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arrhythmogenic right ventricular cardiomyopathy characterized by recurrent syncope during exercise:A case report

World Journal of Clinical Cases 2021年第16期9卷 4095-4103页

作者： Hao-Yu Wu Yi-Wei Cao Tian-Jiao Gao Jian-Li Fu Lei Liang Department of Cardiology Shaanxi Provincial People’s HospitalXi'an 710068Shaanxi ProvinceChina Department of Electrocardiology Shaanxi Provincial People’s HospitalXi'an 710068Shaanxi ProvinceChina Department of Gastroenterology Xi’an Children’s HospitalXi’an 710003Shaanxi ProvinceChina Department of Ultrasonic Diagnosis Center Shaanxi Provincial People’s HospitalXi’an 710068Shaanxi ProvinceChina

BACKGROUND arrhythmogenic right ventricular(RV)cardiomyopathy is a rare and currently underrecognized cardiomyopathy characterized by the replacement of RV myocardium by fibrofatty *** may be asymptomatic or symptomatic(palpitations or syncope)and may induce sudden cardiac death,especially during *** prevent adverse events such as sudden cardiac death and heart failure,early diagnosis and treatment of arrhythmogenic RV cardiomyopathy(ARVC)are *** report a patient with ARVC characterized by recurrent syncope during exercise who was successfully treated with combined endocardial and epicardial catheter *** SUMMARY A 43-year-old man was referred for an episode of syncope during ***,the patient experienced two episodes of syncope without a firm etiological *** electrocardiogram obtained at admission indicated ventricular tachycardia originating from the inferior wall of the right *** ventricular tachycardia was terminated with intravenous propafenone.A repeat electrocardiogram showed a regular sinus rhythm with negative T waves and a delayed S-wave upstroke from leads V1 to *** magnetic resonance imaging showed RV free wall thinning,regional RV akinesia,RV dilatation and fibrofatty infiltration(RV ejection fraction of 38%).An electrophysiological study showed multiple inducible ventricular tachycardia as of a focal mechanism from the right *** and epicardial voltage mapping demonstrated scar tissue in the anterior wall,free wall and posterior wall of the right *** potentials were also *** patient was diagnosed with ARVC and treated with combined endocardial and epicardial catheter ablation with a very satisfactory follow-up *** Clinicians should be aware of ARVC,and further workup,including imaging with multiple modalities,should be *** combination of epicardial and endocardial catheter ablation can lead to a good outcome.

关键词： arrhythmogenic right ventricular cardiomyopathy Endocardial catheter ablation Epicardial catheter ablation Syncope Exercise Case report

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arrhythmogenic right ventricular cardiomyopathy: From genetics to diagnostic and therapeutic challenges

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World Journal of Cardiology 2014年第12期6卷 1234-1244页

作者： Bruno Pinamonti Francesca Brun Luisa Mestroni Gianfranco Sinagra Cardiovascular Department Ospedali Riuniti of Trieste34100Trieste Italy Cardiovascular Institute and Adult Medical Genetics Program University of Colorado Denver AMC Aurora CO 80045-2507 United States

arrhythmogenic right ventricular cardiomyopathy(ARVC) is a genetic disease characterized by myocyte loss and fibro-fatty tissue replacement. Diagnosis of ARVC remains a clinical challenge mainly at its early stages and in patients with minimal echocardiographic right ventricular(RV) abnormalities. ARVC shares some common features with other cardiac diseases, such as RV outflow ventricular tachycardia, Brugada syndrome, and myocarditis, due to arrhythmic expressivity and biventricular involvement. The identification of ARVC can be often challenging, because of the heterogeneous clinical presentation, highly variable intra- and inter-family expressivity and incomplete penetrance. This genotypephenotype "plasticity" is largely unexplained. A familial history of ARVC is present in 30% to 50% of cases, and the disease is considered a genetic cardiomyopathy, usually inherited in an autosomal dominant pattern with variable penetrance and expressivity; in addition, autosomal recessive forms have been reported(Naxos disease and Carvajal syndrome). Diagnosis of ARVC relays on a scoring system, with major or minorcriteria on the Revised Task Force Criteria. Implantable cardioverter defibrillators(ICDs) are increasingly utilized in patients with ARVC who have survived sudden death(SD)(secondary prevention). However, there are few data available to help identifying ARVC patients in whom the prophylactic implantation of an ICD is truly warranted. Prevention of SD is the primary goal of management. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and ICD are the mainstay of treatment of ARVC.

关键词： arrhythmogenic right ventricular cardiomyopathy Sudden cardiac death Risk stratification Genetic Implantable cardioverter-defibrillator

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Ablation strategies for arrhythmogenic right ventricular cardiomyopathy: a systematic review and meta-analysis

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Journal of Geriatric Cardiology 2020年第11期17卷 694-703页

作者： Li-Shui SHEN Li-Min LIU Li-Hui ZHENG Feng HU Zhi-Cheng HU Shang-Yu LIU Jin-Rui GUO Kush Kumar Bhagat Yan YAO Arrhythmia Center Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Arrhythmia Center Yunnan Fuwai Cardiovascular HospitalKunmingChina Eternal hospital JaipurRajasthanIndia

Background Catheter ablation for ventricular tachycardia(VT) in patients with arrhythmogenic right ventricular cardiomyopathy(ARVC) has significantly evolved over the past decade. However, different ablation strategies showed inconsistency in acute and long-term outcomes. Methods We searched the databases of Medline, Embase and Cochrane Library through October 17, 2019 for studies describing the clinical outcomes of VT ablation in ARVC. Data including VT recurrence, all-cause mortality, acute procedural efficacy and major procedural complications were extracted. A meta-analysis with trial sequential analysis was further performed in comparative studies of endo-epicardial versus endocardial-only ablation. Results A total of 24 studies with 717 participants were enrolled. The literatures of epicardial ablation were mainly published after 2010 with total ICD implantation of 73.7%, acute efficacy of 89.8%, major complication of 5.2%, follow-up of 28.9 months, VT freedom of 75.3%, all-cause mortality of 1.1% and heart transplantation of 0.6%. Meta-analysis of 10 comparative studies revealed that compared with endocardial-only approach, epicardial ablation significantly decreased VT recurrence(OR: 0.50;95% CI: 0.30–0.85;P = 0.010), but somehow increased major procedural complications(OR: 4.64;95% CI: 1.28–16.92;P = 0.02), with not evident improvement of acute efficacy(OR: 2.74;95% CI: 0.98–7.65;P = 0.051) or all-cause mortality(OR: 0.87;95% CI: 0.09–8.31;P = 0.90). Conclusion Catheter ablation for VT in ARVC is feasible and effective. Epicardial ablation is associated with better long-term VT freedom, but with more major complications and unremarkable survival or acute efficacy benefit.

关键词： arrhythmogenic right ventricular cardiomyopathy Catheter ablation ventricular tachycardia

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A Heterozygous Phospholamban Variant(p.R14del)Leads to Left ventricular Involvement and Heart Failure Phenotypes in arrhythmogenic right ventricular cardiomyopathy

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Phenomics 2024年第1期4卷 13-23页

作者： Han Mo Xiumeng Hua Mengni Bao Zhe Sun Xiao Chen Mengda Xu Jiangping Song Shenzhen Key Laboratory of Cardiovascular Disease Fuwai Hospital Chinese Academy of Medical SciencesShenzhen 518057China State Key Laboratory of Cardiovascular Disease Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical College167A Beilishi RoadXi Cheng DistrictBeijing 100037China Beijing Key Laboratory of Preclinical Research and Evaluation for Cardiovascular Implant Materials Animal Experimental CentreFuwai HospitalNational Center for Cardiovascular DiseaseChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina

This study aimed to determine the prevalence and clinical features of arrhythmogenic right ventricular cardiomyopathy(ARVC)caused by pathogenic mutations in the Phospholamban(PLN)*** study included 170 patients who had a confrmed diagnosis of ARVC and underwent PLN genetic screening using next-generation *** fndings of this study provide valuable insights into the association between PLN mutations and ARVC,which can aid in the development of more efective diagnostic and treatment strategies for ARVC *** of the patients evaluated,six had a rare pathogenic mutation in PLN with the same p.R14del *** screening revealed that heterozygous carriers of p.R14del exhibited a defnite ARVC *** clinical studies,individuals with the p.R14del mutation experienced a similar rate of malignant arrhythmia events as those with classic desmosome *** adjusting for covariates,individuals with PLN mutations had a two point one seven times greater likelihood of experiencing transplant-related risks compared to those who did not possess PLN mutations(95%CI 1.08–6.82,p=0.035).The accumulation of left ventricular fat and fbers is a pathological marker for ARVC patients with p.R14del *** a cohort of 170 Chinese ARVC patients,three point fve percent of probands had the PLN pathogenic variant(p.R14del)and all were *** data shows that PLN-related ARVC patients are at high risk for ventricular arrhythmias and heart failure,which requires clinical diferentiation from classic ***,carrying the p.R14del mutation can be an independent prognostic risk factor in ARVC patients.

关键词： arrhythmogenic right ventricular cardiomyopathy Phospholamban Left ventricular involvement Heart failure Risk stratifcation

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Screening of pathogenic genes in Chinese patients with arrhythmogenic right ventricular cardiomyopathy

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Chinese Medical Journal 2013年第22期126卷 4238-4241页

作者： BAO Jing-ru WANG Ji-zheng YAO Yan WANG Yi-lu FAN Xiao-han SUN Kai ZHANG Shu HUI Ru-tai SONG Lei Chinese Acad Med Sci Peking Union Med Coll State Key Lab Cardiovasc Dis Natl Ctr Cardiovasc DisFuwai HospArrhythmia Ctr Beijing 100037 Peoples R China Chinese Acad Med Sci Peking Union Med Coll State Key Lab Cardiovasc Dis Natl Ctr Cardiovasc DisFuwai HospClin Ep Lab Beijing 100037 Peoples R China Chinese Acad Med Sci Peking Union Med Coll State Key Lab Cardiovasc DisFuwai Hosp Sino German Lab Mol MedNatl Ctr Cardiovasc Dis Beijing 100037 Peoples R China Chinese Acad Med Sci Peking Union Med Coll State Key Lab Cardiovasc Dis Natl Ctr Cardiovasc DisHypertens CtrFuwai Hosp Beijing 100037 Peoples R China

Background arrhythmogenic right ventricular cardiomyopathy （ARVC） is a heritable cardiac disease predominantly caused by mutations in desmosomal protein genes. Previous genetic analyses of the Chinese ARVC population are limited to small size and restriction to a single gene. This study was aimed to investigate the genotype in a large series of Chinese patients with ARVC through comprehensively screening nine ARVC-causing genes. Methods A total of 100 unrelated ARVC patients and 300 age, gender and ethnicity matched healthy controls were genetically tested with multiplexing targeted resequencing for nine previously reported ARVC-causing genes, including plakophilin-2, desmoplakin, desmoglein-2, desmocollin-2, plakoglobin, transforming growth factor beta-3, transmembrane protein 43, desmin and Lamin A/C. Results Fifty-nine mutations were identified in 64% of the patients, among which, 93% were located in desmosomal protein genes. Plakophilin-2 mutations accounted for 54% of the total and 58% of the desmosomal mutations, with a truncating mutation type making up about 2/3 of the plakophilin-2 mutations. Only four mutations were found in nondesmosomal genes; two in transmembrane protein 43 and two in transforming growth factor beta-3. Two of them （one of each gene） appeared as single missense mutations. No mutation was identified in desmin or Lamin A/C. Multiple mutations were found in 23% of the patients, with plakophilin-2 being found in 57% of the multi-mutation carriers. Conclusions Plakophilin-2 was the most common gene mutation that was identified in Chinese ARVC patients. Nondesmosomal genes should be added to desmosomal protein genes when performing molecular genetic screening in patients with suspected ARVC.

关键词： arrhythmogenic right ventricular cardiomyopathy genetic testing, plakophilin-2

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Mutation of plakophUin-2 gene in arrhythmogenic right ventricular cardiomyopathy

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Chinese Medical Journal 2009年第4期122卷 403-407页

作者： WU Shu-lin WANG Pei-ning HOU Yue-shuang ZHANG Xu-chao SHAN Zhi-xin YU Xi-yong DENG Mei Department of Cardiology Guangdong General Hospital Guangdong Provincial Cardiovascular Institute GuangzhouGuangdong 510080 China Research Center of Guangdong Academy of Medical Science Guangdong General HospitalGuangdong Provincial Cardiovascular Institute GuangzhouGuangdong 510080 China

Background arrhythmogenic right ventricular cardiomyopathy （ARVC） is one of the leading causes of sudden cardiac death. Recent studies have shown that ARVC, which is an inheritable genetic change, results from mutations in genes encoding desmosomal proteins. Plakophilin-2 is an important component of the desmosome. Because the full range of genetic variations related to ARVC is unknown and no related studies of the Chinese population have been reported, we aimed to investigate the genetic variation of plakophilin-2 in ARVC patients from the Southern Region of China. Methods Genomic DNA was isolated from peripheral blood samples of all 34 ARVC patients, who were screened through a clinical evaluation. They were used to detect variations in the sequences of the plakophilin-2 genes by polymerase chain reaction amplification in combination with direct sequencing. Results In exon-1 of the plakophilin-2 gene, a deletion mutation （c.145_148 del GACA） was found in one family pedigree. The mutation was also found in exon-2, 4, and 11 of the plakophilin-2 gene. The QT interval dispersion of the ECG was considerably longer in the mutation group than in the non-mutation group of ARVC patients, and this result was statistically significant （P 〈0.05）. Conclusion We discovered a plakophilin-2 mutation that prolongs the QT interval dispersion in the southern Chinese ARVC population.

关键词： arrhythmogenic right ventricular cardiomyopathy plakophilin-2 gene mutation

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Evaluation of cardiac function in patients with arrhythmogenic right ventricular cardiomyopathy by echocardiography

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South China Journal of Cardiology 2013年第3期14卷 164-170页

作者：陈春晖吴书林张黔桓费洪文黄俊薛玉梅廖洪涛方咸宏詹贤章 Department of Cardiology Guangdong Provincial Cardiovascular InstituteGuangdong General HospitalGuangdong Academy of Medical Sciences Department of Echocardiology Guangdong Provincial Cardiovascular InstituteGuangdong General HospitalGuangdong Academy of Medical Sciences

Background arrhythmogenic right ventricular cardiomyopathy （ARVC） mainly performs local myocardial abnormal movements and tissue Doppler and spot tracking technique can accurately reflect myocardial movement. However, the technique is still rarely used in research of ARVC. Methods The study enrolled 28 ARVC patients and 28 normal controls. right ventricular parameters were measured by two-dimensional echocardiography, tissue Doppler imaging, speckle tracking imaging in order to compare the difference between two groups. Results Morphological indices （right ventricular inflow tract inner diameter and right ventricular outflow tract inner diameter） and functional indices （right ventricular peak S＇, right ventricular E＇/ A＇ ratio, tricuspid annular plane systolic excursion, right ventricular fractional area change and right ventricular inferior and lateral wall longitudinal strain） showed significant difference between the ARVC group and control group. All the above-mentioned indices were analyzed by receiver operating characteristic curve （ROC curves）. Area under the curve （AUC） of right ventricular inferior wall longitudinal strain was the largest one （AUC = 0.94） with an optimal cutoff value of -19.5%. Conclusion Compared with two- dimensional echocardiography and tissue Doppler imaging, right ventricular inferior wall longitudinal strain is a more sensitive predictor for changes of ARVC.

关键词： arrhythmogenic right ventricular cardiomyopathy echocardiography doppler tissue imaging speckle tracking imaging

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Long-term outcomes in patients with arrhythmogenic right ventricular cardiomyopathy

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South China Journal of Cardiology 2012年第4期13卷 211-215,242页

作者：张黔桓黄俊严红邓春玉饶芳朱杰宁吴书林 Department of Cardiology Guangdong Cardiovascular Institute Guangdong Academy of Medical Sciences Research Center of Medical Sciences Guangdong General Hospital

Background arrhythmogenic right ventricular cardiomyopathy （ARVC） is a major cause for sudden cardiac death due to ventricular tachycardia. Litter is known about its long-term outcomes in Chinese ARVC patients. The purpose of this study was to evaluate the long-term clinical outcomes in patients with ARVC and to clarify the risk factors of cardiac events. Methods Forty subjects fulfilling modified Task Force criteria were included in this study. Information on clinical presentation, electrocardiographic and cardiac imaging findings, and long-term outcome of cases were investigated. Results Average follow-up period from onset was 57.5 ± 42.6 months. The mean age at onset of symptoms （32.2 ± 12.7 years） and male predominance （85.0%） were similar to that report- ed in other studies. Palpitations were the most frequent symptom （82.5%）. T-wave inversion was the most com- mon presenting abnormality on resting 12-lead ECG （75%）. ventricular tachycardia with left bundle branch block morphology was subsequently documented in a total of 28（70%） subjects during a study period. The cu- mulative mortality rate was 7.5%. Conclusion Clinical presentation in Chinese ARVC patients was similar to that reported in other studies. ARVC is associated with early mortality that is different to other country popula- tion.

关键词： arrhythmogenic right ventricular cardiomyopathy long-term outcomes clinical

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A validation study of southern China for European prediction model about ventricular arrhythmias for arrhythmogenic right ventricular cardiomyopathy

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South China Journal of Cardiology 2023年第1期24卷 1-7页

作者：骆溢驹刘方舟陈欣刘洋张黔桓邓海张玉华姜风雨陈子恩薛玉梅吴书林廖洪涛 Department of Cardiac Surgery Guangdong Cardiovascular InstituteGuangdong General HospitalGuangdong Academy of Medical SciencesGuangzhou 510080China

Background arrhythmogenic right ventricular dysplasia/cardiomyopathy is an inherited *** Society of Cardiology was devised a new prediction model to estimate ventricular arrhythmias and guide decisions regarding prima... 详细信息

关键词： European prediction model arrhythmogenic right ventricular cardiomyopathy external validation

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A case of sudden death due to arrhythmogenic right ventricular cardiomyopathy

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Chinese Medical Journal 2012年第23期125卷 4318-4318页

作者： Yan Feng-ping Chen Yuan-yuan Gannan Med Coll Dept Forens Sci Ganzhou 341000 Jiangxi Peoples R China

To the editor： arrhythmogenic right ventricular cardiomyopathy （ARVC） is characterized by structural and fianctional abnormalities of the right ventricle due to replacement of the myocardium by fatty and fibrous tissue, often with involvement of the left ventricle （LV）. ARVC has been recognized as a cause of sudden death, especially in the young. We present a case of sudden death due to ARVC based on autopsy findings.

关键词： sudden death arrhythmogenic right ventricular cardiomyopathy

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