Several cardiac outcomes have been reported with West Nile-encephalitis;however, the underlying pathophysiology remains complex. We present a 42-year-old female, with multiple sclerosis, whose neurological symptoms an...Several cardiac outcomes have been reported with West Nile-encephalitis;however, the underlying pathophysiology remains complex. We present a 42-year-old female, with multiple sclerosis, whose neurological symptoms and respiratory decline were finally explained by the diagnosis of West Nile-encephalitis. During her admission, the isolated peaked T-waves indicated the underlying stress-induced cardiomyopathy. The absence of all other causes of hyperacute T-waves, their subsequent resolution with the resolution of infection and improvement in wall motion abnormalities, further supported the association. This case highlights the importance of considering hyperacute T-waves in an approach towards the diagnosis of WNV-encephalitis related atypical variant of stress-induced cardiomyopathy.展开更多
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
BACKGROUND Bradycardia-induced cardiomyopathy(BIC),which is a disease resulting from bradycardia,is characterized by cardiac chamber enlargement and diminished cardiac function.The correction of bradycardia can allow ...BACKGROUND Bradycardia-induced cardiomyopathy(BIC),which is a disease resulting from bradycardia,is characterized by cardiac chamber enlargement and diminished cardiac function.The correction of bradycardia can allow for significant improvements in both cardiac function and structure;however,this disease has been infrequently documented.In this case,we conducted a longitudinal followup of a patient who had been enduring BIC for more than 40 years to heighten awareness and prompt timely diagnosis and rational intervention.CASE SUMMARY A woman who presented with postactivity fatigue and dyspnea was diagnosed with bradycardia at the age of 7.Since she had no obvious symptoms,she did not receive any treatment to improve her bradycardia during the 42-year follow-up,except for the implantation of a temporary pacemaker during labor induction surgery.As time progressed,the patient's heart gradually expanded due to her low ventricular rate,and she was diagnosed with BIC.In 2014,the patient developed atrial fibrillation,her ventricular rate gradually increased,and her heart shape gradually returned to normal.This report describes the cardiac morphological changes caused by the heart rate changes in BIC patients older than 40 years,introduces another possible outcome of BIC,and emphasizes the importance of early intervention in treating BIC.CONCLUSION BIC can induce atrial fibrillation,causing an increased ventricular rate and leading to positive cardiac remodeling.展开更多
BACKGROUND Diabetic cardiomyopathy(DCM),which is a complication of diabetes,poses a great threat to public health.Recent studies have confirmed the role of NLRP3(NOD-like receptor protein 3)activation in DCM developme...BACKGROUND Diabetic cardiomyopathy(DCM),which is a complication of diabetes,poses a great threat to public health.Recent studies have confirmed the role of NLRP3(NOD-like receptor protein 3)activation in DCM development through the inflammatory response.Teneligliptin is an oral hypoglycemic dipeptidyl peptidase-IV inhibitor used to treat diabetes.Teneligliptin has recently been reported to have anti-inflammatory and protective effects on myocardial cells.AIM To examine the therapeutic effects of teneligliptin on DCM in diabetic mice.METHODS Streptozotocin was administered to induce diabetes in mice,followed by treatment with 30 mg/kg teneligliptin.RESULTS Marked increases in cardiomyocyte area and cardiac hypertrophy indicator heart weight/tibia length reductions in fractional shortening,ejection fraction,and heart rate;increases in creatine kinase-MB(CK-MB),aspartate transaminase(AST),and lactate dehydrogenase(LDH)levels;and upregulated NADPH oxidase 4 were observed in diabetic mice,all of which were significantly reversed by teneligliptin.Moreover,NLRP3 inflammasome activation and increased release of interleukin-1βin diabetic mice were inhibited by teneligliptin.Primary mouse cardiomyocytes were treated with high glucose(30 mmol/L)with or without teneligliptin(2.5 or 5μM)for 24 h.NLRP3 inflammasome activation.Increases in CKMB,AST,and LDH levels in glucose-stimulated cardiomyocytes were markedly inhibited by teneligliptin,and AMP(p-adenosine 5‘-monophosphate)-p-AMPK(activated protein kinase)levels were increased.Furthermore,the beneficial effects of teneligliptin on hyperglycaemia-induced cardiomyocytes were abolished by the AMPK signaling inhibitor compound C.CONCLUSION Overall,teneligliptin mitigated DCM by mitigating activation of the NLRP3 inflammasome.展开更多
Objective: This study aimed to evaluate the efficacy of trimetazidine on exercise capacity via a six-minute walk test in patients with ischaemic cardiomyopathy and also evaluate the effect of trimetazidine on left ven...Objective: This study aimed to evaluate the efficacy of trimetazidine on exercise capacity via a six-minute walk test in patients with ischaemic cardiomyopathy and also evaluate the effect of trimetazidine on left ventricular function via echocardiography in the same population. Methods: This prospective observational study, conducted at the National Institute of Cardiovascular Diseases in Dhaka, Bangladesh, enrolled 200 patients with ischaemic cardiomyopathy and a depressed left ventricular ejection fraction (LVEF Results: In this study (n = 200) of ischaemic cardiomyopathy patients, the mean age was 58 years, with 76% of the patients being male. All study subjects received GDMT (Guideline-Directed Medical Therapy) for angina and heart failure. Those who received the modified released form of trimetazidine developed lesions during the 1st and 2nd follow-ups, during which the LVEF, LVIDd, and six-minute walk distance significantly improved (p Conclusion: The findings of the present study demonstrated that the addition of modified-release trimetazidine to GDMT can improve exercise capacity and left ventricular function in patients with ischaemic cardiomyopathy.展开更多
Background: Peripartum cardiomyopathy (PPCM) is a rare disease that typically affects young, healthy women. Because PPCM is associated with significant mortality, timely diagnosis and management are essential. Ventric...Background: Peripartum cardiomyopathy (PPCM) is a rare disease that typically affects young, healthy women. Because PPCM is associated with significant mortality, timely diagnosis and management are essential. Ventricular tachycardia (VT) is a major complication and contributor to sudden death. Available data on VT in patients with PPCM are limited. Aim: This case report demonstrates the clinical presentation, antenatal care, and management of labor and delivery in a patient with PPCM complicated by VT. Case report: 36-year old patient G4P3 presents at 27 weeks gestation to the emergency department complaining of chest tightness, palpitations, and profuse sweating. Peripartum cardiomyopathy was diagnosed after her last pregnancy a few years prior. Ventricular tachycardia was diagnosed at this visit and treated successfully. The remainder of the pregnancy was uneventful until she had another episode of ventricular tachycardia during labor. Treatment using antiarrhythmics (diltiazem, amiodarone, adenosine) highlights the importance of prompt intervention and the need for a range of therapeutic options. Results: This case demonstrated successful VT management during pregnancy and labor, emphasizing multidisciplinary collaboration, influencing maternal and fetal outcomes positively, providing insights into optimal care strategies. Conclusion: Peripartum cardiomyopathy complicated by ventricular tachycardia is a life-threatening combination. This case highlights the importance of timely diagnosis and management with combined care between cardiologists, maternal fetal medicine specialists and anesthesiologists to prevent morbidities and sudden maternal death.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 Apical hypertrophic cardiomyopathy(AHCM)is a subtype of hypertrophic cardiomyopathy.Due to its location,the thickening of the left ventricular apex can be missed on echocardiography.Giant negative T waves(G...BACKGROUND Apical hypertrophic cardiomyopathy(AHCM)is a subtype of hypertrophic cardiomyopathy.Due to its location,the thickening of the left ventricular apex can be missed on echocardiography.Giant negative T waves(GNTs)in left-sided chest leads are the hallmark electrocardiogram(ECG)change of AHCM.CASE SUMMARY The first patient was a 68-year-old woman complaining of recurrent chest tightness persisting for more than 3 years.The second was a 59-year-old man complaining of spasmodic chest tightness persisting for more than 2 years.The third was a 55-year-old woman complaining of recurrent chest pain persisting for 4 mo.In all three cases,GNTs were observed several years prior to apical cardiac hypertrophy after other causes of T-wave inversion were ruled out.CONCLUSION Electrophysiological abnormalities of AHCM appear earlier than structural abnormalities,confirming the early predictive value of ECG for AHCM.展开更多
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 ...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.展开更多
BACKGROUND Diabetic cardiomyopathy(DCM)increases the risk of hospitalization for heart failure(HF)and mortality in patients with diabetes mellitus.However,no specific therapy to delay the progression of DCM has been i...BACKGROUND Diabetic cardiomyopathy(DCM)increases the risk of hospitalization for heart failure(HF)and mortality in patients with diabetes mellitus.However,no specific therapy to delay the progression of DCM has been identified.Mitochondrial dysfunction,oxidative stress,inflammation,and calcium handling imbalance play a crucial role in the pathological processes of DCM,ultimately leading to cardiomyocyte apoptosis and cardiac dysfunctions.Empagliflozin,a novel glucoselowering agent,has been confirmed to reduce the risk of hospitalization for HF in diabetic patients.Nevertheless,the molecular mechanisms by which this agent provides cardioprotection remain unclear.AIM To investigate the effects of empagliflozin on high glucose(HG)-induced oxidative stress and cardiomyocyte apoptosis and the underlying molecular mechanism.METHODS Twelve-week-old db/db mice and primary cardiomyocytes from neonatal rats stimulated with HG(30 mmol/L)were separately employed as in vivo and in vitro models.Echocardiography was used to evaluate cardiac function.Flow cytometry and TdT-mediated dUTP-biotin nick end labeling staining were used to assess apoptosis in myocardial cells.Mitochondrial function was assessed by cellular ATP levels and changes in mitochondrial membrane potential.Furthermore,intracellular reactive oxygen species production and superoxide dismutase activity were analyzed.Real-time quantitative PCR was used to analyze Bax and Bcl-2 mRNA expression.Western blot analysis was used to measure the phosphorylation of AMP-activated protein kinase(AMPK)and myosin phosphatase target subunit 1(MYPT1),as well as the peroxisome proliferator-activated receptor-γcoactivator-1α(PGC-1α)and active caspase-3 protein levels.RESULTSIn the in vivo experiment, db/db mice developed DCM. However, the treatment of db/db mice with empagliflozin(10 mg/kg/d) for 8 wk substantially enhanced cardiac function and significantly reduced myocardial apoptosis,accompanied by an increase in the phosphorylation of AMPK and PGC-1α protein levels, as well as a decrease inthe phosphorylation of MYPT1 in the heart. In the in vitro experiment, the findings indicate that treatment ofcardiomyocytes with empagliflozin (10 μM) or fasudil (FA) (a ROCK inhibitor, 100 μM) or overexpression of PGC-1α significantly attenuated HG-induced mitochondrial injury, oxidative stress, and cardiomyocyte apoptosis.However, the above effects were partly reversed by the addition of compound C (CC). In cells exposed to HG,empagliflozin treatment increased the protein levels of p-AMPK and PGC-1α protein while decreasing phosphorylatedMYPT1 levels, and these changes were mitigated by the addition of CC. Adding FA and overexpressingPGC-1α in cells exposed to HG substantially increased PGC-1α protein levels. In addition, no sodium-glucosecotransporter (SGLT)2 protein expression was detected in cardiomyocytes.CONCLUSION Empagliflozin partially achieves anti-oxidative stress and anti-apoptotic effects on cardiomyocytes under HGconditions by activating AMPK/PGC-1α and suppressing of the RhoA/ROCK pathway independent of SGLT2.展开更多
Background:Strength-trained athletes using anabolic androgenic steroids(AAS)have left ventricular(LV)hypertrophy and myocardial fibrosis that can lead to sudden cardiac death.A similar feature was described in athlete...Background:Strength-trained athletes using anabolic androgenic steroids(AAS)have left ventricular(LV)hypertrophy and myocardial fibrosis that can lead to sudden cardiac death.A similar feature was described in athletes with hypertrophic cardiomyopathy(HCM),which complicates the diagnosis for clinicians.In this context,we aimed to compare the LV function of the 2 populations by measuring global and regional strain and myocardial work using speckle-tracking imaging.Methods:Twenty-four strength-trained asymptomatic athletes using AAS(AAS-Athletes),22 athletes diagnosed with HCM(HCM-Athletes),and 20 healthy control athletes(Ctrl-Athletes)underwent a resting echocardiography to assess LV function.We evaluated LV global and regional strains and myocardial work,with an evaluation of the constructive work(CW),wasted work,and work efficiency(WE).Results:Compared to Ctrl-Athletes,both AAS-Athletes and HCM-Athletes had a thicker interventricular septum,with maj ored values in HCM-Athletes.LV strain was reduced in AAS-Athletes and even more in HCM-Athletes.Consequently,global WE was significantly diminished in both AAS and HCM-Athletes(93%±2%in Ctrl-Athletes,90%±4%in AAS-Athletes,and 90%±5%in HCM-Athletes(mean±SD);p<0.05).Constructive work and WE regional analysis showed specific alterations,with the basal septal segments preferentially affected in AAS-Athletes,and both septal and apical segments affected in HCM-Athletes.Conclusion:The regional evaluation of myocardial work reported specific alterations of the major LV hypertrophy induced by the regular use of AAS compared to the LV hypertrophy due to HCM.This finding could help clinicians to differentiate between these 2 forms of pathological hypertrophy.展开更多
Diabetic cardiomyopathy(DCM)is a metabolic disease and a leading cause of heart failure among people with diabetes.Mass spectrometry imaging(MSI)is a versatile technique capable of combining the molecular specificity ...Diabetic cardiomyopathy(DCM)is a metabolic disease and a leading cause of heart failure among people with diabetes.Mass spectrometry imaging(MSI)is a versatile technique capable of combining the molecular specificity of mass spectrometry(MS)with the spatial information of imaging.In this study,we used MSI to visualize metabolites in the rat heart with high spatial resolution and sensitivity.We optimized the air flow-assisted desorption electrospray ionization(AFADESI)-MSI platform to detect a wide range of metabolites,and then used matrix-assisted laser desorption ionization(MALDI)-MSI for increasing metabolic coverage and improving localization resolution.AFADESI-MSI detected 214 and 149 metabolites in positive and negative analyses of rat heart sections,respectively,while MALDI-MSI detected 61 metabolites in negative analysis.Our study revealed the heterogenous metabolic profile of the heart in a DCM model,with over 105 region-specific changes in the levels of a wide range of metabolite classes,including carbohydrates,amino acids,nucleotides,and their derivatives,fatty acids,glycerol phospholipids,carnitines,and metal ions.The repeated oral administration of ferulic acid during 20 weeks significantly improved most of the metabolic disorders in the DCM model.Our findings provide novel insights into the molecular mechanisms underlying DCM and the potential of ferulic acid as a therapeutic agent for treating this condition.展开更多
BACKGROUND Clinical diagnosis of cirrhotic cardiomyopathy(CCM) often encounters challenges of lack of timeliness and disease severity, with the commonly positive indicator usually associated with advanced heart failur...BACKGROUND Clinical diagnosis of cirrhotic cardiomyopathy(CCM) often encounters challenges of lack of timeliness and disease severity, with the commonly positive indicator usually associated with advanced heart failure.AIM To explore suitable biomarkers for early CCM prediction.METHODS A total of 505 eligible patients were enrolled in this study and divided into four groups according to Child-Pugh classification: Group Ⅰ, Class A without CCM(105 cases);Group Ⅱ, Class A with CCM(175 cases);Group Ⅲ, Class B with CCM(139 cases);and Group Ⅳ, Class C with CCM(86 cases). Logistic regression and receiver operating characteristic(ROC) curve analyses were performed to determine whether red blood cell distribution width(RDW) was an independent risk factor for CCM risk. The relationships between RDW and Child-Pugh scores, Model for End-Stage Liver Disease(MELD) scores, and N-terminal pro-brain natriuretic peptide(NT-proBNP) were analyzed by Pearson correlation analysis.RESULTS A constant RDW increase was evident from Group Ⅰ to Group Ⅳ(12.54 ± 0.85, 13.29 ± 1.19, 14.30 ± 1.96, and 16.25 ± 2.13, respectively). Pearson correlation analysis showed that RDW was positively correlated with Child-Pugh scores(r = 0.642, P < 0.001), MELD scores(r = 0.592, P < 0.001), and NT-proBNP(r = 0.715, P < 0.001). Furthermore, between Group Ⅰ and Group Ⅱ, RDW was the only significant index(odds ratio: 2.175, 95% confidence interval [CI]: 1.549-3.054, P < 0.001), and it reached statistical significance when examined by ROC curve analysis(area under the curve: 0.686, 95%CI: 0.624-0.748, P < 0.001).CONCLUSION RDW can serve as an effective and accessible clinical indicator for the prediction of diastolic dysfunction in CCM, in which a numerical value of more than 13.05% may indicate an increasing CCM risk.展开更多
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 hypertr...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.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 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
Cardiomyopathy is a pathological condition characterized by cardiac pump failure due to myocardial dysfunction and the major cause of advanced heart failure requiring heart transplantation.Although optimized medical t...Cardiomyopathy is a pathological condition characterized by cardiac pump failure due to myocardial dysfunction and the major cause of advanced heart failure requiring heart transplantation.Although optimized medical therapies have been developed for heart failure during the last few decades,some patients with cardiomyopathy exhibit advanced heart failure and are refractory to medical therapies.Desmosome,which is a dynamic cell-to-cell junctional component,maintains the structural integrity of heart tissues.Genetic mutations in desmo-somal genes cause arrhythmogenic cardiomyopathy(AC),a rare inheritable disease,and predispose patients to sudden cardiac death and heart failure.Recent advances in sequencing technologies have elucidated the genetic basis of cardiomyopathies and revealed that desmosome-related cardiomyopathy is concealed in broad cardiomyopathies.Among desmosomal genes,mutations in PKP2(which encodes PKP2)are most frequently identified in patients with AC.PKP2 deficiency causes various pathological cardiac phenotypes.Human cardiomyocytes differentiated from patient-derived induced pluripotent stem cells(iPSCs)in combination with genome editing,which allows the precise arrangement of the targeted genome,are powerful experimental tools for studying disease.This review summarizes the current issues associated with practical medicine for advanced heart failure and the recent advances in disease modeling using iPSC-derived cardiomyocytes targeting desmosome-related cardiomyopathy caused by PKP2 deficiency.展开更多
BACKGROUND Jiawei Jiaotai Pill is commonly used in clinical practice to reduce apoptosis,increase insulin secretion,and improve blood glucose tolerance.However,its mechanism of action in the treatment of diabetic card...BACKGROUND Jiawei Jiaotai Pill is commonly used in clinical practice to reduce apoptosis,increase insulin secretion,and improve blood glucose tolerance.However,its mechanism of action in the treatment of diabetic cardiomyopathy(DCM)remains unclear,hindering research efforts aimed at developing drugs specifically for the treatment of DCM.AIM To explore the pharmacodynamic basis and molecular mechanism of Jiawei Jiaotai Pill in DCM treatment.METHODS We explored various databases and software,including the Traditional Chinese Medicine Systems Pharmacology Database,Uniport,PubChem,GenCards,String,and Cytoscape,to identify the active components and targets of Jiawei Jiaotai Pill,and the disease targets in DCM.Protein-protein interaction network,gene ontology,and Kyoto Encyclopedia of Genes and Genomes analyses were used to determine the mechanism of action of Jiawei Jiaotai Pill in treating DCM.Molecular docking of key active components and core targets was verified using AutoDock software.RESULTS Total 42 active ingredients and 142 potential targets of Jiawei Jiaotai Pill were identified.There were 100 common targets between the DCM and Jiawei Jiaotai Pills.Through this screening process,TNF,IL6,TP53,EGFR,INS,and other important targets were identified.These targets are mainly involved in the positive regulation of the mitogen-activated protein kinase(MAPK)MAPK cascade,response to xenobiotic stimuli,response to hypoxia,positive regulation of gene expression,positive regulation of cell proliferation,negative regulation of the apoptotic process,and other biological processes.It was mainly enriched in the AGE-RAGE signaling pathway in diabetic complications,DCM,PI3K-Akt,interleukin-17,and MAPK signaling pathways.Molecular docking results showed that Jiawei Jiaotai Pill's active ingredients had good docking activity with DCM's core target.CONCLUSION The active components of Jiawei Jiaotai Pill may play a role in the treatment of DCM by reducing oxidative stress,cardiomyocyte apoptosis and fibrosis,and maintaining metabolic homeostasis.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 Takotsubo cardiomyopathy(TTC)can be diagnosed in patients presenting with clinical features of acute coronary syndrome(ACS)by using Mayo clinic criteria.Multiple precipitators have been attributed to causin...BACKGROUND Takotsubo cardiomyopathy(TTC)can be diagnosed in patients presenting with clinical features of acute coronary syndrome(ACS)by using Mayo clinic criteria.Multiple precipitators have been attributed to causing TTC.Rarely it has been reported to occur following an acute envenomation.AIM This review describes the various patterns,mechanisms,and outcomes of envenomation induced TTC.METHODS In this review,we included all studies on“TTC”and“envenomation”published in the various databases before June 2022.To be included in the review articles had to have a distinct diagnosis of TTC and an envenomation RESULTS A total of 20 patients with envenomation induced TTC were identified.Most episodes of envenomation induced TTC were reported following a bee sting,scorpion sting,and snake envenomation.Fear and anxiety related to the sting,direct catecholamine toxicity and administration of exogenous beta-adrenergic agents have been commonly postulated to precipitate TTC in these patients.95%of these patients presented with a clinical picture of ACS.Most of these patients also fulfill at least 3 out of 4 criteria of Mayo clinic criteria for TTC.Echocardiographic evidence of Apical TTC was noted in 72%of patients.94%of these patients had clinical improvement following optimal management and 35%of these patients were treated with guideline directed medications for heart failure.CONCLUSION Envenomation following multiple insect stings and reptile bites can precipitate TTC.Most reported envenomation related TTC has been due to bee stings and scorpion bites.Common mechanisms causing TTC were fear,anxiety,and stress of envenomation.Most of these patients present with clinical presentation of ACS,ST elevation,and elevated troponin.The most common type of TTC in these patients is Apical,which improved following medical management.展开更多
文摘Several cardiac outcomes have been reported with West Nile-encephalitis;however, the underlying pathophysiology remains complex. We present a 42-year-old female, with multiple sclerosis, whose neurological symptoms and respiratory decline were finally explained by the diagnosis of West Nile-encephalitis. During her admission, the isolated peaked T-waves indicated the underlying stress-induced cardiomyopathy. The absence of all other causes of hyperacute T-waves, their subsequent resolution with the resolution of infection and improvement in wall motion abnormalities, further supported the association. This case highlights the importance of considering hyperacute T-waves in an approach towards the diagnosis of WNV-encephalitis related atypical variant of stress-induced cardiomyopathy.
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.
基金Supported by National Natural Science Foundation of China,No.81970241Tianfu Qingcheng Project-Tianfu Science and Technology Elite,No.1358.
文摘BACKGROUND Bradycardia-induced cardiomyopathy(BIC),which is a disease resulting from bradycardia,is characterized by cardiac chamber enlargement and diminished cardiac function.The correction of bradycardia can allow for significant improvements in both cardiac function and structure;however,this disease has been infrequently documented.In this case,we conducted a longitudinal followup of a patient who had been enduring BIC for more than 40 years to heighten awareness and prompt timely diagnosis and rational intervention.CASE SUMMARY A woman who presented with postactivity fatigue and dyspnea was diagnosed with bradycardia at the age of 7.Since she had no obvious symptoms,she did not receive any treatment to improve her bradycardia during the 42-year follow-up,except for the implantation of a temporary pacemaker during labor induction surgery.As time progressed,the patient's heart gradually expanded due to her low ventricular rate,and she was diagnosed with BIC.In 2014,the patient developed atrial fibrillation,her ventricular rate gradually increased,and her heart shape gradually returned to normal.This report describes the cardiac morphological changes caused by the heart rate changes in BIC patients older than 40 years,introduces another possible outcome of BIC,and emphasizes the importance of early intervention in treating BIC.CONCLUSION BIC can induce atrial fibrillation,causing an increased ventricular rate and leading to positive cardiac remodeling.
基金Supported by National Natural Science Foundation of China,No.82000276the Science and Technology Project of Jiangxi Provincial Health Commission,No.202310005.
文摘BACKGROUND Diabetic cardiomyopathy(DCM),which is a complication of diabetes,poses a great threat to public health.Recent studies have confirmed the role of NLRP3(NOD-like receptor protein 3)activation in DCM development through the inflammatory response.Teneligliptin is an oral hypoglycemic dipeptidyl peptidase-IV inhibitor used to treat diabetes.Teneligliptin has recently been reported to have anti-inflammatory and protective effects on myocardial cells.AIM To examine the therapeutic effects of teneligliptin on DCM in diabetic mice.METHODS Streptozotocin was administered to induce diabetes in mice,followed by treatment with 30 mg/kg teneligliptin.RESULTS Marked increases in cardiomyocyte area and cardiac hypertrophy indicator heart weight/tibia length reductions in fractional shortening,ejection fraction,and heart rate;increases in creatine kinase-MB(CK-MB),aspartate transaminase(AST),and lactate dehydrogenase(LDH)levels;and upregulated NADPH oxidase 4 were observed in diabetic mice,all of which were significantly reversed by teneligliptin.Moreover,NLRP3 inflammasome activation and increased release of interleukin-1βin diabetic mice were inhibited by teneligliptin.Primary mouse cardiomyocytes were treated with high glucose(30 mmol/L)with or without teneligliptin(2.5 or 5μM)for 24 h.NLRP3 inflammasome activation.Increases in CKMB,AST,and LDH levels in glucose-stimulated cardiomyocytes were markedly inhibited by teneligliptin,and AMP(p-adenosine 5‘-monophosphate)-p-AMPK(activated protein kinase)levels were increased.Furthermore,the beneficial effects of teneligliptin on hyperglycaemia-induced cardiomyocytes were abolished by the AMPK signaling inhibitor compound C.CONCLUSION Overall,teneligliptin mitigated DCM by mitigating activation of the NLRP3 inflammasome.
文摘Objective: This study aimed to evaluate the efficacy of trimetazidine on exercise capacity via a six-minute walk test in patients with ischaemic cardiomyopathy and also evaluate the effect of trimetazidine on left ventricular function via echocardiography in the same population. Methods: This prospective observational study, conducted at the National Institute of Cardiovascular Diseases in Dhaka, Bangladesh, enrolled 200 patients with ischaemic cardiomyopathy and a depressed left ventricular ejection fraction (LVEF Results: In this study (n = 200) of ischaemic cardiomyopathy patients, the mean age was 58 years, with 76% of the patients being male. All study subjects received GDMT (Guideline-Directed Medical Therapy) for angina and heart failure. Those who received the modified released form of trimetazidine developed lesions during the 1st and 2nd follow-ups, during which the LVEF, LVIDd, and six-minute walk distance significantly improved (p Conclusion: The findings of the present study demonstrated that the addition of modified-release trimetazidine to GDMT can improve exercise capacity and left ventricular function in patients with ischaemic cardiomyopathy.
文摘Background: Peripartum cardiomyopathy (PPCM) is a rare disease that typically affects young, healthy women. Because PPCM is associated with significant mortality, timely diagnosis and management are essential. Ventricular tachycardia (VT) is a major complication and contributor to sudden death. Available data on VT in patients with PPCM are limited. Aim: This case report demonstrates the clinical presentation, antenatal care, and management of labor and delivery in a patient with PPCM complicated by VT. Case report: 36-year old patient G4P3 presents at 27 weeks gestation to the emergency department complaining of chest tightness, palpitations, and profuse sweating. Peripartum cardiomyopathy was diagnosed after her last pregnancy a few years prior. Ventricular tachycardia was diagnosed at this visit and treated successfully. The remainder of the pregnancy was uneventful until she had another episode of ventricular tachycardia during labor. Treatment using antiarrhythmics (diltiazem, amiodarone, adenosine) highlights the importance of prompt intervention and the need for a range of therapeutic options. Results: This case demonstrated successful VT management during pregnancy and labor, emphasizing multidisciplinary collaboration, influencing maternal and fetal outcomes positively, providing insights into optimal care strategies. Conclusion: Peripartum cardiomyopathy complicated by ventricular tachycardia is a life-threatening combination. This case highlights the importance of timely diagnosis and management with combined care between cardiologists, maternal fetal medicine specialists and anesthesiologists to prevent morbidities and sudden maternal death.
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 Apical hypertrophic cardiomyopathy(AHCM)is a subtype of hypertrophic cardiomyopathy.Due to its location,the thickening of the left ventricular apex can be missed on echocardiography.Giant negative T waves(GNTs)in left-sided chest leads are the hallmark electrocardiogram(ECG)change of AHCM.CASE SUMMARY The first patient was a 68-year-old woman complaining of recurrent chest tightness persisting for more than 3 years.The second was a 59-year-old man complaining of spasmodic chest tightness persisting for more than 2 years.The third was a 55-year-old woman complaining of recurrent chest pain persisting for 4 mo.In all three cases,GNTs were observed several years prior to apical cardiac hypertrophy after other causes of T-wave inversion were ruled out.CONCLUSION Electrophysiological abnormalities of AHCM appear earlier than structural abnormalities,confirming the early predictive value of ECG for AHCM.
基金the National Natural Science Foundation of China (No.81870286)the CAM Innovation Fund for Medical Science (2022-I2M1-005 & 2020-I2M-C&T-A-006)。
文摘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.
基金Health Commission of Hebei Province,No.20210196S&T Program of Hebei,No.22377726D。
文摘BACKGROUND Diabetic cardiomyopathy(DCM)increases the risk of hospitalization for heart failure(HF)and mortality in patients with diabetes mellitus.However,no specific therapy to delay the progression of DCM has been identified.Mitochondrial dysfunction,oxidative stress,inflammation,and calcium handling imbalance play a crucial role in the pathological processes of DCM,ultimately leading to cardiomyocyte apoptosis and cardiac dysfunctions.Empagliflozin,a novel glucoselowering agent,has been confirmed to reduce the risk of hospitalization for HF in diabetic patients.Nevertheless,the molecular mechanisms by which this agent provides cardioprotection remain unclear.AIM To investigate the effects of empagliflozin on high glucose(HG)-induced oxidative stress and cardiomyocyte apoptosis and the underlying molecular mechanism.METHODS Twelve-week-old db/db mice and primary cardiomyocytes from neonatal rats stimulated with HG(30 mmol/L)were separately employed as in vivo and in vitro models.Echocardiography was used to evaluate cardiac function.Flow cytometry and TdT-mediated dUTP-biotin nick end labeling staining were used to assess apoptosis in myocardial cells.Mitochondrial function was assessed by cellular ATP levels and changes in mitochondrial membrane potential.Furthermore,intracellular reactive oxygen species production and superoxide dismutase activity were analyzed.Real-time quantitative PCR was used to analyze Bax and Bcl-2 mRNA expression.Western blot analysis was used to measure the phosphorylation of AMP-activated protein kinase(AMPK)and myosin phosphatase target subunit 1(MYPT1),as well as the peroxisome proliferator-activated receptor-γcoactivator-1α(PGC-1α)and active caspase-3 protein levels.RESULTSIn the in vivo experiment, db/db mice developed DCM. However, the treatment of db/db mice with empagliflozin(10 mg/kg/d) for 8 wk substantially enhanced cardiac function and significantly reduced myocardial apoptosis,accompanied by an increase in the phosphorylation of AMPK and PGC-1α protein levels, as well as a decrease inthe phosphorylation of MYPT1 in the heart. In the in vitro experiment, the findings indicate that treatment ofcardiomyocytes with empagliflozin (10 μM) or fasudil (FA) (a ROCK inhibitor, 100 μM) or overexpression of PGC-1α significantly attenuated HG-induced mitochondrial injury, oxidative stress, and cardiomyocyte apoptosis.However, the above effects were partly reversed by the addition of compound C (CC). In cells exposed to HG,empagliflozin treatment increased the protein levels of p-AMPK and PGC-1α protein while decreasing phosphorylatedMYPT1 levels, and these changes were mitigated by the addition of CC. Adding FA and overexpressingPGC-1α in cells exposed to HG substantially increased PGC-1α protein levels. In addition, no sodium-glucosecotransporter (SGLT)2 protein expression was detected in cardiomyocytes.CONCLUSION Empagliflozin partially achieves anti-oxidative stress and anti-apoptotic effects on cardiomyocytes under HGconditions by activating AMPK/PGC-1α and suppressing of the RhoA/ROCK pathway independent of SGLT2.
基金supported by YAKHA Sport,Franceby the Platform 3A,funded by the European Regional Development Fund+3 种基金the French Ministry of Research,Higher Education and Innovationthe Provence-Alpes-Côte-d'Azur regionthe Departmental Council of Vauclusethe Urban Community of Avignon。
文摘Background:Strength-trained athletes using anabolic androgenic steroids(AAS)have left ventricular(LV)hypertrophy and myocardial fibrosis that can lead to sudden cardiac death.A similar feature was described in athletes with hypertrophic cardiomyopathy(HCM),which complicates the diagnosis for clinicians.In this context,we aimed to compare the LV function of the 2 populations by measuring global and regional strain and myocardial work using speckle-tracking imaging.Methods:Twenty-four strength-trained asymptomatic athletes using AAS(AAS-Athletes),22 athletes diagnosed with HCM(HCM-Athletes),and 20 healthy control athletes(Ctrl-Athletes)underwent a resting echocardiography to assess LV function.We evaluated LV global and regional strains and myocardial work,with an evaluation of the constructive work(CW),wasted work,and work efficiency(WE).Results:Compared to Ctrl-Athletes,both AAS-Athletes and HCM-Athletes had a thicker interventricular septum,with maj ored values in HCM-Athletes.LV strain was reduced in AAS-Athletes and even more in HCM-Athletes.Consequently,global WE was significantly diminished in both AAS and HCM-Athletes(93%±2%in Ctrl-Athletes,90%±4%in AAS-Athletes,and 90%±5%in HCM-Athletes(mean±SD);p<0.05).Constructive work and WE regional analysis showed specific alterations,with the basal septal segments preferentially affected in AAS-Athletes,and both septal and apical segments affected in HCM-Athletes.Conclusion:The regional evaluation of myocardial work reported specific alterations of the major LV hypertrophy induced by the regular use of AAS compared to the LV hypertrophy due to HCM.This finding could help clinicians to differentiate between these 2 forms of pathological hypertrophy.
基金supported by the National Natural Science Foundation of China(Grant Nos.:21927808 and 81803483).
文摘Diabetic cardiomyopathy(DCM)is a metabolic disease and a leading cause of heart failure among people with diabetes.Mass spectrometry imaging(MSI)is a versatile technique capable of combining the molecular specificity of mass spectrometry(MS)with the spatial information of imaging.In this study,we used MSI to visualize metabolites in the rat heart with high spatial resolution and sensitivity.We optimized the air flow-assisted desorption electrospray ionization(AFADESI)-MSI platform to detect a wide range of metabolites,and then used matrix-assisted laser desorption ionization(MALDI)-MSI for increasing metabolic coverage and improving localization resolution.AFADESI-MSI detected 214 and 149 metabolites in positive and negative analyses of rat heart sections,respectively,while MALDI-MSI detected 61 metabolites in negative analysis.Our study revealed the heterogenous metabolic profile of the heart in a DCM model,with over 105 region-specific changes in the levels of a wide range of metabolite classes,including carbohydrates,amino acids,nucleotides,and their derivatives,fatty acids,glycerol phospholipids,carnitines,and metal ions.The repeated oral administration of ferulic acid during 20 weeks significantly improved most of the metabolic disorders in the DCM model.Our findings provide novel insights into the molecular mechanisms underlying DCM and the potential of ferulic acid as a therapeutic agent for treating this condition.
基金Supported by the Fujian Provincial Education and Scientific Research Project,No.JAT200121Fujian Provincial Health Technology Project,No.2021QNA021.
文摘BACKGROUND Clinical diagnosis of cirrhotic cardiomyopathy(CCM) often encounters challenges of lack of timeliness and disease severity, with the commonly positive indicator usually associated with advanced heart failure.AIM To explore suitable biomarkers for early CCM prediction.METHODS A total of 505 eligible patients were enrolled in this study and divided into four groups according to Child-Pugh classification: Group Ⅰ, Class A without CCM(105 cases);Group Ⅱ, Class A with CCM(175 cases);Group Ⅲ, Class B with CCM(139 cases);and Group Ⅳ, Class C with CCM(86 cases). Logistic regression and receiver operating characteristic(ROC) curve analyses were performed to determine whether red blood cell distribution width(RDW) was an independent risk factor for CCM risk. The relationships between RDW and Child-Pugh scores, Model for End-Stage Liver Disease(MELD) scores, and N-terminal pro-brain natriuretic peptide(NT-proBNP) were analyzed by Pearson correlation analysis.RESULTS A constant RDW increase was evident from Group Ⅰ to Group Ⅳ(12.54 ± 0.85, 13.29 ± 1.19, 14.30 ± 1.96, and 16.25 ± 2.13, respectively). Pearson correlation analysis showed that RDW was positively correlated with Child-Pugh scores(r = 0.642, P < 0.001), MELD scores(r = 0.592, P < 0.001), and NT-proBNP(r = 0.715, P < 0.001). Furthermore, between Group Ⅰ and Group Ⅱ, RDW was the only significant index(odds ratio: 2.175, 95% confidence interval [CI]: 1.549-3.054, P < 0.001), and it reached statistical significance when examined by ROC curve analysis(area under the curve: 0.686, 95%CI: 0.624-0.748, P < 0.001).CONCLUSION RDW can serve as an effective and accessible clinical indicator for the prediction of diastolic dysfunction in CCM, in which a numerical value of more than 13.05% may indicate an increasing CCM risk.
基金supported by grants from the National Key Research and Development Program of China(2016YFC1300100)National Natural Science Foundation of China(81974042)Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences(2019XK320058 and 2019XK320057).
文摘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.
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.
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 JSPS KAKENHI,No.20K21602,No.21H02915,and No.22K19526the Japan Agency for Medical Research and Development,No.21bm0804008h0005+2 种基金the Cell Science Research Foundationthe Grant for Basic Research of the Japanese Circulation Society(2018)SENSHIN Medical Research Foundation.
文摘Cardiomyopathy is a pathological condition characterized by cardiac pump failure due to myocardial dysfunction and the major cause of advanced heart failure requiring heart transplantation.Although optimized medical therapies have been developed for heart failure during the last few decades,some patients with cardiomyopathy exhibit advanced heart failure and are refractory to medical therapies.Desmosome,which is a dynamic cell-to-cell junctional component,maintains the structural integrity of heart tissues.Genetic mutations in desmo-somal genes cause arrhythmogenic cardiomyopathy(AC),a rare inheritable disease,and predispose patients to sudden cardiac death and heart failure.Recent advances in sequencing technologies have elucidated the genetic basis of cardiomyopathies and revealed that desmosome-related cardiomyopathy is concealed in broad cardiomyopathies.Among desmosomal genes,mutations in PKP2(which encodes PKP2)are most frequently identified in patients with AC.PKP2 deficiency causes various pathological cardiac phenotypes.Human cardiomyocytes differentiated from patient-derived induced pluripotent stem cells(iPSCs)in combination with genome editing,which allows the precise arrangement of the targeted genome,are powerful experimental tools for studying disease.This review summarizes the current issues associated with practical medicine for advanced heart failure and the recent advances in disease modeling using iPSC-derived cardiomyocytes targeting desmosome-related cardiomyopathy caused by PKP2 deficiency.
基金Supported by Natural Science Basic Research Plan in the Shaanxi Province of China,No.2021JM-549,The Plan Project of Shaanxi Provincial Administration of Traditional Chinese Medicine,No.2021-ZZ-JC011The Second Youth Science and Technology Talents Project of Shaanxi Provincial Administration of Traditional Chinese Medicine,No.2023-ZQNY-017.
文摘BACKGROUND Jiawei Jiaotai Pill is commonly used in clinical practice to reduce apoptosis,increase insulin secretion,and improve blood glucose tolerance.However,its mechanism of action in the treatment of diabetic cardiomyopathy(DCM)remains unclear,hindering research efforts aimed at developing drugs specifically for the treatment of DCM.AIM To explore the pharmacodynamic basis and molecular mechanism of Jiawei Jiaotai Pill in DCM treatment.METHODS We explored various databases and software,including the Traditional Chinese Medicine Systems Pharmacology Database,Uniport,PubChem,GenCards,String,and Cytoscape,to identify the active components and targets of Jiawei Jiaotai Pill,and the disease targets in DCM.Protein-protein interaction network,gene ontology,and Kyoto Encyclopedia of Genes and Genomes analyses were used to determine the mechanism of action of Jiawei Jiaotai Pill in treating DCM.Molecular docking of key active components and core targets was verified using AutoDock software.RESULTS Total 42 active ingredients and 142 potential targets of Jiawei Jiaotai Pill were identified.There were 100 common targets between the DCM and Jiawei Jiaotai Pills.Through this screening process,TNF,IL6,TP53,EGFR,INS,and other important targets were identified.These targets are mainly involved in the positive regulation of the mitogen-activated protein kinase(MAPK)MAPK cascade,response to xenobiotic stimuli,response to hypoxia,positive regulation of gene expression,positive regulation of cell proliferation,negative regulation of the apoptotic process,and other biological processes.It was mainly enriched in the AGE-RAGE signaling pathway in diabetic complications,DCM,PI3K-Akt,interleukin-17,and MAPK signaling pathways.Molecular docking results showed that Jiawei Jiaotai Pill's active ingredients had good docking activity with DCM's core target.CONCLUSION The active components of Jiawei Jiaotai Pill may play a role in the treatment of DCM by reducing oxidative stress,cardiomyocyte apoptosis and fibrosis,and maintaining metabolic homeostasis.
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.
文摘BACKGROUND Takotsubo cardiomyopathy(TTC)can be diagnosed in patients presenting with clinical features of acute coronary syndrome(ACS)by using Mayo clinic criteria.Multiple precipitators have been attributed to causing TTC.Rarely it has been reported to occur following an acute envenomation.AIM This review describes the various patterns,mechanisms,and outcomes of envenomation induced TTC.METHODS In this review,we included all studies on“TTC”and“envenomation”published in the various databases before June 2022.To be included in the review articles had to have a distinct diagnosis of TTC and an envenomation RESULTS A total of 20 patients with envenomation induced TTC were identified.Most episodes of envenomation induced TTC were reported following a bee sting,scorpion sting,and snake envenomation.Fear and anxiety related to the sting,direct catecholamine toxicity and administration of exogenous beta-adrenergic agents have been commonly postulated to precipitate TTC in these patients.95%of these patients presented with a clinical picture of ACS.Most of these patients also fulfill at least 3 out of 4 criteria of Mayo clinic criteria for TTC.Echocardiographic evidence of Apical TTC was noted in 72%of patients.94%of these patients had clinical improvement following optimal management and 35%of these patients were treated with guideline directed medications for heart failure.CONCLUSION Envenomation following multiple insect stings and reptile bites can precipitate TTC.Most reported envenomation related TTC has been due to bee stings and scorpion bites.Common mechanisms causing TTC were fear,anxiety,and stress of envenomation.Most of these patients present with clinical presentation of ACS,ST elevation,and elevated troponin.The most common type of TTC in these patients is Apical,which improved following medical management.
