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Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report
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作者 Jia-Rong Li Lei-Yu Feng +2 位作者 Jian-Wei Li Yu Liao Fei-Qi Liu 《World Journal of Clinical Cases》 SCIE 2024年第3期601-606,共6页
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
关键词 POLYNEUROPATHY organomegaly ENDOCRINOPATHY M-PROTEIN skin changes syndrome dilated cardiomyopathy LENALIDOMIDE Dexamethasone Case report
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Construction of A Prediction Model for Atrial Fibrillation in Patients with Dilated Cardiomyopathy and Heart Failure
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作者 Kaizheng Liu Chengjie Liu 《Journal of Clinical and Nursing Research》 2024年第1期228-232,共5页
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
关键词 dilated cardiomyopathy Heart failure Atrial fibrillation Prediction model
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Diagnostic and classification value of immune-related lncRNAs in dilated cardiomyopathy
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作者 CONGCHEN BAI QIHANG KONG +3 位作者 HAO TANG SHUWEN ZHANG JUNTENG ZHOU XIAOJING LIU 《BIOCELL》 SCIE 2023年第11期2517-2533,共17页
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
关键词 dilated cardiomyopathy Immune-associated long noncoding RNA Immune infiltration BIOMARKER
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Pediatric acute heart failure caused by endocardial fibroelastosis mimicking dilated cardiomyopathy:A case report
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作者 Yao-Ying Xie Qiu-Li Li +1 位作者 Xin-Le Li Fan Yang 《World Journal of Clinical Cases》 SCIE 2023年第8期1771-1781,共11页
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
关键词 Endocardial fibroelastosis dilated cardiomyopathy PEDIATRIC Acute heart failure Early identification and diagnosis
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Study of pathogenic genes in a pedigree with familial dilated cardiomyopathy
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作者 Xin-Ru Zhang Hang Ren +2 位作者 Fang Yao Yang Liu Chun-Li Song 《World Journal of Clinical Cases》 SCIE 2023年第11期2412-2422,共11页
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
关键词 dilated cardiomyopathy Gene mutation Whole exomes sequencing Sanger sequencing ANK2p.F3067L Potentially pathogenic gene
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Association between late gadolinium enhancement and outcome in dilated cardiomyopathy:A meta-analysis
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作者 Xin-Yi Feng Wen-Feng He +5 位作者 Tian-Yue Zhang Ling-Li Wang Fan Yang Yu-Ling Feng Chun-Ping Li Rui Li 《World Journal of Radiology》 2023年第11期324-337,共14页
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
关键词 Cardiac magnetic resonance dilated cardiomyopathy Late gadolinium enhancement META-ANALYSIS Myocardial fibrosis PROGNOSIS
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A novel mitochondrial tRNA gene mutation in a chinese family with dilated cardiomyopathy and sensorineural deafness 被引量:3
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作者 Xianghong Wu Xiumei Xie Guotian Ma Guoju Sun Xiaobin Chen 《Journal of Nanjing Medical University》 2006年第5期279-282,共4页
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. Method... 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. 展开更多
关键词 cardiomyopathy dilated mitochondrial DNA MUTATION GENETICS
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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 被引量:2
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作者 Liang Chen Zhongyin Zhou +4 位作者 Huihe Lu Ye Xie Gang Li Jianfei Huang Dongsheng Zhao 《The Journal of Biomedical Research》 CAS CSCD 2018年第4期314-316,共3页
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... 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 展开更多
关键词 related gene filter in a family with familiar DCM AVB Identification of a LMNA c.646C T variant by whole-exome sequencing in combination with a dilated cardiomyopathy
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Mechanism of Anti-β-adrenoceptor Antibody Mediated Myocardial Damage in Dilated Cardiomyopathy 被引量:21
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作者 廖玉华 程龙献 +5 位作者 涂源淑 张金枝 董继华 李淑莉 田元 彭又红 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 SCIE CAS 1997年第1期5-8,共4页
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 pe... 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. 展开更多
关键词 ANTIBODIES Β-ADRENOCEPTOR cytoplasmic free calcium FURA-2/AM dilated cardiomyopathy
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D-dimer level and long-term outcome in patients with end-stage heart failure secondary to idiopathic dilated cardiomyopathy 被引量:10
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作者 Bi HUANG Yuan-Jing LI +3 位作者 Jian SHEN Yuan YANG Gang LIU Su-Xin LUO 《Journal of Geriatric Cardiology》 SCIE CAS CSCD 2019年第8期621-629,共9页
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 prognost... 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. 展开更多
关键词 D-DIMER END-STAGE heart failure IDIOPATHIC dilated cardiomyopathy Long-term OUTCOME
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Clinical characteristics, treatment and prognosis of patients with idiopathic dilated cardiomyopathy: a tertiary center experience 被引量:4
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作者 Abdullah Hagar Xiao-Bo PU +2 位作者 Shi-Jian CHEN Jageshwar-Prasad Shah Mao CHEN 《Journal of Geriatric Cardiology》 SCIE CAS CSCD 2019年第4期320-328,共9页
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... 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. 展开更多
关键词 dilated cardiomyopathy HEART failure MEDICATIONS PROGNOSIS
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Non-invasive evaluation of arrhythmic risk in dilated cardiomyopathy:From imaging to electrocardiographic measures 被引量:5
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作者 Massimo Iacoviello Francesco Monitillo 《World Journal of Cardiology》 CAS 2014年第7期562-576,共15页
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 ri... 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. 展开更多
关键词 dilated cardiomyopathy Major VENTRICULAR ARRHYTHMIAS Prognosis VENTRICULAR REPOLARIZATION Left VENTRICULAR SYSTOLIC function
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Prognostic Value of Feature-Tracking Circumferential Strain in Dilated Cardiomyopathy Patients with Severely Reduced Ejection Fraction Incremental to Late Gadolinium Enhancement 被引量:7
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作者 Shcng-lei SHU Jing WANG +6 位作者 Cheng WANG Feng ZHU Yu-xi JIA Lan ZHANG Xiao-yue ZHOU Tian-jing ZHANG Chuan-sheng ZHENG 《Current Medical Science》 SCIE CAS 2021年第1期158-166,共9页
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
关键词 cardiac magnetic resonance imaging circumferential strain PROGNOSIS late gadolinium enhancement dilated cardiomyopathy
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Effect of arotinolol on right ventricular function in patients with dilated cardiomyopathy 被引量:1
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作者 Hong Yang Li Xu Yongkang Tao Zhimin Xu Xiuqing Du Naqiang Lu Kefei Dou Jinglin Zhao Xianqi Yuan Yanfen Zhao Rongfang Shi Chaomei Fan 《Journal of Geriatric Cardiology》 SCIE CAS CSCD 2007年第3期170-173,共4页
Objective Dilated cardiomyopathy(DCM) is generally considered to be accompanied by both left and right ventricular dysfunction,but most studies only analyze the left ventricular function. In this study,we evaluated th... Objective Dilated cardiomyopathy(DCM) is generally considered to be accompanied by both left and right ventricular dysfunction,but most studies only analyze the left ventricular function. In this study,we evaluated the effect of arotinolol on right ventricular function in patients with DCM. Methods Right ventricular ejection fraction(RVEF) and right ventricular diameter(RVD) were measured by two-dimensional echocardiography(2-DE) in 33 DCM patients;RVEF measured by first-pass radionuclide angiography(FPRA) was compared with that by 2-DE. Results The treatment with arotinolol for one year resulted in a reduction in the right ventricular diameter(baseline,23.0 ± 8.3 mm vs after one-year treatment,20.7 ± 5.4 mm;P=0.004 ) and an associated increase in ejection fraction(baseline,36.9 ± 10.3% vs after one-year treatment,45.8 ± 9.6%;P < 0.001 ) ;there is a high correlation between the 2-DE method and radionuclide ventriculographic method. The correlation coefficient is 0.933(P<0.001) . Conclusion Arotinolol therapy could not only improve left ventricular function,but also improve right ventricular function in DCM patients. 展开更多
关键词 dilated cardiomyopathy arotinolol RIGHT VENTRICULAR FUNCTION
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HLA-DRB1 Gene Polymorphism in Patients with Dilated Cardiomyopathy 被引量:2
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作者 王秋芬 廖玉华 +2 位作者 龚非力 毛焕元 张金枝 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 SCIE CAS 2000年第2期141-142,共2页
To probe into the genetic background and immunopathogenesis of dilated cardiomyopathy (DCM), HLA-DRB1 gene polymorphism in 68 patients with DCM and 175 normal control subjects were analyzed by using the polymerase cha... To probe into the genetic background and immunopathogenesis of dilated cardiomyopathy (DCM), HLA-DRB1 gene polymorphism in 68 patients with DCM and 175 normal control subjects were analyzed by using the polymerase chain reaction/sequence specific primer (PCR/SSP) techniques. It was found that the frequencies of HLA-DRB1 15 and HLA-DRB1 03 alleles were significantly lower in DCM patients than those in normal controls (14. 71 % vs 29. 71 % and 4. 41 % vs 15. 43 %, respectively), the relative risks (RR) in the DCM patients being 0. 41 and 0. 25, respectively, all P<0. 05. However, the frequencied of HLA-DRB1 11 and HLA-DRB1. 12 alleles were significantly higher in the DCM patients than in controls (29. 4 % vs 12. 00 % and 36. 76 % vs 12. 57 %, respectively) with the RR in the DCM patients being 3. 06 and 4. 04, respectively, all P<0. 01. These findings further demonstrated that-immunogenetics might play a predominant pathogenetic role in partial DCM patients. 展开更多
关键词 dilated cardiomyopathy HLA antibody
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Contemporary characteristics and outcomes of adults with familial dilated cardiomyopathy listed for heart transplantation 被引量:1
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作者 Mohamed Khayata Sadeer G Al-Kindi Guilherme H Oliveira 《World Journal of Cardiology》 CAS 2019年第1期38-46,共9页
BACKGROUND Familial dilated cardiomyopathy(FDCM) account for 20%-30% of non-ischemic cardiomyopathies(NICM). Previous published data showed that some patients with FDCM tend to have rapidly progressive disease; howeve... BACKGROUND Familial dilated cardiomyopathy(FDCM) account for 20%-30% of non-ischemic cardiomyopathies(NICM). Previous published data showed that some patients with FDCM tend to have rapidly progressive disease; however, five-year mortality was not significantly different in the familial and non-familial forms of NICM with optimal medical therapy.AIM To better define the characteristics and clinical outcomes of FDCM patients listed for heart transplantation(HT).METHODS We queried the United Network for Organ Sharing Registry to identify FDCM patients listed for HT between January 2008 and September 2015 and compared them to NICM and ischemic cardiomyopathy(ICM) patients. We included all patients ≥ 18 years old and we separated patients to three groups: FDCM, NICM and ICM. Chi-square test was used to compare between categorical variables, the t-test was used to compare between continues variables, and Cox-proportional hazards model was used to perform time-dependent survival analyses.RESULTS Of the 24809 adults listed for HT, we identified 677 patients(2.7%) with the diagnosis of FDCM. Compared to patients with NICM and ICM, FDCM patients were younger(FDCM 43.9 ± 13.5 vs NICM 50.9 ± 12.3, P < 0.001, vs ICM 58.5 ±8.1, P < 0.001), more frequently listed as status 2(FDCM 35.2% vs NICM 26.5%, P< 0.001), with significantly lower left ventricular assist device(LVAD) utilization(FDCM 18.4% vs NICM 25.1%, P < 0.001; vs ICM 25.6%, P < 0.001), but higher use of total artificial heart(FDCM 1.3% vs NICM 0.6%, P = 0.039; vs ICM 0.4%, P =0.002). Additionally, patients with FDCM were less frequently delisted for clinical deterioration or death and more likely to be transplanted compared to those with NICM [hazard ratio(HR): 0.617, 95% confidence interval(CI): 0.47-0.81; HR: 1.25,95%CI: 1.14-1.37, respectively], and ICM(HR: 0.5, 95%CI: 0.38-0.66; HR: 1.18,95%CI: 1.08-1.3, respectively). There was more frequent rejection among patients with FDCM(FDCM 11.4% vs NICM 9.8%, P = 0.28; vs ICM 8.4%, P = 0.034). One,three, and five post-transplant survival of patients with FDCM(91%, 88% and80%) was similar to those with NICM(91%, 84%, 79%, P = 0.225), but superior to those with ICM(89%, 82%, 75%, P = 0.008), respectively.CONCLUSION End-stage FDCM patients are more likely to be transplanted, more likely to have early rejection, and have similar or higher survival than patients with other cardiomyopathies. 展开更多
关键词 FAMILIAL dilated cardiomyopathy END-STAGE heart failure Wait list TRANSPLANT OUTCOMES
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Dhcr24 activates the PI3K/Akt/HKII pathway and protects against dilated cardiomyopathy in mice 被引量:3
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作者 Wei Dong Fei-fei Guan +5 位作者 Xu Zhang Shan Gao Ning Liu Wei Chen Lian-feng Zhang Dan Lu 《Animal Models and Experimental Medicine》 2018年第1期40-52,共13页
Background: 24-dehydrocholesterol reductase(Dhcr24) catalyzes the last step of cholesterol biosynthesis, which is required for normal development and antiapoptotic activities of tissues. We found that Dhcr24 expressio... Background: 24-dehydrocholesterol reductase(Dhcr24) catalyzes the last step of cholesterol biosynthesis, which is required for normal development and antiapoptotic activities of tissues. We found that Dhcr24 expression decreased in the cTnTR141 Wdilated cardiomyopathy(DCM) transgenic mice. Therefore, we tested whether rescued expression of Dhcr24 could prevent the development of DCM and its possible mechanism.Methods: Heart tissue specific transgenic overexpression mice of Dhcr24 was generated, then was crossed to c TnTR141 Wmouse to obtain the double transgenic mouse(DTG). The phenotypes were demonstrated by the survival, cardiac geometry and function analysis, as well as microstructural and ultrastructural observations based on echocardiography and histology examination. The pathway and apoptosis were analysed by western blotting and TUNEL assay in vivo and in vitro.Results: We find that Dhcr24 decreased in hearts tissues of cTnTR141 Wand LMNAE82 KDCM mice. The transgenic overexpression of Dhcr24 significantly improves DCM phenotypes in cTnTR141 Wmice, and activates PI3K/Akt/HKII pathway, followed by a reduction of the translocation of Bax and release of cytochrome c, caspase-9 and caspase-3 activation and myocyte apoptosis. Knockdown the expression of Dhcr24 reduces the activation of PI3K/Akt/HKII pathway and inhibition of the mitochondrial-dependent apoptosis. The anti-apoptotic effect of Dhcr24 could be completely removed by the inhibition of PI3K pathway and partly removed by the HKII inhibitor in H9c2 cell line.Conclusion: Compensatory expression of Dhcr24 protect against DCM through activated PI3K/Akt/HKII pathway and reduce Bax translocation. This is the first investigation for the molecular mechanism of Dhcr24 participate in development of DCM. 展开更多
关键词 apoptosis Dhcr24 dilated cardiomyopathy gene expression and REGULATION
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Classification of New Biomarkers of Dilated Cardiomyopathy Based on Pathogenesis—An Update 被引量:1
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作者 Muhammad Nabeel Dookhun Yali Sun +2 位作者 Huayiyang Zou Xiaoqian Cao Xinzheng Lu 《Health》 2018年第3期300-312,共13页
Dilated Cardiomyopathy (DCM) is a complex heart disease affecting the heart musculature and vasculature, involving one or several underlying pathophysiological mechanisms. Identifying potential biomarkers for dilated ... Dilated Cardiomyopathy (DCM) is a complex heart disease affecting the heart musculature and vasculature, involving one or several underlying pathophysiological mechanisms. Identifying potential biomarkers for dilated cardiomyopathy is a challenge owing to various aetiologies involved. Studying the biomarkers involved in DCM will ultimately give a better insight about which pathophysiological pathways are involved in the onset of the disease. Owing to its multifactorial aetiologies, response to treatment is usually poor. If we can find the exact underlying causes, a better treatment approach could be implemented. One way to obtain better insight of DCM is to study the biomarkers released. Through biomarkers, we can know which underlying mechanisms are involved. Biomarkers can provide us with clinical information such as diagnostic, prognostic, risk stratification as well as response to treatment. Underlying mechanisms such as inflammation, stress/strain, myocyte injury, matrix remodelling, oxidative stress, neurohormones involvement, among others, can contribute to the onset of DCM. Different mechanisms will yield different biomarkers. So it would be wise to classify those biomarkers involving in DCM based on their respective pathogenesis. Moreover, most importantly is to be able to make use of the information that biomarker pertains. However, specificity of those biomarkers poses a problem. One way of making these biomarkers clinically useful is to make use of a biomarker modelling score system. 展开更多
关键词 dilated cardiomyopathy Biomarkers Biomarkers Model Biomarker SCORE DCM Biomarkers NEW Biomarkers
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Experiences of a Dilated Cardiomyopathy Patient Suffering to Maintain Life Partnership Based on Margaret Newman’s Theory 被引量:1
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作者 Ryoko Igarashi 《Open Journal of Nursing》 2019年第1期31-40,共10页
Background: Dilated cardiomyopathy is a refractory myocardial disease with a poor prognosis. Mrs. G experienced cardiopulmonary arrest during hospitalization. She constantly struggles with uncertainty and experiences ... Background: Dilated cardiomyopathy is a refractory myocardial disease with a poor prognosis. Mrs. G experienced cardiopulmonary arrest during hospitalization. She constantly struggles with uncertainty and experiences immense mental suffering from her symptoms, treatments, anxiety, and prognosis. This condition requires appropriate nursing care. Purpose: This study, which is based on Margaret Newman’s theory, aims to clarify the experience of illness of Mrs. G who has dilated cardiomyopathy. Methods: This involved interpretive and dialectical praxis research. The researcher and Mrs. G formed a partnership through discussion. Hermeneutical analysis was performed on the discussion content. Results: The experience of illness of Mrs. G consisted of 6 phases: “Looking back on the shock of having an illness and receiving medical treatment”, “Looking back on one’s life until present”, “Confusion resulting from uncertainty”, “Self-insight”, “Recognizing one’s true feelings of wanting to live”, and “Commitment and decision to live”. Mrs. G could find a new self and expand her consciousness. Conclusion: Mrs. G found meaning in coping with her illness and discovered the value of living while accepting her own destiny. She connected to her own life and became more prepared to live with hope. Thus, partnership in nursing care according to Newman’s theory can benefit patients with distress from dilated cardiomyopathy. This nursing support can improve patient outlook. 展开更多
关键词 dilated cardiomyopathy Patients Experience of Illness Margaret NEWMAN THEORY Interpretive Phenomenon
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Impact of Left Bundle Branch Block on Left Ventricular Mechanics in Patients with Idiopathic Dilated Cardiomyopathy 被引量:1
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作者 Ahmed Emara Hala Mahfouz Badran +3 位作者 Waleed Abdou Naglaa Fahim Mohamed Fathi Magdi H. Yacoub 《World Journal of Cardiovascular Diseases》 2019年第2期132-148,共17页
Objectives: Left bundle branch block (LBBB) is commonly associated with heart failure. We evaluated the prevalence and impact of LBBB on left ventricular mechanics using 2D strain imaging in patients with idiopathic d... Objectives: Left bundle branch block (LBBB) is commonly associated with heart failure. We evaluated the prevalence and impact of LBBB on left ventricular mechanics using 2D strain imaging in patients with idiopathic dilated cardiomyopathy (IDCM). Methods: We included 101 patients with IDCM with mean age 38 ± 18 years: 74% were males and 13.9% of them were in NYHA Class III-IV. LBBB was present in 26 (37%) of included patients. Myocardial mechanics including longitudinal, circumferential strain and rotation and LV synchronization were assessed using two-dimensional strain imaging. Results: LBBB group had higher LV volumes, and PAP compared with non LBBB. Peak LV longitudinal systolic strain (εsys) of the septum and global LV SRsys were significantly lower in LBBB compared to non LBBB group (P 0.01, 0.03). TTP-d was greater in LBBB in comparison to non LBBB group (274.5 ± 116 versus 209.4 ± 139, P 0.02). The electromechanical delay between septal segments was 35 ± 18 ms and between lateral wall segments: 48 ± 24 ms, between anterior wall segments: 21 ± 11 and between inferior wall segments: 41 ± 12. Consequently, LV mechanical dyssynchrony was more evident in IDCM patients with LBBB. QRS width was correlated inversely with LV longitudinal strain and strain rate and electromechanical delay (P 0.0001) in non LBBB group. In LBBB QRS width was not related to cardiac mechanics. Using univariate analysis and after a multiple covariate adjustment, the baseline LBBB was associated with a significantly increased LV dysfunction. Conclusion:After correcting for potential confounders, LBBB was found to be associated with more deterioration of LV mechanics and exaggerated LV dyssynchrony in patients with IDCM. 展开更多
关键词 IDIOPATHIC dilated cardiomyopathy LBBB 2D Strain Imaging
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