Bicuspid aortic valve with associated aortopathy, significant left ventricular hypertrophy or concomitant hypertrophic cardiomyopathy: A diagnostic and therapeutic challenge

Due to its prevalence of 0.5%to 2%in the general population,with a 75%predominance among men,bicuspid aortic valve is the most common congenital heart defect.It is frequently accompanied by other cardiac congenital anomalies,and clinical presentation can vary significantly,with stenosis being the most common manifestation,often resulting in mild to moderate concentric hypertrophy of the left ventricle.Echocardiography is the primary diagnostic modality utilized for establishing the diagnosis,and it is often the sole diagnostic tool relied upon by clinicians.However,due to the heterogeneous clinical presentation and possible associated anomalies(which are often overlooked in clinical practice),it is necessary to employ various diagnostic methods and persist in finding the accurate diagnosis if multiple inconsistencies exist.By employing this approach,we can effectively manage these patients and provide them with appropriate treatment.Through a clinical case from our practice,we provide an overview of the literature on bicuspid aortic valve with aortophaty and the possible association with hypertrophic cardiomyopathy,diagnostic methods,and treatment options.This review article highlights the critical significance of achieving an accurate diagnosis in patients with bicuspid aortic valve and significant left ventricular hypertrophy.It is crucial to exclude other possible causes of left ventricular outflow tract obstruction,such as sub-or supra-aortic obstructions,and hypertrophic cardiomyopathy.

The Concordance Rates between LV Hypertrophy and RV Hypertrophy in Patients with Hypertrophic Cardiomyopathy as Diagnosed by Cardiovascular MRI with Fibrosis Imaging

Introduction: CMR has become the leading modality to define the clinical impact of hypertrophic cardiomyopathy (HCM). Late gadolinium enhancement (LGE) accurately identifies regions of myocardial fibrosis. It is well known that myocardial fibrosis can occur in patients with HCM and is independently linked to a poorer prognosis than those without fibrosis by CMR. Hypothesis: We hypothesize that there is significant RV involvement in HCM when incorporates a CMR analysis for RV hypertrophy and fibrosis. Methods: A retrospective review of all patients referred for HCM was performed. SSFP/LGE techniques were used to diagnose patients with HCM, using gadolinium administration (0.15 mmol/kg). Post-injection (10 minutes) LGE images were obtained using manual T1-weighted, IR-preparations. Regions of myocardium with LGE signals were visually designated as fibrotic. LV/RV mass indices (LVMI/RVMI) and ejection fractions were calculated. Results: Via 72 patients referred for HCM, 47(65%) were CMR confirmed. The mean LVMI was 108 ± 44 g/m2 while the mean RVMI was 30 ± 21 g/m2. As well, 34/47 (72%) had evidence of LV fibrosis while 24/47 (51%) had evidence for RV fibrosis. Of the RVH positive patients, 26/34 (76%) patients were LV LGE positive and 18/34 (52%) were RV LGE positive. Conclusion: The high frequency of RVH and RV fibrosis in the setting of HCM is surprising in that this phenomenon is rarely described. However, there is no reason to expect the phenotypic expression should be limited to the LV. Interestingly, as for the LV, the presence or absence of RV fibrosis has little predictive power towards the systolic function.

Isolated Hyperacute T-Waves in West Nile Encephalitis Indicating Atypical Variant of Stress-Induced Cardiomyopathy

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.

An initial study on left ventricular diastolic function in patients with hypertrophy cardiomyopathy using single-beat, real-time, three-dimensional echocardiography

ObjectivesTo 由使用单个敲击的、即时、三维的 echocardiography (RT-3DE ) 与 hypertrophic 心肌症(HCM ) 在病人估计地区性的心脏舒张的功能 .MethodsSixty -- 有在和五十匹配年龄、匹配性的正常控制的湾穴节奏的 HCM 的五个病人被二维的 echocardiography (2DE ) 和 RT-3DE 学习。参数由包括的 RT-3DE 分析了：留给室( LV )体积，左室的喷射部分( LVEF )，结束心脏舒张的球状索引( EDSI )，心脏舒张的 dyssynchrony 索引( DDI )，分散结束扩张( DISPED )，并且由 RT-3DE 使 17 部分卷时间 curves.ResultsEvaluated 正常化， LVEF 与 2DE 相比是稍微更低的( 63.2 &#x000b1 ；6.8% 对 59.1 &#x000b1；6.4% ， P &#x0003c；0.0001 ) 。正常题目为所有 LV 片断有相对一致的容量的曲线。在 HCM 病人，部分容量的曲线是 dyssynchronous。在结束扩张的增加的 DDI 和 DISPED 与 HCM 在病人被观察(9.95 &#x000b1；3.75， 41.76 &#x000b1；17.19， P &#x0003c；0.0001 ) ，并且并非所有反常容量的片断与 HCM 发生在 hypertrophic regions.ConclusionsPatients 在扩张阶段，和这现出症状之前的潜的损害介绍了地区性的心脏舒张的 dyssynchrony 能被单个敲击的 RT-3DE 认出。

Effect of Trimetazidine on Functional Capacity in Patient with Ischaemic Cardiomyopathy (TOFCAPI)

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.

Atorvastatin ameliorated myocardial fibrosis by inhibiting oxidative stress and modulating macrophage polarization in diabetic cardiomyopathy

In this editorial,we commented on the article published in the recent issue of the World Journal of Diabetes.Diabetic cardiomyopathy(DCM)is characterized by myocardial fibrosis,ventricular hypertrophy and diastolic dysfunction in diabetic patients,which can cause heart failure and threaten the life of patients.The pathogenesis of DCM has not been fully clarified,and it may involve oxidative stress,inflammatory stimulation,apoptosis,and autophagy.There is lack of effective therapies for DCM in the clinical practice.Statins have been widely used in the clinical practice for years mainly to reduce cholesterol and stabilize arterial plaques,and exhibit definite cardiovascular protective effects.Studies have shown that statins also have anti-inflammatory and antioxidant effects.We were particularly concerned about the recent findings that atorvastatin alleviated myocardial fibrosis in db/db mice by regulating the antioxidant stress and antiinflammatory effects of macrophage polarization on diabetic myocardium,and thereby improving DCM.

Teneligliptin mitigates diabetic cardiomyopathy by inhibiting activation of the NLRP3 inflammasome

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.

Peripartum Cardiomyopathy Complicated by Ventricular Tachycardia during Labor: A Case Report and Literature Review

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.

Cardiac remodeling in patients with atrial fibrillation reversing bradycardia-induced cardiomyopathy:A case report

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.

Cardioprotective Potential of Cymbopogon citratus Essential Oil against Isoproterenol-induced Cardiomyocyte Hypertrophy:Possible Involvement of NLRP3 Inflammasome and Oxidative Phosphorylation Complex Subunits

Objective:Cymbopogon citratus(DC.)Stapf is a medicinal and edible herb that is widely used for the treatment of gastric,nervous and hypertensive disorders.In this study,we investigated the cardioprotective effects and mechanisms of the essential oil,the main active ingredient of Cymbopogon citratus,on isoproterenol(ISO)-induced cardiomyocyte hypertrophy.Methods:The compositions of Cymbopogon citratus essential oil(CCEO)were determined by gas chromatography-mass spectrometry.Cardiomyocytes were pretreated with 16.9µg/L CCEO for 1 h followed by 10µmol/L ISO for 24 h.Cardiac hypertrophy-related indicators and NLRP3 inflammasome expression were evaluated.Subsequently,transcriptome sequencing(RNA-seq)and target verification were used to further explore the underlying mechanism.Results:Our results showed that the CCEO mainly included citronellal(45.66%),geraniol(23.32%),and citronellol(10.37%).CCEO inhibited ISO-induced increases in cell surface area and protein content,as well as the upregulation of fetal gene expression.Moreover,CCEO inhibited ISO-induced NLRP3 inflammasome expression,as evidenced by decreased lactate dehydrogenase content and downregulated mRNA levels of NLRP3,ASC,CASP1,GSDMD,and IL-1β,as well as reduced protein levels of NLRP3,ASC,pro-caspase-1,caspase-1(p20),GSDMD-FL,GSDMD-N,and pro-IL-1β.The RNA-seq results showed that CCEO inhibited the increase in the mRNA levels of 26 oxidative phosphorylation complex subunits in ISO-treated cardiomyocytes.Our further experiments confirmed that CCEO suppressed ISO-induced upregulation of mt-Nd1,Sdhd,mt-Cytb,Uqcrq,and mt-Atp6 but had no obvious effects on mt-Col expression.Conclusion:CCEO inhibits ISO-induced cardiomyocyte hypertrophy through the suppression of NLRP3 inflammasome expression and the regulation of several oxidative phosphorylation complex subunits.

Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report

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.

Takotsubo Cardiomyopathy Triggered by Acute Pancreatitis: A Case Report

Takotsubo cardiomyopathy is a heart condition that is widely known to be caused by stress. It presents with symptoms that are similar to a myocardial infarction even though the coronary arteries are clear. This case report details the clinical characteristics, diagnostic assessment, and management plan of a 55-year-old male patient with a past medical history of alcoholism who arrived at the emergency department with the typical symptoms of acute pancreatitis. The case demonstrates the progression of Takotsubo cardiomyopathy, which was triggered by acute pancreatitis in the context of alcoholism, and underlines the significance of early detection and management to enhance the patient’s outcomes.

Construction of A Prediction Model for Atrial Fibrillation in Patients with Dilated Cardiomyopathy and Heart Failure

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.

Predicting apical hypertrophic cardiomyopathy using T-wave inversion:Three case reports

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.

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

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.

Empagliflozin ameliorates diabetic cardiomyopathy probably via activating AMPK/PGC-1αand inhibiting the RhoA/ROCK pathway

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.

Value of red blood cell distribution width in prediction of diastolic dysfunction in cirrhotic cardiomyopathy

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.

Hypertrophic cardiomyopathy secondary to deficiency in lysosomeassociated membrane protein-2: A case report

BACKGROUND Danon disease(DD),in which mutations in the X-linked lysosome-associated membrane protein-2(LAMP-2)gene result in hypertrophic cardiomyopathy,is a rare disease,reported primarily in small samples or cases.However,with the development of cardiac magnetic resonance imaging and genetic technology in recent years,the number of reports has increased.CASE SUMMARY We report a case of DD in an adolescent male patient,confirmed by genetic testing.The patient was admitted to our hospital with complaints of a three-year history of chest tightness and shortness of breath.His preliminary clinical diagnosis is hypertrophic cardiomyopathy.Our report includes the patient’s clinical course from hospital admission to death,step-by-step diagnosis,treatment course,and noninvasive imaging features.We highlight how a noninvasive diagnostic approach,based solely on clinical and imaging“red flags”for DD,can be used to achieve a diagnosis of DD with a high degree of confidence.CONCLUSION DD is a very dangerous cardiomyopathy,and it is necessary to achieve early diagnosis and treatment.

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

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.

A misdiagnosed transthyretin in an elderly with myocardial hypertrophy and atrioventricular block:a case report

Cardiac amyloidosis(CA)is an infiltrative and restrictive cardiomyopathy that leads to heart failure(HF),decreased quality of life,and death.[1–3]There are two main subtypes of the disease,transthyretin cardiac amyloidosis(ATTR-CA)and immunoglobulin light chain CA.The former was further subdivided into wild-type(ATTRwt-CA)and or variant(ATTRv-CA)according to the presence or absence of a mutations in the transthyretin(TTR)gene.