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Clinical characteristics and management of coexistent cardiomyopathy in patients with bicuspid aortic valve

Journal of Geriatric Cardiology 2023年第3期20卷 205-213页

作者： Di ZHANG Yi-Ting LU Ze-Min ZHOU Yu-Xiao HU Xin-Chang LIU Yi QU Ya-Xin LIU Xian-Liang ZHOU Department of Cardiology Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Emergency and Critical Care Center Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina

BACKGROUND Bicuspid aortic valve(BAV)is the most common congenital heart disease.However,the prevalence,clinical characteristics,and current management of BAV associated with inherited cardiomyopathy,including hypertrophic cardiomy-opathy(HCM),dilated cardiomyopathy(DCM),and left ventricular noncompaction(LVNC)have not been well described.METHODS Consecutive patients diagnosed with BAV at a large tertiary cardiovascular referral center between 2009 and 2018 were retrospectively assessed for HCM,DCM,and LVNC based on clinical and echocardiographic criteria.Patients with coexist-ent conditions were investigated further.RESULTS Of 3533 patients with BAV screened,57(1.6%)had concomitant cardiomyopathy.BAV was combined with HCM in 30 of these patients,with DCM in 19,and with LVNC in eight.Forty-six patients(80.7%)were male,and the mean age at first dia-gnosis was 47 years for BAV with HCM,49 years for BAV with DCM,and 35 years for BAV with LVNC.Heart failure and aortic valve dysfunction were common in these patients,and the prevalence of coexisting aortopathy was 43.3%,26.3%and 25.0%,re-spectively,for BAV with HCM,DCM and LVNC.During the index hospitalization,24 of the 57 patients(42.1%)underwent sur-gery,16(28%)underwent aortic valve and/or aortic surgery,and 16 of the 30 patients with HCM had a Morrow procedure.There were no deaths or other major adverse cardiovascular events.CONCLUSIONS The prevalence of inherited cardiomyopathy was higher in our patients with BAV than in the general popula-tion.Aortopathy and heart failure were common,with almost half of patients requiring surgery at diagnosis.

关键词： patients cardiomyopathy aortic

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Different clinical characteristics and outcomes of hypertrophic cardiomyopathy with and without hypertension:seeking the truth

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Journal of Geriatric Cardiology 2023年第2期20卷 109-120页

作者： Yu ZHANG Ming-Hao LIU Mo ZHANG Gui-Xin WU Jie LIU Ji-Zheng WANG Xiao-Lu SUN Wen JIANG Dong WANG Lian-Ming KANG Xue-Yi WU Yu-Bao ZOU Lei SONG State Key Laboratory of Cardiovascular Disease Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina cardiomyopathy Ward Fuwai HospitalNational Center for Cardiovascular DiseaseChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Department of Cardiology Fuwai HospitalNational Center for Cardiovascular DiseaseChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina National Clinical Research Center of Cardiovascular Diseases Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina

OBJECTIVE To determine the different clinical characteristics and outcomes of hypertrophic cardiomyopathy(HCM)patients with and without hypertension(HT).METHODS A total of 696 HCM patients were included in this study and all HCM diagnoses were confirmed by the genetic test.Patients were analyzed separately in the septal reduction therapy(SRT)cohort and the non-SRT cohort.The primary endpoint was cardiovascular death and the secondary endpoint was all-cause death.Outcome analyses were conducted to evaluate the associations between HT and outcomes in HCM.Medications before enrollment and at discharge were collected in the post-hoc analyses.RESULTS HCM patients without HT were younger,had a lower body mass index,were more likely to have a family history of HCM,and had a smaller left ventricular(LV)end-diastolic diameter than those with HT in both cohorts.A thicker LV wall,a higher level of N-terminal pro-B-type natriuretic peptide,and a higher extent of LV late gadolinium enhancement were additionally observed in patients without HT in the non-SRT cohort.The presence of HT did not alter the distribution pattern of late gadolinium enhancement,as well as the constituent ratio of eight disease-causing sarcomeric gene variants in both cohorts.Outcome analyses showed that in the non-SRT cohort,patients without HT had higher risks of cardiovascular death(HR=2.537,P=0.032)and all-cause death(HR=3.309,P=0.032).While such prognostic divergence was not observed in the SRT cohort.Further post-hoc analyses in the non-SRT cohort found that patients without HT received fewer non-dihydropyridine calcium channel blockers and angiotensinconverting enzyme inhibitors/angiotensin receptor blockers before enrollment and at discharge.CONCLUSIONS HCM patients without HT had worse clinical conditions and higher mortality than patients with HT overall,which may result from active medical therapy in HT patients.Active SRT may have a substantial de-risking effect on patients meeting the indications.

关键词： clinical hypertension cardiomyopathy

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Regulation of mammalian target of rapamycin on ferroptosis:from mechanism to therapeutics in septic cardiomyopathy

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Chinese Medical Journal 2022年第23期135卷 2815-2817页

作者： Guoyu Zhao Hao Wang Na Cui Department of Critical Care Medicine State Key Laboratory of Complex Severe and Rare DiseasesPeking Union Medical College HospitalChinese Academy of Medical Science and Peking Union Medical CollegeBeijing 100730China Department of Critical Care Medicine Beijing Jishuitan HospitalBeijing 100035China

Sepsis refers to multiple organ dysfunction caused by the host’s dysfunctional response to infection.Most importantly,myocardial injury in septic patients,that is,septic cardiomyopathy(SIC),accounts for 1/3 to 1/2 of the total number of hospital deaths.The pathogenesis of SIC remains largely unclear,for which in-depth exploration is of great significance to improve the clinical prognosis of this patient population.Ferroptosis was discovered as a form of regulated cell death in 2012,which is characterized by iron dependence and lipid peroxide accumulation.It has long been thought that ferroptosis plays a deleterious role in the pathophysiology of various diseases.Recently,the role of ferroptosis in the occurrence and development of SIC has attracted extensive attention,warranting further studies.

关键词： cardiomyopathy prognosis clinical

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Isogenic human pluripotent stem cell disease models reveal ABRA deficiency underlies cTnT mutation-induced familial dilated cardiomyopathy

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Protein & Cell 2022年第1期13卷 65-71页

作者： Bin Li Yongkun Zhan Qianqian Liang Chen Xu Xinyan Zhou Huanhuan Cai Yufan Zheng Yifan Guo Lei Wang Wenqing Qiu Baiping Cui Chao Lu Ruizhe Qian Ping Zhou Haiyan Chen Yun Liu Sifeng Chen Xiaobo Li Ning Sun Department of Physiology and Pathophysiology State Key Laboratory of Medical NeurobiologySchool of Basic Medical SciencesFudan UniversityShanghai 200032China Shanghai Institute of Precision Medicine Ninth People's HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Shanghai Key Lab of Birth Defect Children's Hospital of Fudan UniversityShanghai 201102China Shanghai Key Laboratory of Clinical Geriatric Medicine Research Center on Aging and MedicineFudan UniversityShanghai 200032China Department of Echocardiography Zhongshan HospitalFudan UniversityShanghai 200032China Department of Biochemistry School of Basic Medical SciencesFudan UniversityShanghai 200032China

Dear Editor,Dilated cardiomyopathy(DCM)is a common form of inherited cardiomyopathy.In the past decades,single mutations in various genes encoding sarcomeric,cytoskeletal,and channel proteins etc.have been found to be associated with DCM(Hershberger et al.,2013;McNally and Mestroni,2017).However,the mechanisms how single mutations in sarcomeric or structural genes lead to the disease remain elusive.An interesting phenomenon often seen in familial cardiomyopathy is that different single mutations on the same gene can cause either DCM or hypertrophic cardiomyopathy(HCM)(Kathiresan and Srivastava,2012),which exhibit almost opposite disease phenotypes.DCM is characterized by thinned myocardium and septum,ventricular chamber dilation,and systolic dysfunction(Jefferies and Towbin,2010;McNally and Mestroni,2017),while HCM exhibits thickened myocardium and septum,reduced ventricular chamber,and diastolic dysfunction(Richard et al.,2003).At the cellular level,HCM cardiomyocytes exhibit concentric hypertrophy characterized by assembly of myofilaments in parallel and widening of the myocytes.In contrast,DCM cardiomyocytes show eccentric hypertrophy,with assembly of the myofilaments in series and myocyte elongation(Kehat and Molkentin,2010).

关键词： cardiomyopathy familial

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Late onset of neutral lipid storage disease due to a rare PNPLA2 mutation in a patient with myopathy and cardiomyopathy

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Chinese Medical Journal 2022年第19期135卷 2389-2391页

作者： Ye Tian Shiyao Wang Fang Wang Li Yi Mingrui Dong Xu Huang Department of Pulmonary and Critical Care Medicine Center of Respiratory MedicineChina-Japan Friendship HospitalNational Center for Respiratory MedicineInstitute of Respiratory MedicineChinese Academy of Medical SciencesNational Clinical Research Center for Respiratory DiseasesBejing 100029China Department of Cardiology China-Japan Friendship HospitalBeijing 100029China Department of Neurology China-Japan Friendship HospitalBeijing 100029China.

To the Editor:Neutral lipidd storage disease with myopathy(NLSDM)is a rare autosomal recessive disorder caused by mutations in the PNPLA2 gene.The gene encodes adipose triglyceride lipase(ATGL),an enzyme that catalyzes hydrolysis of triglycerides in mammalian adipose tissue and plays key roles in the function of lipid droplets(LDs).The results of many biochemical studies have revealed intracellular localization of ATGLwithLDs,but catalyticactivity iscompletely lost in the context of PNPLA2mutation.

关键词： myopathy rare cardiomyopathy

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A nomogram predicting atrial fibrillation in patients with dilated cardiomyopathy

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Chinese Medical Journal 2022年第8期135卷 991-993页

作者： Yangming Mao Yanmin Liu Jian Zhang Yeqian Zhu Xinguang Chen Ling Sun Yao Lu Hongwu Chen Weizhu Ju Gang Yang Pengcheng Zhao Minglong Chen Fengxiang Zhang Section of Pacing and Electrophysiology Division of CardiologyThe First Affiliated Hospital of Nanjing Medical UniversityNanjingJiangsu 210029China Department of Cardiology Qinghai Province HospitalThe Affiliated Hospital of Qinghai UniversityXiningQinghai 810007China Department of Cardiology Xuzhou Central HospitalThe Affiliated Xuzhou Hospital of Nanjing Medical UniversityXuzhouJiangsu 221009China

To the Editor:Dilated cardiomyopathy(DCM)is a type of primary myocardial disease with unknown etiology.[1]It is a disorder of the heart muscle mainly characterized by left ventricular dilation and systolic dysfunction,resulting from the response of the myocardium to genetic and environmental insults.The clinical manifestations arc progressive heart failure,arrhythmia,thromboembolism,and even sudden death.

关键词： cardiomyopathy fibrillation clinical

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Sorting nexin 3 exacerbates doxorubicin-induced cardiomyopathy via regulation of TFRC-dependent ferroptosis

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Acta Pharmaceutica Sinica B 2023年第12期13卷 4875-4892页

作者： Wenjing Yu Yuehuai Hu Zhiping Liu Kaiteng Guo Dinghu Ma Mingxia Peng Yuemei Wang Jing Zhang Xiaolei Zhang Panxia Wang Jiguo Zhang Peiqing Liu Jing Lu National and Local United Engineering Lab of Druggability and New Drugs Evaluation School of Pharmaceutical SciencesSun Yat-sen UniversityGuangzhou 510006China Guangdong Provincial Key Laboratory of New Drug Design and Evaluation Guangdong Province Engineering Laboratory for Druggability and New Drug EvaluationSchool of Pharmaceutical SciencesSun Yat-sen UniversityGuangzhou 510006China School of Pharmacy Jinan UniversityGuangzhou 510632China School of Pharmaceutical Sciences Shandong First Medical University&Shangdong Academy of Medical SciencesTaian 271016China

The clinical utilization of doxorubicin(Dox)in various malignancies is restrained by its major adverse effect:irreversible cardiomyopathy.Extensive studies have been done to explore the prevention of Dox cardiomyopathy.Currently,ferroptosis has been shown to participate in the incidence and development of Dox cardiomyopathy.Sorting Nexin 3(SNX3),the retromer-associated cargo binding protein with important physiological functions,was identified as a potent therapeutic target for cardiac hypertrophy in our previous study.However,few study has shown whether SNX3 plays a critical role in Dox-induced cardiomyopathy.In this study,a decreased level of SNX3 in Dox-induced cardiomyopathy was observed.Cardiac-specific Snx3 knockout(Snx3-cKO)significantly alleviated cardiomyopathy by downregulating Dox-induced ferroptosis significantly.SNX3 was further demonstrated to exacerbate Dox-induced cardiomyopathy via induction of ferroptosis in vivo and in vitro,and cardiac-specific Snx3 transgenic(Snx3-cTg)mice were more susceptible to Dox-induced feroptosis and cardiomyopathy.Mechanistically,SNX3 facilitated the recycling of transferrin 1 receptor(TFRC)via direct interaction,disrupting iron homeostasis,increasing the accumulation of iron,triggering ferroptosis,and eventually exacerbating Dox-induced cardiomyopathy.Overall,these findings established a direct SNX3-TFRC-ferroptosis positive regulatory axis in Dox-induced cardiomyopathy and suggested that targeting SNX3 provided a new effective therapeutic strategy for Dox-induced cardiomyopathy through TFRCdependentferroptosis.

关键词： SNX3 Ferroptosis TFRC cardiomyopathy Doxorubicin Iron homeostasis Cell death Mitochondria

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Identification of CHMP4C as a new risk gene for inherited dilated cardiomyopathy

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Journal of Genetics and Genomics 2022年第2期49卷 169-172页

作者： Nianwei Zhou Lu Tang Yingying Jiang Xuejie Li Shifang Shan Bo Yuan Shengmei Qin Cuizhen Pan Xiaolin Wang Xianhong Shu Zilong Qiu Junbo Ge Department of Echocardiography Zhongshan HospitalFudan UniversityShanghai Institute of Cardiovascular DiseaseShanghai Institute of Medical ImagingShanghaiChina Institute of Neuroscience State Key Laboratory of NeuroscienceCAS Center for excellence in Brain Science and Intelligence TechnologyChinese Academy of SciencesShanghaiChina Department of Cardiology Zhongshan HospitalFudan UniversityShanghai Institute of Cardiovascular DiseaseShanghaiChina Department of Interventional Radiology Zhongshan HospitalFudan UniversityShanghai Institute of Medical ImagingShanghaiChina

Dilated cardiomyopathy(DCM) is a primary myocardial disease of unknown cause that is characterized by ventricular enlargement and ventricular systolic dysfunction(Reichart et al., 2019). DCM exhibits obvious heterogeneity, and its outcomes extend from arrhythmia to heart failure. Early arrhythmia is a common condition that may progressively become aggravated, and death can occur at any stage of the disease. The most serious complications are heart failure and sudden death(Mc Nally et al., 2013).

关键词： cardiomyopathy ventricular

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Haploinsufficiency of syncoilin leads to hypertrophic cardiomyopathy

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Genes & Diseases 2022年第6期9卷 1412-1414页

作者： Liang-Liang Fan Zhuang-Zhuang Yuan Zi-Jun Jiao Hui Zeng Yi-Feng Yang Zhi-Ping Tan Clinical Center for Gene Diagnosis and Therapy Department of Cardiovascular SurgeryThe Second Xiangya Hospital of Central South UniversityChangshaHunan 410011PR China Department of Cell Biology The School of Life SciencesCentral South UniversityChangshaHunan 410013PR China Hunan Key Laboratory of Animal for Human Disease School of Life SciencesCentral South UniversityChangshaHunan 410013PR China

Here we reported a SYNC nonsense variant in a Chinese family with hypertrophic cardiomyopathy(HCM)and firstly linked syncoilin(SYNC)to HCM.HCM is an inherited cardiovascular disease,affecting approximately 1:500 people,that is characterized by thickening of left ventricle(LV),especially the interventricular septum(IVS),and diastolic ventricular failure.1 To date,more than 15 genes of two groups underlying HCM have been identified.1 About 35%e60%HCM patients present autosomal dominant inheritance and carry a pathogenic variant in sarcomeric protein genes,such as b-myosin heavy chain(MYH7),myosin binding protein C(MYBPC3),and Troponin T(TNNT2).1 In addition,non-sarcomeric genetic causes of disease have also been observed in about 25%HCM patients,mainly related to metabolic storage diseases,mitochondrial cardiomyopathies,inborn errors of metabolism etc.

关键词： metabolism cardiomyopathy ventricular

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Desmoplakin and clinical manifestations of desmoplakin cardiomyopathy

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Chinese Medical Journal 2021年第15期134卷 1771-1779页

作者： Zhong-Yu Yuan Li-Ting Cheng Ze-Feng Wang Yong-Quan Wu Department of Cardiology Beijing Anzhen HospitalCapital Medical UniversityBeijing 100029China

Desmoplakin(DSP),encoded by the DSP gene,is the main desmosome component and is abundant in the myocardial tissue.There are three DSP isoforms that assume the role of supporting structural stability through intercellular adhesion.It has been found that DSP regulates the transcription of adipogenic and fibrogenic genes,and maintains appropriate electrical conductivity by regulating gap junctions and ion channels.DSP is essential for normal myocardial development and the maintenance of its structural functions.Studies have suggested that DSP gene mutations are associated with a variety of hereditary cardiomyopathy,such as arrhythmia cardiomyopathy,dilated cardiomyopathy(DCM),left ventricular noncompaction,and is also closely associated with the Carvajal syndrome,Naxos disease,and erythro-keratodermia-cardiomyopathy syndrome with skin and heart damage.The structure and function of DSP,as well as the clinical manifestations of DSP-related cardiomyopathy were reviewed in this article.

关键词： Desmoplakin Desmosome Mutation Wnt Signaling Pathway cardiomyopathy

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