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...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.展开更多
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 fibr...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.展开更多
BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic h...BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic heart failure.However,acute heart failure(AHF)without obvious associated triggers is rare.Prior to the report of endomyocardial biopsy,the diagnosis and treatment of EFE are highly susceptible to being confounded with other primary cardiomyopathies.Here,we report a case of pediatric AHF caused by EFE mimicking dilated cardiomyopathy(DCM),with the aim of providing a valuable reference for clinicians to early identify and diagnose EFE-induced AHF.CASE SUMMARY A 13-mo-old female child was admitted to hospital with retching.Chest X-ray demonstrated enhanced texture in both lungs and an enlarged heart shadow.Color doppler echocardiography showed an enlarged left heart with ventricular wall hypokinesis and decreased left heart function.Abdominal color ultrasonography revealed a markedly enlarged liver.Pending the result of the endomyocardial biopsy report,the child was treated with a variety of resuscitative measures including nasal cannula for oxygen,intramuscular sedation with chlorpromazine and promethazine,cedilanid for cardiac contractility enhancement,and diuretic treatment with furosemide.Subsequently,the child’s endomyocardial biopsy report result was confirmed as EFE.After the above early interventions,the child’s condition gradually stabilized and improved.One week later,the child was discharged.During a 9-mo follow-up period,the child took intermittent low-dose oral digoxin with no signs of recurrence or exacerbation of the heart failure.CONCLUSION Our report suggests that EFE-induced pediatric AHF may present in children over 1 year of age without any apparent precipitants,and that the associated clinical presentations are grossly similar to that of pediatric DCM.Nonetheless,it is still possible to be diagnosed effectively on the basis of the comprehensive analysis of auxiliary inspection findings before the result of the endomyocardial biopsy is reported.展开更多
BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients wi...BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients with DCM contributes to variable disease severity and complicates overall prognosis,which can be very poor.AIM To identify pathogenic genes in DCM through pedigree analysis.METHODS Our research team identified a patient with DCM in the clinic.Through invest-igation,we found that the family of this patient has a typical DCM pedigree.High-throughput sequencing technology,next-generation sequencing,was used to sequence the whole exomes of seven samples in the pedigree.RESULTS A novel and potentially pathogenic gene mutation-ANK2p.F3067L-was discovered.The mutation was completely consistent with the clinical information for this DCM pedigree.Sanger sequencing was used to further verify the locus of the mutation in pedigree samples.These results were consistent with those of high-throughput sequencing.CONCLUSIONS ANK2p.F3067L is considered a novel and potentially pathogenic gene mutation in DCM.展开更多
Background:Various physiological mechanisms are linked to dilated cardiomyopathy(DCM)development,including oxidative stress,immune irregularities,inflammation,fibrosis,and genetic changes.However,precise molecular dri...Background:Various physiological mechanisms are linked to dilated cardiomyopathy(DCM)development,including oxidative stress,immune irregularities,inflammation,fibrosis,and genetic changes.However,precise molecular drivers of DCM,especially regarding abnormal immune responses,remain unclear.This study investigates immune-related long non-coding RNAs(lncRNAs)in DCM’s diagnostic and therapeutic potential.Methods:GSE141910,GSE135055,and GSE165303 datasets were acquired from the GEO database.LASSO,SVM-RFE,and random forest algorithms identified DCM-associated immune-related lncRNAs.Diagnostic capabilities were assessed by Nomogram and receiver operating characteristic(ROC)curves.Multivariate linear regression explored lncRNA correlations with ejection fraction.Single-sample gene set enrichment analysis(ssGSEA)gauged immune cell infiltration/functions.Functional enrichment analyses were performed using Gene set variation analysis(GSVA),gene ontology(GO),and the Kyoto Encyclopedia of Genes and Genomes(KEGG).Consensus clustering categorized DCM cases.Results:Ten immune-related lncRNAs emerged:C10orf71-AS1,FHAD1-AS1,SCIRT,FNDC1-AS1,MELTFAS1,LOC101928834,GDNF-AS1,DCXR-DT,C3orf36,and LOC107985323.These lncRNAs,tied to immunomodulation,showed promising DCM diagnostic accuracy.Adjusted for confounders,they independently correlated with ejection fraction.Using lncRNA expression,DCM patients were grouped into subtypes.Subtype C1 displayed a higher level of immune cell infiltration and immune checkpoint expression compared to subtype C2,emphasizing the variations in the immune microenvironment.Conclusion:This study identifies ten immune-related lncRNAs for further exploration in DCM diagnosis and subtyping.Based on expression patterns,we propose two potential DCM subtypes.Notably,findings are preliminary and hypothesis-generating,demanding validation and further investigation.This research provides insights into DCM diagnosis and classification.展开更多
BACKGROUND The prognostic value of late gadolinium enhancement(LGE)derived from cardiovascular magnetic resonance(CMR)is well studied,and several new metrics of LGE have emerged.However,some controversies remain;there...BACKGROUND The prognostic value of late gadolinium enhancement(LGE)derived from cardiovascular magnetic resonance(CMR)is well studied,and several new metrics of LGE have emerged.However,some controversies remain;therefore,further discussion is needed,and more precise risk stratification should be explored.AIM To investigate the associations between the positivity,extent,location,and pattern of LGE and multiple outcomes in dilated cardiomyopathy(DCM).METHODS PubMed,Ovid MEDLINE,and Cochrane Library were searched for studies that investigated the prognostic value of LGE in patients with DCM.Pooled hazard ratios(HRs)and 95%confidence intervals were calculated to assess the role of LGE in the risk stratification of DCM.RESULTS Nineteen studies involving 7330 patients with DCM were included in this metaanalysis and covered a wide spectrum of DCM,with a mean left ventricular ejection fraction between 21%and 50%.The meta-analysis revealed that the presence of LGE was associated with an increased risk of multiple adverse outcomes(all-cause mortality,HR:2.14;arrhythmic events,HR:5.12;and composite endpoints,HR:2.38;all P<0.001).Furthermore,every 1%increment in the extent of LGE was associated with an increased risk of all-cause mortality.Analysis of a subgroup revealed that the prognostic value varied based on different location and pattern of LGE.Additionally,we found that LGE was a stronger predictor of arrhythmic events in patients with greater left ventricular ejection fraction.CONCLUSION LGE by CMR in patients with DCM exhibited a substantial value in predicting adverse outcomes,and the extent,location,and pattern of LGE could provide additional information for risk stratification.展开更多
Myocardial fiber deformation measurements have been reported to be associated with adverse outcomes in patients with acute heart failure and those with myocardial infarction.However,few studies have addressed the prog...Myocardial fiber deformation measurements have been reported to be associated with adverse outcomes in patients with acute heart failure and those with myocardial infarction.However,few studies have addressed the prognostic value of global circumferential strain(GCS)in dilated cardiomyopathy(DCM)patients with severely impaired systolic function.This study aimed to evaluate the prognostic value of cardiac magnetic resonance(CMR)-derived GCS in DCM patients with severely reduced ejection.Consecutive DCM patients with severely reduced ejection fraction(EF<35%)who underwent CMR were included.GCS was calculated from CMR cine images.The clinical endpoint was a composite of all-cause mortality,heart transplantation,implantable cardioverter defibrillator(ICD)implantation and aborted sudden cardiac death(SCD).A total of 129 patients with a mean EF of 15.33%(11.36%–22.27%)were included.During a median follow-up of 518 days,endpoint events occurred in 50 patients.Patients with GCS≥the median(−5.17%)had significantly reduced event-free survival as compared with those with GCS<the median(P<0.01).GCS was independently associated with adverse events after adjusting for clinical and imaging risk factors including extent of late gadolinium enhancement(LGE)(P<0.05).Adding GCS into the model including the extent of LGE resulted in significant improvements in the C-statistic(from 0.706 to 0.742;P<0.05)with a continuous net reclassification improvement(NRI)of 29.71%.It was concluded that GCS derived from CMR could be useful for risk stratification in DCM patients with severely reduced EF,which may increase common imaging risk factors including LGE.展开更多
BACKGROUND Nemaline myopathy(NM)is a rare type of congenital myopathy,with an incidence of 1:50000.Patients with NM often exhibit hypomyotonia and varying degrees of muscle weakness.Skeletal muscles are always affecte...BACKGROUND Nemaline myopathy(NM)is a rare type of congenital myopathy,with an incidence of 1:50000.Patients with NM often exhibit hypomyotonia and varying degrees of muscle weakness.Skeletal muscles are always affected by this disease,while myocardial involvement is uncommon.However,with improvements in genetic testing technology,it has been found that NM with a mutation in the myopalladin(MYPN)gene not only causes slow,progressive muscle weakness but also results in dilated or hypertrophic cardiomyopathy.CASE SUMMARY A 3-year-old pre-school boy was admitted to our hospital with cough,edema,tachypnea,and an increased heart rate.The patient was clinically diagnosed with severe dilated cardiomyopathy and heart failure,and subsequent gene examination confirmed the diagnosis of NM with a mutation in MYPN.Captopril,diuretics,low-dose digoxin,and dobutamine were administered.After 22 d of hospitalization,the patient was discharged due to the improvement of clinical symptoms.During the follow-up period,the patient died of refractory heart failure.CONCLUSION Decreased muscular tone and dilated cardiomyopathy are common features of MYPN-mutated NM.Heart transplantation may be a solution to this type of cardiomyopathy.展开更多
Background: The aim of this study was to determine the relation of left atrial (LA) volume and LA volume index with left ventricular function and to determine the association of duration of symptoms and left atrial vo...Background: The aim of this study was to determine the relation of left atrial (LA) volume and LA volume index with left ventricular function and to determine the association of duration of symptoms and left atrial volume index in patients with dilated cardiomyopathy. Materials and Methods: This was an observational, single centre study conducted in India. A total of 50 patients who were admitted to department of cardiology from July, 2008 to February, 2009 with diagnosis of dilated cardiomyopathy and an ejection fraction of <40% were included. Results: Of the 50 patients, 34 (68%) were males. 27 (54%) patients were in NYHA class II and 23 (46%) patients were in NYHA class III. LA volume was found to be ≥40 ml in all patients. LV function and LA volume were found to be correlated (r = -0.789, p < 0.01). Similarly, there was a correlation between LV function and LA volume index (r = -0.826, p < 0.01). There was no correlation between LA volume index and duration of symptoms (r = 0.04). Conclusion: It can be concluded that there is a strong inverse correlation between LA volume and left ventricular function and also between LA volume index and left ventricular function. The patients with NYHA class III were having larger left atrial volume than those with NYHA class II. Moreover, the duration of symptoms has no correlation with left atrial volume index.展开更多
Myocardial segmentation and classification play a major role in the diagnosis of cardiovascular disease.Dilated Cardiomyopathy(DCM)is a kind of common chronic and life-threatening cardiopathy.Early diagnostics signifi...Myocardial segmentation and classification play a major role in the diagnosis of cardiovascular disease.Dilated Cardiomyopathy(DCM)is a kind of common chronic and life-threatening cardiopathy.Early diagnostics significantly increases the chances of correct treatment and survival.However,accurate and rapid diagnosis of DCM is still challenge due to high variability of cardiac structure,low contrast cardiac magnetic resonance(CMR)images,and intrinsic noise in synthetic CMR images caused by motion artifact and cardiac dynamics.Moreover,visual assessment and empirical evaluation are widely used in routine clinical diagnosis,but they are subject to high inter-observer variability and are both subjective and non-reproducible.To solve this problem,we proposed an effective unified multi-task framework for dilated cardiomyopathy CMR segmentation and classification simultaneously,and we firstly update one independent encoder from both recovery decoder and parallel attention path sharing some partial weights.This can encode both task choices into good embedding,but each one can achieve significant improvements respectively from the given embedding.It consists of three branches:extraction path,attention path,and recovery path,which allows the model to learn more higher-level intermediate representations and makes a more accurate prediction.We validated our approach on a DCM dataset,which contains 1155 CMR LGE images.Experimental results show that our multi-task network has achieved accuracy of 97.63%,AUC of 98.32%,demonstrating effectively segmenting the myocardium,quickly and accurately diagnosing the presence or absence of dilation.展开更多
BACKGROUND The clinical course of acute myocarditis ranges from the occurrence of a few symptoms to the development of fatal fulminant myocarditis.Specifically,fulminant myocarditis causes clinical deterioration very ...BACKGROUND The clinical course of acute myocarditis ranges from the occurrence of a few symptoms to the development of fatal fulminant myocarditis.Specifically,fulminant myocarditis causes clinical deterioration very rapidly and aggressively.The long-term prognosis of myocarditis is varied,and it fully recovers without leaving any special complications.However,even after recovery,heart failure may occur and eventually progress to dilated cardiomyopathy(DCM),which causes serious left ventricular dysfunction.In the case of follow-up observation,no clear guidelines have been established.CASE SUMMARY We report the case of a 21-year-old woman who presented with dyspnea.She became hemodynamically unstable and showed sustained fatal arrhythmias with decreased heart function.She was clinically diagnosed with fulminant myocarditis based on her echocardiogram and cardiac magnetic resonance results.After 2 d,she was readmitted to the emergency department under cardiopulmonary resuscitation and received mechanical ventilation and extracorporeal membrane oxygenation.An implantable cardioverter defibrillator was inserted for secondary prevention.She recovered and was discharged.Prior to being hospitalized for sudden cardiac function decline and arrhythmia,she had been well for 7 years without any complications.She was finally diagnosed with dilated cardiomyopathy.CONCLUSION DCM may develop unexpectedly in patients who have been cured of acute fulminant myocarditis and have been stable with a long period of remission.Therefore,they should be carefully and regularly observed clinically throughout long-term follow-up.展开更多
Background:Signifi cant left ventricular dysfunction may arise in right-sided accessory pathways with ventricular preexcitation in the absence of recurrent or incessant tachycardia.This has just been realized and not ...Background:Signifi cant left ventricular dysfunction may arise in right-sided accessory pathways with ventricular preexcitation in the absence of recurrent or incessant tachycardia.This has just been realized and not enough attention has been paid to it.Methods:In the last 7 years,we identifi ed 12 consecutive children with a diagnosis of ventricular preexcitation–induced dilated cardiomyopathy.This report describes the clinical and echocardiographic characteristics of the patients before and after ablation.Results:Dyssynchronous ventricular contraction was observed by M-mode echocardiography and two-dimensional strain analysis in all patients.The basal and middle segments of the interventricular septum became thin and moved similarly to an aneurysm,with typical bulging during the end of systole.The locations of the accessory pathways were the right-sided septum(n=5)and the free wall(n=7).Left ventricular synchrony was obtained shortly after ablation.The left ventricular function recovered to normal and the left ventricular end-diastolic diameter decreased gradually during follow-up.Conclusions:A causal relationship between ventricular preexcitation and the development of dilated cardiomyopathy is supported by the complete recovery of left ventricular function and reversed left ventricular remodeling after the loss of ventricular preexcitation.Preexcitation-related dyssynchrony was thought to be the crucial mechanism.Ventricular preexcitation–induced dilated cardiomyopathy is an indication for ablation with a good prognosis.展开更多
BACKGROUND Alstr?m syndrome(AS)is a rare autosomal recessive disease that is generally induced by mutations of the Alstr?m syndrome 1(ALMS1)gene.We report a case of AS,extend the spectrum of ALMS1 mutations and highli...BACKGROUND Alstr?m syndrome(AS)is a rare autosomal recessive disease that is generally induced by mutations of the Alstr?m syndrome 1(ALMS1)gene.We report a case of AS,extend the spectrum of ALMS1 mutations and highlight the biological role of ALMS1 to explore the relationship between dilated cardiomyopathy(DCM)and mutations in ALMS1.CASE SUMMARY We present the case of an infant with AS mainly manifesting with DCM that was caused by a novel mutation of the ALMS1 gene.Whole-exome sequencing revealed a simultaneous large deletion and point mutation in ALMS1,leading to frameshift and missense mutations,respectively,rather than nonsense or frameshift mutations,which have been reported previously.Upon optimized anti-remodeling therapy,biohumoral exams and arrhythmic burden of the infant were alleviated at follow-up after 6 mo.CONCLUSION We identified novel mutations of ALMS1 and extended the spectrum of ALMS1 mutations in an infant with AS.展开更多
BACKGROUND Heart failure is a health burden responsible for high morbidity and mortality worldwide, and dilated cardiomyopathy(DCM) is one of the most common causes of heart failure. DCM is a disease of the heart musc...BACKGROUND Heart failure is a health burden responsible for high morbidity and mortality worldwide, and dilated cardiomyopathy(DCM) is one of the most common causes of heart failure. DCM is a disease of the heart muscle and is characterized by enlargement and dilation of at least one ventricle alongside impaired contractility with left ventricular ejection fraction < 40%. It is also associated with abnormalities in cytoskeletal proteins, mitochondrial ATP transporter, microvasculature, and fibrosis. However, the pathogenesis and potential biomarkers of DCM remain to be investigated.AIM To investigate the candidate genes and pathways involved in DCM patients.METHODS Two expression datasets(GSE3585 and GSE5406) were downloaded from the Gene Expression Omnibus database. The differentially expressed genes(DEGs) between the DCM patients and healthy individuals were identified using the R package “linear models for microarray data.” The pathways with common DEGs were analyzed via Gene Ontology(GO), Kyoto Encyclopedia of Genes and Genomes(KEGG), and gene set enrichment analyses. Moreover, a protein-protein interaction network(PPI) was constructed to identify the hub genes and modules. The MicroRNA Database was applied to predict the microRNAs(miRNAs) targeting the hub genes. Additionally, immune cell infiltration in DCM was analyzed using CIBERSORT.RESULTS In total, 97 DEGs(47 upregulated and 50 downregulated) were identified. GO analysis showed that the DEGs were mainly enriched in “response to growth factor,” “extracellular matrix,” and “extracellular matrix structural constituent.” KEGG pathway analysis indicated that the DEGs were mainly enriched in “protein digestion and absorption” and “interleukin 17(IL-17) signaling pathway.” The PPI network suggested that collagen type Ⅲ alpha 1 chain(COL3A1) and COL1A2 contribute to the pathogenesis of DCM. Additionally, visualization of the interactions between miRNAs and the hub genes revealed that hsa-miR-5682 and hsa-miR-4500 interacted with both COL3A1 and COL1A2, and thus these miRNAs might play roles in DCM. Immune cell infiltration analysis revealed that DCM patients had more infiltrated plasma cells and fewer infiltrated B memory cells, T follicular helper cells, and resting dendritic cells.CONCLUSION COL1A2 and COL3A1 and their targeting miRNAs, hsa-miR-5682 and hsa-miR-4500, may play critical roles in the pathogenesis of DCM, which are closely related to the IL-17 signaling pathway and acute inflammatory response. These results may provide useful clues for the diagnosis and treatment of DCM.展开更多
We designed this study to identify potential key protein interaction networks,genes,and correlated pathways in dilated cardiomyopathy(DCM)via bioinformatics methods.We selected the GSE3586 microarray dataset,consistin...We designed this study to identify potential key protein interaction networks,genes,and correlated pathways in dilated cardiomyopathy(DCM)via bioinformatics methods.We selected the GSE3586 microarray dataset,consisting of 15 dilated cardiomyopathic heart biopsy samples and 13 nonfailing heart biopsy samples.Initially,the GSE3586 dataset was downloaded and was analyzed with the limma package to identify differentially expressed genes(DEGs).A total of 172 DEGs consisting of 162 upregulated genes and ten downregulated genes in DCM were selected by the criterion of adjusted P values less than 0.01 and the log2-fold change of 0.6 or greater.Gene Ontology functional enrichment analysis and Kyoto Encyclopedia of Genes and Genomes(KEGG)pathway analysis were performed to view the biological processes,cellular components,molecular function,and KEGG pathways of the DEGs.Next,protein-protein interactions were constructed,and the hub protein modules were identifi ed.Then we selected the key genes DLD,UQCRC2,DLAT,SUCLA2,ATP5A1,PRDX3,FH,SDHD,and NDUFV1,which are involved in a wide range of biological activities,such as the citrate cycle,oxidation-reduction processes and cellular respiration,and energy derivation by oxidation of organic compounds in mitochondria.Finally,we found that currently there are no related gene-targeting drugs after exploring the predicted interactions between key genes and drugs,and transcription factors.In conclusion,our study provides greater understanding of the pathogenesis and underlying molecular mechanisms in DCM.This contributes to the exploration of potential gene therapy targets.展开更多
Dilated cardiomyopathy(DCM)is a type of composite cardiomyopathy characterized by left ventricular,right ventricular or double ventricular enlargement and diastolic dysfunction caused by genetic and non-genetic causes...Dilated cardiomyopathy(DCM)is a type of composite cardiomyopathy characterized by left ventricular,right ventricular or double ventricular enlargement and diastolic dysfunction caused by genetic and non-genetic causes,with a high incidence,High mortality,gradual rejuvenation,etc.,are common types of cardiomyopathy.With the progressive development of the disease,it can cause arrhythmia,severe heart failure,thromboembolism,and even sudden death.Currently,there is no effective treatment plan,and the high mortality rate is a major problem in the treatment of cardiovascular diseases.Long-chain non-coding RNAs(lncRNAs)are a class of RNAs with a molecular mass of more than 200 bt and which are not involved or rarely involved in protein coding.Studies have shown that lncRNAs play an important role in the occurrence and development of DCM.Finding relevant clinical diagnostic markers and therapeutic targets is the current research focus in the field of DCM.This article reviews the mechanism and research progress of lncRNAs in the occurrence and development of DCM,and aims to provide a new direction for the prevention and treatment of DCM.展开更多
Background:To evaluate the clinical efficacy of oral administration of Chinese medicines with warming Yang effects and Western medicine in the treatment of heart failure caused by dilated cardiomyopathy.Methods:Seven ...Background:To evaluate the clinical efficacy of oral administration of Chinese medicines with warming Yang effects and Western medicine in the treatment of heart failure caused by dilated cardiomyopathy.Methods:Seven databases including Chinese National Knowledge Infrastructure,Weipu database,Wanfang database,China Biomedical Literature Service System,Cochrane library,PubMed,and Embase were searched.The search date was from established to October 2019.Randomized clinical trials of dilated cardiomyopathy patients with heart failure under the combination treatment of Chinese medicines with warming Yang effects and Western medicine were included.We then assessed the methodological quality of included literatures,extracted valid data and conducted systematically evaluation according to the Cochrane systematic review method.Outcome index included clinical efficacy,left ventricular ejection fraction,and brain natriuretic peptide.Results:Meta-analysis results showed that the clinical effective rate was higher in the combination group than that in the control group(RR=1.24,95%CI(1.14,1.34),P<0.01).In addition,the combination group could improve left ventricular ejection fraction compared with the control group(Std.MD=0.85,95%CI(0.69,1.01),P<0.01).However,the meta-analysis of brain natriuretic peptide levels showed that the included literatures have higher heterogeneity and the meta-analysis results were unstable.So the results of brain natriuretic peptide were abandoned.Conclusion:Oral administration of Chinese medicines with warming Yang effects combined with Western medicine is better than Western medicine alone in improving clinical effect and left ventricular ejection fraction,but it is not clear whether brain natriuretic peptide can be improved.Because the quality of the included studies is not high and inconsistent outcome index,more standardized and rigorous randomized double-blind controlled trials are needed to validate and guide clinical use.展开更多
BACKGROUND Right ventricular(RV)function is frequently overlooked during dilated cardiomyopathy(DCM)evaluation.AIM To evaluate RV function in children with idiopathic DCM using relatively recent echocardiographic moda...BACKGROUND Right ventricular(RV)function is frequently overlooked during dilated cardiomyopathy(DCM)evaluation.AIM To evaluate RV function in children with idiopathic DCM using relatively recent echocardiographic modalities.METHODS We prospectively studied the cardiac function in 50 children with idiopathic DCM and 50 healthy children as a control group,using four-dimensional echocardiography(4-DE),Tissue Doppler Imaging(TDI),and two-dimensional-speckles tracking echocardiography(2-D-STE).RV EF was measured by 4-DE.RESULTS The auto left(LV)ejection fractions(EF)measured by 2-D-STE were significantly lower in the patients'group than in the control.The sphericity index was also significantly lower in children with DCM than in the control.RV EF measured by 4-DE was significantly lower in the patient's group than the control.RV S wave,e´/a'ratio,myocardial performance index(MPI),and tricuspid annular plane systolic excursion(TAPSE)were significantly impaired in children with DCM than in control.Both LV and RV global longitudinal strains(GLS)were significantly reduced in children with DCM than in control.RVGLS was significantly associated with the duration since diagnosis,tricuspid annulus S wave,RV MPI,and TAPSE,but not with the age of the patients,RV EF,or e´/a'ratio.CONCLUSION There was impairment of the RV LGS and other systolic and diastolic parameters in children with DCM.STE and TDI can help to detect the early decline of RV function.展开更多
Objective:To investigate the effect of metoprolol on cardiac function and prognosis in patients with dilated cardiomyopathy.Methods:100 patients with dilated cardiomyopathy treated in our hospital from January 2018 to...Objective:To investigate the effect of metoprolol on cardiac function and prognosis in patients with dilated cardiomyopathy.Methods:100 patients with dilated cardiomyopathy treated in our hospital from January 2018 to December 2019 were randomly divided into control group(n=50)and observation group(n=50).The control group was treated with conventional methods,and the observation group was treated with conventional methods and metoprolol for 6 months.The cardiac function[left ventricular ejection fraction(LVEF),stroke volume(SV),cardiac output(CO)]and prognosis[Glasgow Outcome Scale(GOS)score]of the two groups before and at the end of 6 months of intervention were compared,and the incidence of adverse reactions of the two groups were compared.Results:After 6 months of treatment,the levels of LVEF,SV and CO in the two groups were higher than before treatment,and the comparison level between the observation group and the control group was higher,the difference was statistically significant(P<0.05);After 6 months of treatment,the GOS score of the observation group was higher than that of the control group,and the difference was statistically significant(P<0.05);There was no significant difference in the total incidence of adverse reactions between the two groups(P>0.05).Conclusion:Metoprolol can improve the cardiac function and prognosis of patients with dilated cardiomyopathy,without increasing the incidence of adverse reactions.展开更多
Objective To evaluate the clinical significance of QT dispersion (QTd, QTcd) in dilated cardiomyopathy (DCM). Methods QTd and QTcd were measured on simultaneously recording 12 lead electrocardiograms (ECGs) in 60 DCM ...Objective To evaluate the clinical significance of QT dispersion (QTd, QTcd) in dilated cardiomyopathy (DCM). Methods QTd and QTcd were measured on simultaneously recording 12 lead electrocardiograms (ECGs) in 60 DCM patients and compared with 60 healthy subjects. Results The values of QTd and QTcd in DCM were significantly higher than those in control group (P<0 01). With subgroup analysis, QTd and QTcd in patients with cardiac sudden death (CSD) were longer than those in survivors and those died of progressive heart failure (P<0 05), patients with ventricular tachycardia (VT) or with severe heart failure than those without (compared with patients with ventricular premature beats [VPB], P<0 05, compared with patients without ventricular arrhythmia [VA], P<0 01) or with mild heart failure (P<0 01). The values of QTd and QTcd in patients with VPB were greater than those in patients without VA(P<0 05). There were significant differences in the rates of VT, CSD and heart failure between the groups of QTd>110 ms and QTd≤110 ms(P<0 01 or P<0 05), in contrast to ejection fraction(EF) and fractional shortening (FS)(P>0 05). Conclusion The values of QTd and QTcd increased in DCM patients were susceptive index for monitoring maligant VA in DCM, also important prognostic markers of CSD. QTd was correlated with NYHA functional class but not with EF and FS.展开更多
文摘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.
文摘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.
文摘BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic heart failure.However,acute heart failure(AHF)without obvious associated triggers is rare.Prior to the report of endomyocardial biopsy,the diagnosis and treatment of EFE are highly susceptible to being confounded with other primary cardiomyopathies.Here,we report a case of pediatric AHF caused by EFE mimicking dilated cardiomyopathy(DCM),with the aim of providing a valuable reference for clinicians to early identify and diagnose EFE-induced AHF.CASE SUMMARY A 13-mo-old female child was admitted to hospital with retching.Chest X-ray demonstrated enhanced texture in both lungs and an enlarged heart shadow.Color doppler echocardiography showed an enlarged left heart with ventricular wall hypokinesis and decreased left heart function.Abdominal color ultrasonography revealed a markedly enlarged liver.Pending the result of the endomyocardial biopsy report,the child was treated with a variety of resuscitative measures including nasal cannula for oxygen,intramuscular sedation with chlorpromazine and promethazine,cedilanid for cardiac contractility enhancement,and diuretic treatment with furosemide.Subsequently,the child’s endomyocardial biopsy report result was confirmed as EFE.After the above early interventions,the child’s condition gradually stabilized and improved.One week later,the child was discharged.During a 9-mo follow-up period,the child took intermittent low-dose oral digoxin with no signs of recurrence or exacerbation of the heart failure.CONCLUSION Our report suggests that EFE-induced pediatric AHF may present in children over 1 year of age without any apparent precipitants,and that the associated clinical presentations are grossly similar to that of pediatric DCM.Nonetheless,it is still possible to be diagnosed effectively on the basis of the comprehensive analysis of auxiliary inspection findings before the result of the endomyocardial biopsy is reported.
基金Supported by the Jilin Provincial Healthcare Talent Special Program,No.2019SCZT08.
文摘BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients with DCM contributes to variable disease severity and complicates overall prognosis,which can be very poor.AIM To identify pathogenic genes in DCM through pedigree analysis.METHODS Our research team identified a patient with DCM in the clinic.Through invest-igation,we found that the family of this patient has a typical DCM pedigree.High-throughput sequencing technology,next-generation sequencing,was used to sequence the whole exomes of seven samples in the pedigree.RESULTS A novel and potentially pathogenic gene mutation-ANK2p.F3067L-was discovered.The mutation was completely consistent with the clinical information for this DCM pedigree.Sanger sequencing was used to further verify the locus of the mutation in pedigree samples.These results were consistent with those of high-throughput sequencing.CONCLUSIONS ANK2p.F3067L is considered a novel and potentially pathogenic gene mutation in DCM.
基金funded by the Chinese National Natural Science Foundation(No.12072215)Science and Technology Department of Sichuan Province(2021YFS0120 and 2023NSFSC1640)+1 种基金Chinese Postdoctoral Science Foundation(2022M722278)Chunhui Program of Ministry of Education of China(No.HZKY20220573).
文摘Background:Various physiological mechanisms are linked to dilated cardiomyopathy(DCM)development,including oxidative stress,immune irregularities,inflammation,fibrosis,and genetic changes.However,precise molecular drivers of DCM,especially regarding abnormal immune responses,remain unclear.This study investigates immune-related long non-coding RNAs(lncRNAs)in DCM’s diagnostic and therapeutic potential.Methods:GSE141910,GSE135055,and GSE165303 datasets were acquired from the GEO database.LASSO,SVM-RFE,and random forest algorithms identified DCM-associated immune-related lncRNAs.Diagnostic capabilities were assessed by Nomogram and receiver operating characteristic(ROC)curves.Multivariate linear regression explored lncRNA correlations with ejection fraction.Single-sample gene set enrichment analysis(ssGSEA)gauged immune cell infiltration/functions.Functional enrichment analyses were performed using Gene set variation analysis(GSVA),gene ontology(GO),and the Kyoto Encyclopedia of Genes and Genomes(KEGG).Consensus clustering categorized DCM cases.Results:Ten immune-related lncRNAs emerged:C10orf71-AS1,FHAD1-AS1,SCIRT,FNDC1-AS1,MELTFAS1,LOC101928834,GDNF-AS1,DCXR-DT,C3orf36,and LOC107985323.These lncRNAs,tied to immunomodulation,showed promising DCM diagnostic accuracy.Adjusted for confounders,they independently correlated with ejection fraction.Using lncRNA expression,DCM patients were grouped into subtypes.Subtype C1 displayed a higher level of immune cell infiltration and immune checkpoint expression compared to subtype C2,emphasizing the variations in the immune microenvironment.Conclusion:This study identifies ten immune-related lncRNAs for further exploration in DCM diagnosis and subtyping.Based on expression patterns,we propose two potential DCM subtypes.Notably,findings are preliminary and hypothesis-generating,demanding validation and further investigation.This research provides insights into DCM diagnosis and classification.
基金the Research Grant of the National Natural Science Foundation of China,No.81801674Sichuan Province Science and Technology Support Program,No.2021YJ0242.
文摘BACKGROUND The prognostic value of late gadolinium enhancement(LGE)derived from cardiovascular magnetic resonance(CMR)is well studied,and several new metrics of LGE have emerged.However,some controversies remain;therefore,further discussion is needed,and more precise risk stratification should be explored.AIM To investigate the associations between the positivity,extent,location,and pattern of LGE and multiple outcomes in dilated cardiomyopathy(DCM).METHODS PubMed,Ovid MEDLINE,and Cochrane Library were searched for studies that investigated the prognostic value of LGE in patients with DCM.Pooled hazard ratios(HRs)and 95%confidence intervals were calculated to assess the role of LGE in the risk stratification of DCM.RESULTS Nineteen studies involving 7330 patients with DCM were included in this metaanalysis and covered a wide spectrum of DCM,with a mean left ventricular ejection fraction between 21%and 50%.The meta-analysis revealed that the presence of LGE was associated with an increased risk of multiple adverse outcomes(all-cause mortality,HR:2.14;arrhythmic events,HR:5.12;and composite endpoints,HR:2.38;all P<0.001).Furthermore,every 1%increment in the extent of LGE was associated with an increased risk of all-cause mortality.Analysis of a subgroup revealed that the prognostic value varied based on different location and pattern of LGE.Additionally,we found that LGE was a stronger predictor of arrhythmic events in patients with greater left ventricular ejection fraction.CONCLUSION LGE by CMR in patients with DCM exhibited a substantial value in predicting adverse outcomes,and the extent,location,and pattern of LGE could provide additional information for risk stratification.
基金the National Natural Science Foundation of China(Nos.81701653 and 81570348).
文摘Myocardial fiber deformation measurements have been reported to be associated with adverse outcomes in patients with acute heart failure and those with myocardial infarction.However,few studies have addressed the prognostic value of global circumferential strain(GCS)in dilated cardiomyopathy(DCM)patients with severely impaired systolic function.This study aimed to evaluate the prognostic value of cardiac magnetic resonance(CMR)-derived GCS in DCM patients with severely reduced ejection.Consecutive DCM patients with severely reduced ejection fraction(EF<35%)who underwent CMR were included.GCS was calculated from CMR cine images.The clinical endpoint was a composite of all-cause mortality,heart transplantation,implantable cardioverter defibrillator(ICD)implantation and aborted sudden cardiac death(SCD).A total of 129 patients with a mean EF of 15.33%(11.36%–22.27%)were included.During a median follow-up of 518 days,endpoint events occurred in 50 patients.Patients with GCS≥the median(−5.17%)had significantly reduced event-free survival as compared with those with GCS<the median(P<0.01).GCS was independently associated with adverse events after adjusting for clinical and imaging risk factors including extent of late gadolinium enhancement(LGE)(P<0.05).Adding GCS into the model including the extent of LGE resulted in significant improvements in the C-statistic(from 0.706 to 0.742;P<0.05)with a continuous net reclassification improvement(NRI)of 29.71%.It was concluded that GCS derived from CMR could be useful for risk stratification in DCM patients with severely reduced EF,which may increase common imaging risk factors including LGE.
基金the Sichuan Province Science and Technology Support Program of China。
文摘BACKGROUND Nemaline myopathy(NM)is a rare type of congenital myopathy,with an incidence of 1:50000.Patients with NM often exhibit hypomyotonia and varying degrees of muscle weakness.Skeletal muscles are always affected by this disease,while myocardial involvement is uncommon.However,with improvements in genetic testing technology,it has been found that NM with a mutation in the myopalladin(MYPN)gene not only causes slow,progressive muscle weakness but also results in dilated or hypertrophic cardiomyopathy.CASE SUMMARY A 3-year-old pre-school boy was admitted to our hospital with cough,edema,tachypnea,and an increased heart rate.The patient was clinically diagnosed with severe dilated cardiomyopathy and heart failure,and subsequent gene examination confirmed the diagnosis of NM with a mutation in MYPN.Captopril,diuretics,low-dose digoxin,and dobutamine were administered.After 22 d of hospitalization,the patient was discharged due to the improvement of clinical symptoms.During the follow-up period,the patient died of refractory heart failure.CONCLUSION Decreased muscular tone and dilated cardiomyopathy are common features of MYPN-mutated NM.Heart transplantation may be a solution to this type of cardiomyopathy.
文摘Background: The aim of this study was to determine the relation of left atrial (LA) volume and LA volume index with left ventricular function and to determine the association of duration of symptoms and left atrial volume index in patients with dilated cardiomyopathy. Materials and Methods: This was an observational, single centre study conducted in India. A total of 50 patients who were admitted to department of cardiology from July, 2008 to February, 2009 with diagnosis of dilated cardiomyopathy and an ejection fraction of <40% were included. Results: Of the 50 patients, 34 (68%) were males. 27 (54%) patients were in NYHA class II and 23 (46%) patients were in NYHA class III. LA volume was found to be ≥40 ml in all patients. LV function and LA volume were found to be correlated (r = -0.789, p < 0.01). Similarly, there was a correlation between LV function and LA volume index (r = -0.826, p < 0.01). There was no correlation between LA volume index and duration of symptoms (r = 0.04). Conclusion: It can be concluded that there is a strong inverse correlation between LA volume and left ventricular function and also between LA volume index and left ventricular function. The patients with NYHA class III were having larger left atrial volume than those with NYHA class II. Moreover, the duration of symptoms has no correlation with left atrial volume index.
基金This work was supported by the National Natural Science Foundation of China(61602066)the Project of Sichuan Outstanding Young Scientific and Technological Talents(19JCQN0003)+2 种基金the major Project of Education Department in Sichuan(17ZA0063 and 2017JQ0030)in part by the Natural Science Foundation for Young Scientists of CUIT(J201704)the Sichuan Science and Technology Program(2019JDRC0077).
文摘Myocardial segmentation and classification play a major role in the diagnosis of cardiovascular disease.Dilated Cardiomyopathy(DCM)is a kind of common chronic and life-threatening cardiopathy.Early diagnostics significantly increases the chances of correct treatment and survival.However,accurate and rapid diagnosis of DCM is still challenge due to high variability of cardiac structure,low contrast cardiac magnetic resonance(CMR)images,and intrinsic noise in synthetic CMR images caused by motion artifact and cardiac dynamics.Moreover,visual assessment and empirical evaluation are widely used in routine clinical diagnosis,but they are subject to high inter-observer variability and are both subjective and non-reproducible.To solve this problem,we proposed an effective unified multi-task framework for dilated cardiomyopathy CMR segmentation and classification simultaneously,and we firstly update one independent encoder from both recovery decoder and parallel attention path sharing some partial weights.This can encode both task choices into good embedding,but each one can achieve significant improvements respectively from the given embedding.It consists of three branches:extraction path,attention path,and recovery path,which allows the model to learn more higher-level intermediate representations and makes a more accurate prediction.We validated our approach on a DCM dataset,which contains 1155 CMR LGE images.Experimental results show that our multi-task network has achieved accuracy of 97.63%,AUC of 98.32%,demonstrating effectively segmenting the myocardium,quickly and accurately diagnosing the presence or absence of dilation.
文摘BACKGROUND The clinical course of acute myocarditis ranges from the occurrence of a few symptoms to the development of fatal fulminant myocarditis.Specifically,fulminant myocarditis causes clinical deterioration very rapidly and aggressively.The long-term prognosis of myocarditis is varied,and it fully recovers without leaving any special complications.However,even after recovery,heart failure may occur and eventually progress to dilated cardiomyopathy(DCM),which causes serious left ventricular dysfunction.In the case of follow-up observation,no clear guidelines have been established.CASE SUMMARY We report the case of a 21-year-old woman who presented with dyspnea.She became hemodynamically unstable and showed sustained fatal arrhythmias with decreased heart function.She was clinically diagnosed with fulminant myocarditis based on her echocardiogram and cardiac magnetic resonance results.After 2 d,she was readmitted to the emergency department under cardiopulmonary resuscitation and received mechanical ventilation and extracorporeal membrane oxygenation.An implantable cardioverter defibrillator was inserted for secondary prevention.She recovered and was discharged.Prior to being hospitalized for sudden cardiac function decline and arrhythmia,she had been well for 7 years without any complications.She was finally diagnosed with dilated cardiomyopathy.CONCLUSION DCM may develop unexpectedly in patients who have been cured of acute fulminant myocarditis and have been stable with a long period of remission.Therefore,they should be carefully and regularly observed clinically throughout long-term follow-up.
文摘Background:Signifi cant left ventricular dysfunction may arise in right-sided accessory pathways with ventricular preexcitation in the absence of recurrent or incessant tachycardia.This has just been realized and not enough attention has been paid to it.Methods:In the last 7 years,we identifi ed 12 consecutive children with a diagnosis of ventricular preexcitation–induced dilated cardiomyopathy.This report describes the clinical and echocardiographic characteristics of the patients before and after ablation.Results:Dyssynchronous ventricular contraction was observed by M-mode echocardiography and two-dimensional strain analysis in all patients.The basal and middle segments of the interventricular septum became thin and moved similarly to an aneurysm,with typical bulging during the end of systole.The locations of the accessory pathways were the right-sided septum(n=5)and the free wall(n=7).Left ventricular synchrony was obtained shortly after ablation.The left ventricular function recovered to normal and the left ventricular end-diastolic diameter decreased gradually during follow-up.Conclusions:A causal relationship between ventricular preexcitation and the development of dilated cardiomyopathy is supported by the complete recovery of left ventricular function and reversed left ventricular remodeling after the loss of ventricular preexcitation.Preexcitation-related dyssynchrony was thought to be the crucial mechanism.Ventricular preexcitation–induced dilated cardiomyopathy is an indication for ablation with a good prognosis.
基金Supported by Natural Science Foundation of Hunan Province,No.2019JJ60087
文摘BACKGROUND Alstr?m syndrome(AS)is a rare autosomal recessive disease that is generally induced by mutations of the Alstr?m syndrome 1(ALMS1)gene.We report a case of AS,extend the spectrum of ALMS1 mutations and highlight the biological role of ALMS1 to explore the relationship between dilated cardiomyopathy(DCM)and mutations in ALMS1.CASE SUMMARY We present the case of an infant with AS mainly manifesting with DCM that was caused by a novel mutation of the ALMS1 gene.Whole-exome sequencing revealed a simultaneous large deletion and point mutation in ALMS1,leading to frameshift and missense mutations,respectively,rather than nonsense or frameshift mutations,which have been reported previously.Upon optimized anti-remodeling therapy,biohumoral exams and arrhythmic burden of the infant were alleviated at follow-up after 6 mo.CONCLUSION We identified novel mutations of ALMS1 and extended the spectrum of ALMS1 mutations in an infant with AS.
基金Supported by National Nature Science Foundation of China,No.81960051,No.8217021743,and No.82160060Project of High–Level Innovative Talents of Guizhou Province,No.[2016]4034Construction Funding from Characteristic Key Laboratory of Guizhou Province,No.[2021]313.
文摘BACKGROUND Heart failure is a health burden responsible for high morbidity and mortality worldwide, and dilated cardiomyopathy(DCM) is one of the most common causes of heart failure. DCM is a disease of the heart muscle and is characterized by enlargement and dilation of at least one ventricle alongside impaired contractility with left ventricular ejection fraction < 40%. It is also associated with abnormalities in cytoskeletal proteins, mitochondrial ATP transporter, microvasculature, and fibrosis. However, the pathogenesis and potential biomarkers of DCM remain to be investigated.AIM To investigate the candidate genes and pathways involved in DCM patients.METHODS Two expression datasets(GSE3585 and GSE5406) were downloaded from the Gene Expression Omnibus database. The differentially expressed genes(DEGs) between the DCM patients and healthy individuals were identified using the R package “linear models for microarray data.” The pathways with common DEGs were analyzed via Gene Ontology(GO), Kyoto Encyclopedia of Genes and Genomes(KEGG), and gene set enrichment analyses. Moreover, a protein-protein interaction network(PPI) was constructed to identify the hub genes and modules. The MicroRNA Database was applied to predict the microRNAs(miRNAs) targeting the hub genes. Additionally, immune cell infiltration in DCM was analyzed using CIBERSORT.RESULTS In total, 97 DEGs(47 upregulated and 50 downregulated) were identified. GO analysis showed that the DEGs were mainly enriched in “response to growth factor,” “extracellular matrix,” and “extracellular matrix structural constituent.” KEGG pathway analysis indicated that the DEGs were mainly enriched in “protein digestion and absorption” and “interleukin 17(IL-17) signaling pathway.” The PPI network suggested that collagen type Ⅲ alpha 1 chain(COL3A1) and COL1A2 contribute to the pathogenesis of DCM. Additionally, visualization of the interactions between miRNAs and the hub genes revealed that hsa-miR-5682 and hsa-miR-4500 interacted with both COL3A1 and COL1A2, and thus these miRNAs might play roles in DCM. Immune cell infiltration analysis revealed that DCM patients had more infiltrated plasma cells and fewer infiltrated B memory cells, T follicular helper cells, and resting dendritic cells.CONCLUSION COL1A2 and COL3A1 and their targeting miRNAs, hsa-miR-5682 and hsa-miR-4500, may play critical roles in the pathogenesis of DCM, which are closely related to the IL-17 signaling pathway and acute inflammatory response. These results may provide useful clues for the diagnosis and treatment of DCM.
文摘We designed this study to identify potential key protein interaction networks,genes,and correlated pathways in dilated cardiomyopathy(DCM)via bioinformatics methods.We selected the GSE3586 microarray dataset,consisting of 15 dilated cardiomyopathic heart biopsy samples and 13 nonfailing heart biopsy samples.Initially,the GSE3586 dataset was downloaded and was analyzed with the limma package to identify differentially expressed genes(DEGs).A total of 172 DEGs consisting of 162 upregulated genes and ten downregulated genes in DCM were selected by the criterion of adjusted P values less than 0.01 and the log2-fold change of 0.6 or greater.Gene Ontology functional enrichment analysis and Kyoto Encyclopedia of Genes and Genomes(KEGG)pathway analysis were performed to view the biological processes,cellular components,molecular function,and KEGG pathways of the DEGs.Next,protein-protein interactions were constructed,and the hub protein modules were identifi ed.Then we selected the key genes DLD,UQCRC2,DLAT,SUCLA2,ATP5A1,PRDX3,FH,SDHD,and NDUFV1,which are involved in a wide range of biological activities,such as the citrate cycle,oxidation-reduction processes and cellular respiration,and energy derivation by oxidation of organic compounds in mitochondria.Finally,we found that currently there are no related gene-targeting drugs after exploring the predicted interactions between key genes and drugs,and transcription factors.In conclusion,our study provides greater understanding of the pathogenesis and underlying molecular mechanisms in DCM.This contributes to the exploration of potential gene therapy targets.
基金National Natural Science Foundation of China(No.81960861,81460712)Guangxi Science key Research and Development Program(No.AB19110006)Guangxi Postgraduate Education Innovation Program(No.YCSY2020099)。
文摘Dilated cardiomyopathy(DCM)is a type of composite cardiomyopathy characterized by left ventricular,right ventricular or double ventricular enlargement and diastolic dysfunction caused by genetic and non-genetic causes,with a high incidence,High mortality,gradual rejuvenation,etc.,are common types of cardiomyopathy.With the progressive development of the disease,it can cause arrhythmia,severe heart failure,thromboembolism,and even sudden death.Currently,there is no effective treatment plan,and the high mortality rate is a major problem in the treatment of cardiovascular diseases.Long-chain non-coding RNAs(lncRNAs)are a class of RNAs with a molecular mass of more than 200 bt and which are not involved or rarely involved in protein coding.Studies have shown that lncRNAs play an important role in the occurrence and development of DCM.Finding relevant clinical diagnostic markers and therapeutic targets is the current research focus in the field of DCM.This article reviews the mechanism and research progress of lncRNAs in the occurrence and development of DCM,and aims to provide a new direction for the prevention and treatment of DCM.
文摘Background:To evaluate the clinical efficacy of oral administration of Chinese medicines with warming Yang effects and Western medicine in the treatment of heart failure caused by dilated cardiomyopathy.Methods:Seven databases including Chinese National Knowledge Infrastructure,Weipu database,Wanfang database,China Biomedical Literature Service System,Cochrane library,PubMed,and Embase were searched.The search date was from established to October 2019.Randomized clinical trials of dilated cardiomyopathy patients with heart failure under the combination treatment of Chinese medicines with warming Yang effects and Western medicine were included.We then assessed the methodological quality of included literatures,extracted valid data and conducted systematically evaluation according to the Cochrane systematic review method.Outcome index included clinical efficacy,left ventricular ejection fraction,and brain natriuretic peptide.Results:Meta-analysis results showed that the clinical effective rate was higher in the combination group than that in the control group(RR=1.24,95%CI(1.14,1.34),P<0.01).In addition,the combination group could improve left ventricular ejection fraction compared with the control group(Std.MD=0.85,95%CI(0.69,1.01),P<0.01).However,the meta-analysis of brain natriuretic peptide levels showed that the included literatures have higher heterogeneity and the meta-analysis results were unstable.So the results of brain natriuretic peptide were abandoned.Conclusion:Oral administration of Chinese medicines with warming Yang effects combined with Western medicine is better than Western medicine alone in improving clinical effect and left ventricular ejection fraction,but it is not clear whether brain natriuretic peptide can be improved.Because the quality of the included studies is not high and inconsistent outcome index,more standardized and rigorous randomized double-blind controlled trials are needed to validate and guide clinical use.
文摘BACKGROUND Right ventricular(RV)function is frequently overlooked during dilated cardiomyopathy(DCM)evaluation.AIM To evaluate RV function in children with idiopathic DCM using relatively recent echocardiographic modalities.METHODS We prospectively studied the cardiac function in 50 children with idiopathic DCM and 50 healthy children as a control group,using four-dimensional echocardiography(4-DE),Tissue Doppler Imaging(TDI),and two-dimensional-speckles tracking echocardiography(2-D-STE).RV EF was measured by 4-DE.RESULTS The auto left(LV)ejection fractions(EF)measured by 2-D-STE were significantly lower in the patients'group than in the control.The sphericity index was also significantly lower in children with DCM than in the control.RV EF measured by 4-DE was significantly lower in the patient's group than the control.RV S wave,e´/a'ratio,myocardial performance index(MPI),and tricuspid annular plane systolic excursion(TAPSE)were significantly impaired in children with DCM than in control.Both LV and RV global longitudinal strains(GLS)were significantly reduced in children with DCM than in control.RVGLS was significantly associated with the duration since diagnosis,tricuspid annulus S wave,RV MPI,and TAPSE,but not with the age of the patients,RV EF,or e´/a'ratio.CONCLUSION There was impairment of the RV LGS and other systolic and diastolic parameters in children with DCM.STE and TDI can help to detect the early decline of RV function.
文摘Objective:To investigate the effect of metoprolol on cardiac function and prognosis in patients with dilated cardiomyopathy.Methods:100 patients with dilated cardiomyopathy treated in our hospital from January 2018 to December 2019 were randomly divided into control group(n=50)and observation group(n=50).The control group was treated with conventional methods,and the observation group was treated with conventional methods and metoprolol for 6 months.The cardiac function[left ventricular ejection fraction(LVEF),stroke volume(SV),cardiac output(CO)]and prognosis[Glasgow Outcome Scale(GOS)score]of the two groups before and at the end of 6 months of intervention were compared,and the incidence of adverse reactions of the two groups were compared.Results:After 6 months of treatment,the levels of LVEF,SV and CO in the two groups were higher than before treatment,and the comparison level between the observation group and the control group was higher,the difference was statistically significant(P<0.05);After 6 months of treatment,the GOS score of the observation group was higher than that of the control group,and the difference was statistically significant(P<0.05);There was no significant difference in the total incidence of adverse reactions between the two groups(P>0.05).Conclusion:Metoprolol can improve the cardiac function and prognosis of patients with dilated cardiomyopathy,without increasing the incidence of adverse reactions.
文摘Objective To evaluate the clinical significance of QT dispersion (QTd, QTcd) in dilated cardiomyopathy (DCM). Methods QTd and QTcd were measured on simultaneously recording 12 lead electrocardiograms (ECGs) in 60 DCM patients and compared with 60 healthy subjects. Results The values of QTd and QTcd in DCM were significantly higher than those in control group (P<0 01). With subgroup analysis, QTd and QTcd in patients with cardiac sudden death (CSD) were longer than those in survivors and those died of progressive heart failure (P<0 05), patients with ventricular tachycardia (VT) or with severe heart failure than those without (compared with patients with ventricular premature beats [VPB], P<0 05, compared with patients without ventricular arrhythmia [VA], P<0 01) or with mild heart failure (P<0 01). The values of QTd and QTcd in patients with VPB were greater than those in patients without VA(P<0 05). There were significant differences in the rates of VT, CSD and heart failure between the groups of QTd>110 ms and QTd≤110 ms(P<0 01 or P<0 05), in contrast to ejection fraction(EF) and fractional shortening (FS)(P>0 05). Conclusion The values of QTd and QTcd increased in DCM patients were susceptive index for monitoring maligant VA in DCM, also important prognostic markers of CSD. QTd was correlated with NYHA functional class but not with EF and FS.
