly middle-aged and male.The mean age of patients with DPHCM was 53.43±12.79 years.Heart failure,dyspnoea,and atrial fibrillation were the most prevalent symptoms observed,accompanied by an average left ventricular end-diastolic size of 58.62 mm.CONCLUSION Our findings corroborate the pathogenicity of the MYH7(c.746G>A,p.Arg249Glu)mutation for DPHCM and suggest that the Arg249Gln mutation may be responsible for high mortality.

An improved deep dilated convolutional neural network for seismic facies interpretation

With the successful application and breakthrough of deep learning technology in image segmentation,there has been continuous development in the field of seismic facies interpretation using convolutional neural networks.These intelligent and automated methods significantly reduce manual labor,particularly in the laborious task of manually labeling seismic facies.However,the extensive demand for training data imposes limitations on their wider application.To overcome this challenge,we adopt the UNet architecture as the foundational network structure for seismic facies classification,which has demonstrated effective segmentation results even with small-sample training data.Additionally,we integrate spatial pyramid pooling and dilated convolution modules into the network architecture to enhance the perception of spatial information across a broader range.The seismic facies classification test on the public data from the F3 block verifies the superior performance of our proposed improved network structure in delineating seismic facies boundaries.Comparative analysis against the traditional UNet model reveals that our method achieves more accurate predictive classification results,as evidenced by various evaluation metrics for image segmentation.Obviously,the classification accuracy reaches an impressive 96%.Furthermore,the results of seismic facies classification in the seismic slice dimension provide further confirmation of the superior performance of our proposed method,which accurately defines the range of different seismic facies.This approach holds significant potential for analyzing geological patterns and extracting valuable depositional information.

Bioinformatics Analysis of the Relationship between Dilated Cardiomyopathy and Chronic Heart Failure

Objective: To screen and analyze the differentially expressed genes between dilated cardiomyopathy (DCM) and chronic heart failure (CHF) based on bioinformatics methods. Methods: The Gene Expression Omnibus (GEO) database was used for data retrieval, and the chip data GSE3585 was downloaded, which was the original data of DCM and normal control group. At the same time, the chip data GSE76701 was downloaded, which was the original data of CHF and control group. Differentially expressed mRNAs (DEmRNAs) were screened by R language limma package, the data were standardized, and the common differentially expressed genes were screened. GO function and KEGG pathway enrichment analysis were performed on the common differentially expressed genes. String11.0 online tool was used for data analysis to obtain differentially expressed genes, and the results were imported into Cytoscape 3.9.1 software. The results were imported into Cytoscape 3.9.1 software, and the common expression gene module was obtained by MOCDE algorithm. Nine Hub genes were obtained by 10 algorithms such as MCC. Results: A total of 248 differentially expressed genes were screened. GO analysis showed that differentially expressed genes were mainly concentrated in 9 different physiological and pathological processes. KEGG analysis showed that the main signaling pathways involved in differentially expressed genes were 2, and 9 key differentially expressed genes were predicted: NPPB, NPPA, MYH6, FRZB, ASPN, SFRP4, RPS4Y1, DDX3Y. Conclusion: This study preliminarily explored the molecular mechanism of DCM and CHF, and obtained the common differentially expressed genes of the two diseases. Further experimental studies are needed to verify the correlation between gene expression and clinicopathological features. Provide new ideas for clinical drug treatment research.

Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report

BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro-gressive sensorimotor polyneuropathy,organ enlargement,endocrine disorders,darkening skin,a monoclonal plasma cell proliferative disorder,and lymph node hyperplasia.The organomegaly consists of hepatosplenomegaly and/or lym-phadenopathy;cases of cardiomyopathy are rare.Diagnoses are often delayed because of the atypical nature of the syndrome,exposing patients to possibly severe disability.Therefore,identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.lenalidomide and dexamethasone.CONCLUSION When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin,the POEMS syndrome is the most possible diagnosis.

Construction of A Prediction Model for Atrial Fibrillation in Patients with Dilated Cardiomyopathy and Heart Failure

Dilated cardiomyopathy(DCM)is a common myocardial disease characterized by enlargement of the heart cavity and decreased systolic function,often leading to heart failure(HF)and arrhythmia.The occurrence of atrial fibrillation(AF)is closely related to the progression and prognosis of the disease.In recent years,with the advancement of medical imaging and biomarkers,models for predicting the occurrence of AF in DCM patients have gradually become a research hotspot.This article aims to review the current situation of AF in DCM patients and explore the importance and possible methods of constructing predictive models to provide reference for clinical prevention and treatment.We comprehensively analyzed the risk factors for AF in DCM patients from epidemiological data,pathophysiological mechanisms,clinical and laboratory indicators,electrocardiogram and imaging parameters,and biomarkers,and evaluated the effectiveness of existing predictive models.Through analysis of existing literature and research,this article proposes a predictive model that integrates multiple parameters to improve the accuracy of predicting AF in DCM patients and provide a scientific basis for personalized treatment.

Multi-scale Attention Dilated Residual Image Denoising Network Based on Skip Connection

In the field of image denoising, deep learning technology holds a dominance. However, the current network model tends to lose fine-grained information with the depth of the network. To address this issue, this paper proposes a Multi-scale Attention Dilated Residual Image Denoising Network(MADRNet) based on skip connection, which consists of Dense Interval Transmission Block(DTB), Sparse Residual Block(SRB), Dilated Residual Attention Reconstruction Block(DRAB) and Noise Extraction Block(NEB). The DTB enhances the classical dense layer by reducing information redundancy and extracting more accurate feature information. Meanwhile, SRB improves feature information exchange and model generalization through the use of sparse mechanism and skip connection strategy with different expansion factors. The NEB is primarily responsible for extracting and estimating noise. Its output, together with that of the sparse residual module, acts on the DRAB to effectively prevent loss of shallow feature information and improve denoising effect. Furthermore, the DRAB integrates an dilated residual block into an attention mechanism to extract hidden noise information while using residual learning technology to reconstruct clear images. We respectively examined the performance of MADRNet in gray image denoising, color image denoising and real image denoising. The experiment results demonstrate that proposed network outperforms some excellent image denoising network in terms of peak signal-to-noise ratio, structural similarity index measurement and denoising time. The proposed network effectively addresses issues associated with the loss of detail information.

A novel mitochondrial tRNA gene mutation in a chinese family with dilated cardiomyopathy and sensorineural deafness

Objective： To determine whether a mutation of mitochondrial DNA induces familial dilated cardiomyopathy in Chinese families with cardiomyopathy, and analyzed the correlation between the genotype and phenotype. Methods： Affected members in three Chinese families of the familial dilated cardiomyopathy underwent clinical evaluation and DNA analysis. Polymerase chain reaction and direct DNA sequencing were used to screen for mitochondrial DNA mutation. The type of mtDNA variations and clinical situation were analysed on the patients with mitochondrial DNA mutation. Results： The mitochondrial A3434G mutation was identified in one of the three families,the 3434 th nucleotide A was replaced by G, which led to change of amino acid. No mutations were identified in the clinically unaffected members of the family and all members of the other two families. Conclusion： This study indicates that the mitochondrial A3434G mutation maybe related with familial dilated cardiomyopathy and deafness.

1D-CNN:Speech Emotion Recognition System Using a Stacked Network with Dilated CNN Features

Emotion recognition from speech data is an active and emerging area of research that plays an important role in numerous applications,such as robotics,virtual reality,behavior assessments,and emergency call centers.Recently,researchers have developed many techniques in this field in order to ensure an improvement in the accuracy by utilizing several deep learning approaches,but the recognition rate is still not convincing.Our main aim is to develop a new technique that increases the recognition rate with reasonable cost computations.In this paper,we suggested a new technique,which is a one-dimensional dilated convolutional neural network(1D-DCNN)for speech emotion recognition(SER)that utilizes the hierarchical features learning blocks(HFLBs)with a bi-directional gated recurrent unit(BiGRU).We designed a one-dimensional CNN network to enhance the speech signals,which uses a spectral analysis,and to extract the hidden patterns from the speech signals that are fed into a stacked one-dimensional dilated network that are called HFLBs.Each HFLB contains one dilated convolution layer(DCL),one batch normalization(BN),and one leaky_relu(Relu)layer in order to extract the emotional features using a hieratical correlation strategy.Furthermore,the learned emotional features are feed into a BiGRU in order to adjust the global weights and to recognize the temporal cues.The final state of the deep BiGRU is passed from a softmax classifier in order to produce the probabilities of the emotions.The proposed model was evaluated over three benchmarked datasets that included the IEMOCAP,EMO-DB,and RAVDESS,which achieved 72.75%,91.14%,and 78.01%accuracy,respectively.

Identification of a LMNA (c.646C>T) variant by whole-exome sequencing in combination with a dilated cardiomyopathy (DCM) related gene filter in a family with familiar DCM

Dilated cardiomyopathy（DCM）is characterized by the dilated heart chambers and reduced systolic function in the absence of specific aetiology[1].Approximately one third of DCM cases are hereditary.In recent years,DCM concomitant with arrhythmias and sudden death resulting from gene mutation has been widely

Rare combination of dilated cardiomyopathy and ankylosing spondylitis in a family

A 65-year-old man presented to our cardiovascular department due to fatigue and palpation on exertion during the previous three weeks. He had a medical history of diabetes mellitus and hyperlipemia without hypertension or myocarditis. However, he mentioned that his son had dilated cardiomyopathy （DCM） and ankylosing spondylitis （AS）. Examination at admission revealed a blood pressure of 115/69 mmHg and pulse of 82 beats/min. The results of routine blood tests for creatine kinase （CK）, CK muscle and brain （CK-MB）, troponin T, and thyroid function were all within normal limits. ECG revealed I, aVL, and V4--6 T wave inversion （Figure 1）. Ultrasonic cardiography showed that the left ventricular ejection fraction （EF） was 32%, in- dicating ventricular wall dysfunction. Angiography showed no coronary stenosis. Radionuclide imaging demonstrated myopathy of the anterior, part of the inferior, the posterior,and the lateral apical segments based on reduced radioactivity with a patchy pattern, consistent with DCM （Figure 2A）.

Clinical outcome of cardiac resynchronization therapy in dilated-phase hypertrophic cardiomyopathy

Backgrounds Clinical trials have demonstrated that cardiac resynchronization therapy （CRT） is effective in patients with ＂non-is- chemic cardiomyopathy＂. However, patients with dilated-phase hypertrophic cardiomyopathy （DHCM） have been generally excluded from such trials. We aimed to compare the clinical outcome of CRT in patients with DHCM, idiopathic dilated cardiomyopathy （IDCM）, or ischemic cardiomyopathy （ICM）. Methods A total of 312 consecutive patients （DHCM： n = 16; IDCM： n = 231; ICM： n = 65） undergoing CRT in Fuwai hospital were studied respectively. Response to CRT was defmed as reduction in left ventricular end-systolic volume （LVESV） _〉 15% at 6-month follow-up. Results Compared with DHCM, IDCM was associated with a lower total mortality （HR： 0.35, 95% CI： 0.13-0.90）, cardiac mortality （HR： 0.29; 95% CI： 0.11-0.77）, and total mortality or heart failure （HF） hospitalizations （HR： 0.34, 95% CI： 0.17-0.69）, independent of known confounders. Compared with DHCM, the total mortality, cardiac mortality and total mortality or HF hospitalizations favored ICM but were not statistically significant （HR： 0.59, 95% CI： 0.22-1.61; HR： 0.59, 95% CI： 0.21-1.63; HR： 0.54, 95% CI： 0.26-1.15; respectively）. Response rate to CRT was lower in the DHCM group than the other two groups although the differences didn＇t reach statistical significance. Conclusions Compared with IDCM, DHCM was associated with a worse outcome after CRT. The clinical outcome of DHCM patients receiving CRT was similar to or even worse than that of ICM patients. These indicate that DHCM behaves very differently after CRT.

TCM Treatment of Hypertrophic Cardiomyopaphy—A Case Report

Case History Patient Wu, aged 66 years, was admitted to this hospital on February 28, 1997 due to paroxysmal chest distress, palpitation and shortness of breath for more than 15 years and exacerbation of these symptoms in recent 3 months.

Cytotoxic T lymphocyte associated antigen-4 gene exon 1A49→G polymorphisms confer susceptibility to idiopathic dilated cardiomyopathy

Autoimmune mechanisms, including cellular and humoral immune, are likely to participate in the pathogenesis of at least a subgroup of idiopathic dilated cardiomyopathy （IDC）, in which cellular immune-mediation plays a more important role. Cytotoxic T lymphocyte associated antigen-4 （CTLA-4） is the major negative regulatory factor of cellular immunity. This study was conducted to investigate the association of CTIA-4 gene exon 1 A49→G polymorphism with susceptibility to IDC in Han Chinese and its influences on serum soluble CTIA-4 （sCrLA-4） and Th1/Th2 cytokine bias. Polymerase chain reaction-restriction fragment length polymorphism （PCR-RFLP） techniques were used to analyze the dimorphism of CTL4-4 exon 1 in the unrelated Han ethnic population in Heilongjiang Province （ including 48 IDC patients and 50 normal controls）. Serum sCTLA-4, IFN-γ and IL-4 were evaluated by ELISA, with the ratio of IFN-γ/IL-4 as indicator for Th1/Th2 bias. Compared with controls, the frequencies of GG genotype （0.6042 and 0.3600, P = 0.012） and the G allele （0.7396 and 0.5600, P = 0.008） were significantly increased in IDC patients. Increased serum sCTIA-4 was found in the 1DC group compared with the controls [ （ 1.87 ± 1.06） μg/L vs. （0.54 ± 0.19） 〉g/L, P 〈 0.05 ~. 1FN-7 was much lower in IDC patients than that of the controls [ （ 16.38 ± 6.25） ng/L vs. （29.81 ± 10.66） ng/L （P 〈 0.05）~., whereas no statistical difference of IL-4 was found between the two groups I （12.85 ± 1.86） ng/L vs. （12.11 ± 2.76） ng/L], so the ratio of IFN-γ/IL-4 declined significantly （ 1.63 ± 0.50 vs. 3.01 ± 0.89, P 〈 0.05）. Linear regression analysis manifested a significant interrelationship between the GG genotype, G allele frequencies and serum sCTLA-4, IFN-γ/IL-4 in the IDC group （ r = 0.57, P = 0. 021 and r = 0.32, P = 0. 036）. CTLA-4 gene A49→G substitution was associated with an increased IDC risk, which implicated that the CTLA-4 gene exon 1 may have a considerable role in autoimmune cardiac damage, possibly via a Thr→Ala change in signal peptide, which influences the protein synthesis and modification processes, with a result of functional alteration of sCTLA-4. The bias of Th1/Th2 paradigm was associated with the increased sCTIA-4 under certain background of immunogenetics.

HLA-DQA1 Polymorphism in Idiopathic Dilated Cardiomyopathy

To determine whether the possession of certain HLA-DQA1 alleles was associated with the risk of developing idiopathic dilated cardiomyopathy (IDC) and to substantiate the role of an autoimmunologic pathogenesis in IDC. Type the alleles of HLA-DQA1 by polymerase chain reaction with sequence-specific primers (PCR-SSP) technique in 38 patients of idiopathic dilated cardiomyopathy (7 women and 31 men), aged from 17 to 56 years old with diagnosis being according to World Health Organization criteria (IDC group), in 50 patients of end-stage heart failure of known etiology (18 women and 32 men), with ages ranging from 34 to 72(HF group), and in the control group consisting of presumably 100 healthy subjects (39 women and 61 men) from the health survey, aged from 30 to 59 years old. The frequency of HLA-DQA1*0501 in the DCM patients was significantly elevated than that in the HF and the control group. Molecular analysis of the DQA1 gene polymorphism performed in the three subgroups shows an increased frequency of DQA1*0501 among patients with less EF. The HF group carries a high frequency of HLA-DQA1*0301. An increased frequency of DQA1*0201 and DQA1*0103 was found in the control group. HLA-DQA1*0501 is an associated gene of idiopathic dilated cardiomyopathy and the possession of DQA1*0301 may be indicative of the known etiologic heart failure, suggesting that the mechanisms involved in the pathogenesis of IDC and otherwise heart failure are different. Immunologic abnormalities may be a major contributor to the susceptibility of developing of IDC.

EFFECTS OF EPCs OR b-FGF INTRAMYOCARDIAL INFUSION ON CARDIAC FUNCTION AND NEOVASCULARIZATION FOR DILATED CARDIOMYOPATHY RATS

Objective To compare the different effects of endothelia progenitor cells （ EPCs ） or basic fibroblast growth factor （b-FGF） intromyocardial infusion on cardiac function and neovascularization for dilated cardiomyopathy（ DCM） rats. Methods Fifty adult female rats received inguinal subcutaneous injections of isoproterenol （ISO, 250 mg/kg） for induction of DCM. Four weeks later, the model rats were randomly divided into EPCs group, b-FGF group and control group. The 2×106 EPCs （ resolved in 100 μL PBS） , 100 μL b-FGF （ lO0 μg/mL ） and 100 μL PBS were evenly transplanted into the myocardium of EPCs group, b-FGF group and control group, respectively. Three months later, echocardiographic examination and regional myocardial blood flow （RMBF） measurement were performed. EPCs were traced by fluorescence in situ hybridization （FISH）. The protein and mRNA expression of b-FGF in each group was measured by ELISA assay and reverse transcription-polymerase chain reaction （ RT-PCR ） . Results Three months after transplantation, sry positive cells were detected only in EPCs group. The cardiac function as well as RMBF was significantly improved in EPCs group compared with b-FGF group or control group. There was higher capillary density in EPCs group. The protein and mRNA expression of b-FGF was stronger than b-FGF group and control group. Conclusion Transplantation of EPCs can improve cardiac function, induce neovascularization and increase RMBF for DCM rats. The treatment with EPCs has better effect than administration of b-FGF alone.

Mechanism of Anti-β-adrenoceptor Antibody Mediated Myocardial Damage in Dilated Cardiomyopathy

Antibodies against &-adrenoceptor can be detected in serum of patients with dilated cardiomyopathy (DCM), which have 5-agonist-like activity, and induce a positive chronotropic effect on cardiac myocytes by its persistence at full strength. Effects of the antibodies against Padrenoceptor from sera of patients with DCM on myocardial cytotoxicity and cytoplasmic free Ca2+-concentration (LCa2+ji) were observed in the cultured single layer SD rat ventricular cells by using the cytotoxicity assay and fluorescent Ca2+- indicat0r fura-2/AM. The positive sera of the anti-&adrenoceptor antibodies from patients with DCM markedly enhanced myocardial [Ca2+]i. Betaloc, a 5, -receptor blocker, might inhibit the increase of the antibody-mediated myocardial [Ca2+]i, and the sera from healthy donors had no effect on myocardial [Ca2+]i,. Our results suggest that the anti-β-adrenoceptor antibody might increase myocardial [Ca2+]i, and result in myocardial damage. The antibodies might activate receptor-gating Ca2+-channel, thereby causing myocardial [Ca2+]i, rise and calcium overload. Early use of betaloc is recommended in the treatment of dilated cardiomyopathy.

D-dimer level and long-term outcome in patients with end-stage heart failure secondary to idiopathic dilated cardiomyopathy

Background Previous studies had demonstrated hemostatic abnormalities in patients with heart failure (HF) and several studies have shown that abnormal coagulation indices, represented by elevated D-dimer, had prognostic significance in patients with compatible or acute decompensated HF. However, the impact of D-dimer on the outcome in patients with end-stage HF remains unclear. Methods A total of 244 consecutive patients with end-stage HF due to idiopathic dilated cardiomyopathy (DCM) were prospectively enrolled from February 2011 to September 2014. D-dimer levels were measured and its prognostic value was assessed. Primary endpoint was all-cause mortality during the follow-up period. Secondary endpoints were stroke, bleeding, occurrence of sustained ventricular tachycardia or ventricular fibrillation, and major adverse cardiovascular events (MACE). Results D-dimer was significantly elevated in the non-survivors (median: 0.8 vs. 1.1 mg/L, P < 0.001). Traditional markers including B-type natriuretic peptide, troponin I, left ventricular ejection fraction, and left ventricular end-diastolic dimension provided limited prognostic value;but the addition of D-dimer refined the risk stratification. The optimal cut-off value of D-dimer to predict all-cause mortality was 0.84 mg/L by receiver operator characteristic analysis. Elevated D-dimer level was independently associated with increased risk of long-term all-cause mortality (HR = 2.315, 95% CI: 1.570–3.414, P < 0.001) and MACE (HR = 1.256, 95% CI: 1.058–1.490, P = 0.009), and the predictive value was independent of age, sex, atrial fibrillation and anticoagulation status. Conclusions Elevated D-dimer level was independently associated with poor long-term outcome in patients with end-stage HF secondary to idiopathic DCM, and the predictive value was superior to that of traditional prognostic markers.

Clinical characteristics, treatment and prognosis of patients with idiopathic dilated cardiomyopathy: a tertiary center experience

Background Contemporary heart failure medications have led to considerable improvement in the survival of patients with heart failure. However,limited evidence is available regarding the effect of those medications in patients with idiopathic dilated cardiomyopathy (IDCM),particularly in China. We sought to analyze the trends in clinical characteristics and the prescription rate of recommended therapies and its prognostic impact in patients with IDCM. Methods From 2009 to 2016,1441 consecutive patients (age: 55±14 years,68% men,LVEF: 33%± 12%) fulfilling World Health Organization criteria for IDCM were enrolled in the current retrospective cohort study. Temporal trends of baseline clinical characteristics,treatment and prognosis were analyzed,and potential influential factors were explored. Results Rates of patients receiving angiotensin-converting enzyme inhibitors/angiotensin II receptors blockers,β-blockers,aldosterone receptor antagonists and diuretics increased from 55%,45%,58%,51% in 2009 to 67%,69%,71%,64% in 2016,respectively (P < 0.05);whereas,the proportion of patients receiving digoxin decreased from 39% in 2009 to 28% in 2016 (P < 0.05). The overall proportion of patients with optimal guideline-directed medical therapy (GDMT) was 44.6%;however,that rate increased from 33% in 2009 to 41%,49% and 56% in 2012,2014 and 2016 respectively (P < 0.05). Patients with optimal GDMT had a better outcome than those without,but there was no temporal trend toward improvement in the overall long-term prognosis of IDCM patients with the years. There was a trend towards admission of patients with milder disease and toward increased admission to a cardiology ward with the years. Conclusions An improvement in prescription rates of guideline-recommended medications in IDCM patients was observed. However,it remains suboptimal,and there is still some room for improvement. The prognosis of patients with optimal GDMT was better than those without. Moreover,the following patient category also had an improved prognosis: patients with LVEF ≥ 40%,with device therapy,and those admitted to a cardiology ward.

Non-invasive evaluation of arrhythmic risk in dilated cardiomyopathy:From imaging to electrocardiographic measures

Malignant ventricular arrhythmias are a major adverse event and worsen the prognosis of patients affected by ischemic and non-ischemic dilated cardiomyopathy.The main parameter currently used to stratify arrhythmic risk and guide decision making towards the implantation of a cardioverter defibrillator is the evaluation of the left ventricular ejection fraction.However,this strategy is characterized by several limitations and consequently additional parameters have been suggested in order to improve arrhythmic risk stratification.The aim of this review is to critically revise the prognostic significance of non-invasive diagnostic tools in order to better stratify the arrhythmic risk prognosis of dilated cardiomyopathy patients.

Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.