Cardiomyopathy in a nutshell: from etiopathogenesis and diagnosis to treatment

Cardiomyopathy is defined as a heterogeneous group of myocardial disorders wherein the cardiac muscle is functionally and structurally abnormal, without the presence of any congenital heart disease (CHD), coronary artery disease (CAD), valvular disease, and hypertension sufficient enough to lead to abnormality of the myocardium. Based on etiology, cardiomyopathies are classified into primary (acquired, mixed, or genetic) and secondary, which results in different phenotypes like hypertrophic, restrictive, dilated, etc. patterns. Hypertrophic cardiomyopathy is the most common type of primary cardiomyopathy among all cardiomyopathies usually presenting as exertional dyspnea, heart failure, atypical chest pain, syncope, and sudden cardiac death (SCD). Dilated cardiomyopathy is genetic or acquired, causing classic symptoms of heart failure with reduced ejection fraction. Restrictive cardiomyopathy is mostly associated with systemic disease and is rare. Diagnosis of cardiomyopathy includes a detailed evaluation of history, and physical examination followed by a workup including blood test, genetic testing, electrocardiography, and echocardiography testing. Treatment includes initially staging the therapy for heart failure, restriction of physical activity, evaluation of the need for implantable cardioverter-defibrillators, optimization of drugs, and consideration of heart transplantation in refractory cases. Genetic testing of families is now available as an emerging modality for early diagnosis and prevention in relatives of diagnosed cases. This review evaluates the causes, early diagnosis, and early treatment and prevention modalities for cardiomyopathies to reduce morbidity and mortality caused by it.

Dilated cardiomyopathy in pregnancy: a case report

The prevalence of dilated cardiomyopathy (DCM) is 1:2500 among the women of childbearing age. DCM often develops during second trimester or may be present prior pregnancy. We report a successful pregnancy outcome in a young pre-eclamptic woman with DCM who presented with cardiac failure. Multidisciplinary team involved. Anti-failure drugs, antihypertensives and thromboprophylaxis administered. Caesarean section was performed at 30 weeks because of spontaneous onset of labour to avoid undue stress on failing heart. Intra operative and post-operative recovery was uneventful. Follow up showed improvement in ejection fraction. Counselling prior pregnancy, stable cardiac status, regular monitoring, personalised care ensures safe maternal and foetal outcome.

Signaling Pathways Related to Dilated Cardiomyopathy and Their Regulation by Traditional Chinese Medicine： A Review / 中国实验方剂学杂志

Dilated cardiomyopathy （DCM） is characterized by the main pathological changes of global cardiac enlargement， especially left ventricular enlargement. Clinical manifestations include decreased heart function， arrhythmia， thromboembolism， and even sudden death. It is one of the refractory cardiovascular diseases. Conservative drug treatment is still the main approach in clinical practice， but due to its unavoidable side effects such as low blood pressure， it is often difficult to achieve a satisfactory prognosis. The combination of traditional Chinese medicine and Western medicine can effectively improve side effects and enhance efficacy. The research has found that nuclear transcription factors-κB （NF-κB）， adenylate activated protein kinase （AMPK）/mammalian rapamycin target protein （mTOR）， transforming growth factor-β （TGF-β）/Smads， Toll like receptors （TLR） 4/c-Jun amino terminal kinase （JNK）， mitogen activated protein kinase （MAPK）， phosphatidylinositol 3-kinase （PI3K）/protein kinase （Akt）， and other signaling pathways play a crucial regulatory role in the occurrence and development of DCM. Traditional Chinese medicine can improve myocardial fibrosis， reverse ventricular remodeling， alleviate oxidative stress， and achieve anti-inflammatory and other effects by regulating the above signaling pathways， thus improving DCM. Due to its multi-target and multi-mechanism characteristics， it has the advantages of high safety and good tolerance and has become an important part of current clinical treatment.

Study on the therapeutic effect of Xuefu Zhuyu Capsule combined with sacubitril valsartan on patients with dilated cardiomyopathy / 重庆医学

Objective To study the therapeutic effect of Xuefu Zhuyu Capsule combined with sacubitril valsartan on dilated cardiomyopathy.Methods A total of 70 patients with dilated cardiomyopathy who were hospitalized in the Hospital from January to December 2020 were selected as the research objects and random-ly divided into control group and experimental group,with 35 cases in each group.The control group was only treated with sacubitril valsartan,and the experimental group was treated with Xuefu Zhuyu Capsule combined with sacubitril valsartan.According to the patient's blood pressure and renal function,sacubitril valsartan was titrated from a small dose to the maximum dose.Xuefu Zhuyu Capsule was uesd 2.4 g each time,twice a day,and the treatment time was three months.The symptoms of heart failure,glycosylated hemoglobin(HbA1c),low density lipoprotein(LDL),total cholesterol(TC),triglyceride(TG),ALT,AST,N-terminal pro-brain natriuretic peptide(NT-proBNP),left ventricular end-systolic diameter(LVEDd),left ventricular end-dias-tolic diameter(LVEDs),ejection fraction(EF)and the incidence of arrhythmia were observed in the two groups after treatment.Results After treatment,the level of NT-proBNP in the experimental group was sig-nificantly lower than that in the control group(P＜0.05).There was no significant difference in LDL,TC,TG,LVEDd,LVEDs and EF between the two groups(P＞0.05).The incidence of atrial tachycardia and ven-tricular premature beat in the experimental group was higher than that in the control group(P＜0.05).Con-clusion Xuefu Zhuyu Capsule combined with sacubitril valsartan can significantly improve the symptoms of patients with heart failure and reduce the level of NT-proBNP,but it may increase the proportion of patients with atrial tachycardia and ventricular premature beats.

The relationship between serum c-kit mRNA level and myocardial fibrosis,cardiac function and prognosis in patients with dilated cardiomyopathy complicated with heart failure / 国际检验医学杂志

Objective To investigate the relationship between serum stem cell factor receptor(c-kit)and myocardial fibrosis,cardiac function and prognosis in patients with dilated cardiomyopathy(DCM)complicat-ed with heart failure.Methods A total of 77 patients with DCM complicated with heart failure who were trea-ted in 3201 Hospital from May 2020 to June 2022 were enrolled in the study as study group,and 70 DCM pa-tients without heart failure were enrolled as the control group.The levels of serum c-kit mRNA and three my-ocardial fibrosis markers[α-smooth muscle actin(α-SMA),collagen type Ⅰ and collagen type Ⅲ]were detec-ted in the two groups.Cardiac function parameters were obtained by echocardiography.The relationship be-tween serum c-kit mRNA level and myocardial fibrosis and cardiac function was analyzed.According to the oc-currence of major adverse cardiovascular events(MACE),the patients were divided into poor prognosis group and good prognosis group.Multivariate Logistic regression analysis was used to analyze the factors affecting the prognosis of DCM patients complicated with heart failure.Receiver operating characteristic(ROC)curve was used to analyze the efficacy of serum c-kit mRNA level in predicting the prognosis of DCM patients.Results The serum c-kit mRNA level in the study group was lower than that in the control group(P＜0.05).The levels of three myocardial fibrosis indicators in the study group were higher than those in the con-trol group(P＜0.05).The left ventricular ejection fraction(LVEF)in the study group was lower than that in the control group(P＜0.05),and the left ventricular end-diastolic volume(LVEDV)and left ventricular end-systolic volume(LVESV)in the study group were higher than those in the control group(P＜0.05).Pearson correlation analysis showed that serum c-kit mRNA level was positively correlated with LVEF(r=0.677,P＜0.05),while negatively correlated with α-SMA,collagen type Ⅰ,collagen type Ⅲ,LVEDV and LVESV(r=-0.725,-0.748,-0.744,-0.745,-0.662,P＜0.05).Multivariate Logistic regression analysis showed that serum c-kit mRNA level is an independent factor for the prognosis of DCM patients with heart failure.ROC curve analysis showed that serum c-kit mRNA level had a sensitivity of 82.80％,a specificity of 81.80％,and an area under the curve of 0.829(95％CI:0.745-0.912,P＜0.001)for evaluating the progno-sis of DCM patients complicated with heart failure.Conclusion The serum c-kit mRNA level is significantly decreased in patients with DCM complicated with heart failure,and the serum c-kit mRNA level is correlated with myocardial fibrosis and cardiac function.The detection of serum c-kit mRNA level has a high efficacy in evaluating the prognosis of patients with DCM complicated with heart failure.

Clinical efficacy of vericiguat in the treatment of acute decompensated heart failure and its effects on LVEF of patients with different LVDd / 中国药房

OBJECTIVE To observe the efficacy of vericiguat in the treatment of acute decompensated heart failure （HF） and its effect on left ventricular ejection fraction （LVEF） in patients with different left ventricular end-diastolic internal diameters （LVDd）. METHODS A total of 52 patients with acute decompensated HF who were hospitalized in Anqing Municipal Hospital from September 2022 to May 2023 and were stabilized by intravenous injection of diuretics or vasodilators and then given vericiguat orally were selected. Clinical baseline data were collected， and blood creatinine （Scr）， estimated glomerular filtration rate （eGFR）， N-terminal pro-B-type natriuretic peptide （NT-proBNP）， echocardiographic indexes（LVEF， LVDd）， and Kansas City cardiomyopathy questionnaire （KCCQ） scores and so on were recorded and analyzed at 1 and 6 months after treatment. The patients were divided into two subgroups according to whether they had dilated cardiomyopathy or not， the baseline data were compared between the two subgroups， and the changes in the above follow-up indexes for the entire population and two subgroups of patients were analyzed by using mixed effect model. RESULTS Patients in the dilated cardiomyopathy group were significantly younger than those in the nondilated cardiomyopathy group， while the former had a lower proportion of patients with combined coronary artery disease， longer LVDd， lower LVEF levels， and more HF patients with reduced ejection fraction （P＜0.05）. Compared with baseline values， KCCQ scores were significantly higher in patients in the whole population， non-dilated cardiomyopathy group and dilated cardiomyopathy group at 1 month after treatment （P＜0.001）. The difference between the whole population in terms of elevated KCCQ scores and decreased lg（NT-proBNP） levels was statistically significant at 6 months after treatment （P＜0.05）； the levels of lg（NT-proBNP）of two subgroups were significantly decreased and KCCQ scores were significantly increased （P＜0.05）， but the difference was not statistically significant （P＞0.05）； LVEF levels of the patients E-mail：guangf4-508@163.com in both subgroups were significantly elevated （P＜0.05） and the difference between the subgroups amounted to 7.52% （P＝0.030）. Mixed effect model result showed that whether patients had coronary artery disease and different baseline levels of LVDd were likely to affect follow-up LVEF levels， with coronary artery disease contributing to follow-up LVEF elevation （P＝0.043）， but the coronary artery disease subgroup × time interaction was not significant （P＞0.05）；compared with patients with baseline LVDd ≥62 mm， patients with baseline LVDd≤61 mm had a faster LVEF improvement （P＜0.05）. CONCLUSIONS Vericiguat is able to improve cardiac function and quality of life in patients with acute decompensated HF without negatively affecting their renal function. The drug is able to significantly improve LVEF levels in patients with baseline LVDd ≤61 mm.

Clinical efficacy of vericiguat in the treatment of acute decompensated heart failure and its effects on LVEF of patients with different LVDd / 中国药房

OBJECTIVE To observe the efficacy of vericiguat in the treatment of acute decompensated heart failure （HF） and its effect on left ventricular ejection fraction （LVEF） in patients with different left ventricular end-diastolic internal diameters （LVDd）. METHODS A total of 52 patients with acute decompensated HF who were hospitalized in Anqing Municipal Hospital from September 2022 to May 2023 and were stabilized by intravenous injection of diuretics or vasodilators and then given vericiguat orally were selected. Clinical baseline data were collected， and blood creatinine （Scr）， estimated glomerular filtration rate （eGFR）， N-terminal pro-B-type natriuretic peptide （NT-proBNP）， echocardiographic indexes（LVEF， LVDd）， and Kansas City cardiomyopathy questionnaire （KCCQ） scores and so on were recorded and analyzed at 1 and 6 months after treatment. The patients were divided into two subgroups according to whether they had dilated cardiomyopathy or not， the baseline data were compared between the two subgroups， and the changes in the above follow-up indexes for the entire population and two subgroups of patients were analyzed by using mixed effect model. RESULTS Patients in the dilated cardiomyopathy group were significantly younger than those in the nondilated cardiomyopathy group， while the former had a lower proportion of patients with combined coronary artery disease， longer LVDd， lower LVEF levels， and more HF patients with reduced ejection fraction （P＜0.05）. Compared with baseline values， KCCQ scores were significantly higher in patients in the whole population， non-dilated cardiomyopathy group and dilated cardiomyopathy group at 1 month after treatment （P＜0.001）. The difference between the whole population in terms of elevated KCCQ scores and decreased lg（NT-proBNP） levels was statistically significant at 6 months after treatment （P＜0.05）； the levels of lg（NT-proBNP）of two subgroups were significantly decreased and KCCQ scores were significantly increased （P＜0.05）， but the difference was not statistically significant （P＞0.05）； LVEF levels of the patients E-mail：guangf4-508@163.com in both subgroups were significantly elevated （P＜0.05） and the difference between the subgroups amounted to 7.52% （P＝0.030）. Mixed effect model result showed that whether patients had coronary artery disease and different baseline levels of LVDd were likely to affect follow-up LVEF levels， with coronary artery disease contributing to follow-up LVEF elevation （P＝0.043）， but the coronary artery disease subgroup × time interaction was not significant （P＞0.05）；compared with patients with baseline LVDd ≥62 mm， patients with baseline LVDd≤61 mm had a faster LVEF improvement （P＜0.05）. CONCLUSIONS Vericiguat is able to improve cardiac function and quality of life in patients with acute decompensated HF without negatively affecting their renal function. The drug is able to significantly improve LVEF levels in patients with baseline LVDd ≤61 mm.

Research on the risk factors and predictive model for intracardiac thrombosis in patients with dilated cardiomyopathy / 安徽医科大学学报

Objective @#To explore the risk factors for intracardiac thrombosis in dilated cardiomyopathy (DCM) pa- tients and to construct , validate , and evaluate a nomogram prediction model based on these factors .@*Methods @#88 patients diagnosed with DCM and complicated with intracardiac thrombus , and 544 patients without intracardiac thrombus were included . The participants were randomly divided into training and validation sets at a ratio of 7 ∶ 3 . U sing both univariate and multivariate Logistic regression analyses , independent risk factors for intracardiac thrombosis in DCM patients were identified . A nomogram prediction model was constructed using R software . The model ’s validity and performance were assessed using the receiver operating characteristic (ROC) curve , the Hos- mer-Lemeshow goodness-of-fit test , calibration curve , and decision curve . @*Results @#The binary Logistic regression analysis showed that age , atrial fibrillation , left ventricular end-diastolic diameter ( LVEDD) , brain natriuretic peptide ( BNP) , and β-blockers were independently associated with intracardiac thrombosis in DCM patients . Based on these five factors , a nomogram was constructed and validated . The area under the ROC curve for the training set was 0. 823 (95% CI: 0. 760 ~ 0. 887) and 0 . 803 (95% CI: 0 . 705 ~ 0 . 901) for the validation set , in- dicating a good discriminative ability. The Hosmer-Lemeshow test results for the calibration curve were ( χ2 = 6. 679 , P = 0. 572) for the training set and ( χ2 = 2 . 588 , P = 0. 958) for the validation set , indicating a good fit between predicted and ob served outcomes . The decision curve showed a high net clinical benefit in the threshold range of 0. 05 ~ 0. 92 . @*Conclusion @#Based on age , atrial fibrillation , LVEDD , BNP , and β-blockers , the nomo- gram prediction model exhibits good discriminative and calibration abilities , and high clinical benefit. It can effec- tively guide clinicians in early intervention of risk factors , reducing the risk of intracardiac thrombosis in DCM pa- tients .

Survival Analysis in Adult Heart Transplantation: Experience from a Brazilian Single Center

ABSTRACT Introduction: Heart transplantation is the gold standard for advanced heart failure treatment. This study examines the survival rates and risk factors for early mortality in adult heart transplant recipients at a Brazilian center. Methods: This retrospective cohort study involved 255 adult heart transplant patients from a single center in Brazil. Data were collected from medical records and databases including three defined periods (2012-2015, 2016-2019, and 2020-2022). Statistical analysis employed Kaplan-Meier survival curves, Cox proportional hazards analysis for 30-day mortality risk factors, and Log-rank tests. Results: The recipients were mostly male (74.9%), and the mean age was 46.6 years. Main causes of heart failure were idiopathic dilated cardiomyopathy (33.9%), Chagas cardiomyopathy (18%), and ischemic cardiomyopathy (14.3%). The study revealed an overall survival of 68.1% at one year, 58% at five years, and 40.8% at 10 years after heart transplantation. Survivalimproved significantly over time, combining the most recent periods (2016 to 2022) it was 73.2% in the first year and 63% in five years. The main risk factors for 30-day mortality were longer time on cardiopulmonary bypass, the initial period of transplants (2012 to 2015), older age of the donor, and nutritional status of the donor (overweight or obese). The main causes of death within 30 days post-transplant were infection and primary graft dysfunction. Conclusion: The survival analysis by period demonstrated that the increased surgical volume, coupled with the team's experience and modifications to the immunosuppression protocol, contributed to the improved early and mid-term outcomes.

A study of clinical profile and outcome in children with dilated cardiomyopathy

Background: Objective was to analyse the clinical profile of children diagnosed with Dilated cardiomyopathy and to correlate the same with the severity and functional status on 2-D echocardiography.Methods: It was a prospective, retrospective and observational study. A total of 150 subjects were selected from patients visiting the cardiology OPD, admitted in medical wards, paediatric Intensive Care Unit of a tertiary care referral centre. The demographic profile, symptomatology, heart functions and compliance were recorded. The cases were evaluated by detailed history, general and cardiovascular examination and laboratory investigations along with 2D echocardiography.Results: The demographic profile is significant as the age at diagnosis predicts the risk of mortality with older children having the worst outcome. A significant association observed between decreasing ejection fraction and increasing mortality with a p value (p=0.02). Signs of pedal edema and hepatomegaly are associated with unfavourable outcome with a significant p value of (p=0.03). The overall prognosis of children with Dilated cardiomyopathy steadily improved with timely use of appropriate medications. Only 1 patient in this study had undergone orthotopic heart transplant at the age of 15 years (case of chemotherapy induced Dilated cardiomyopathy).Conclusions: Dilated cardiomyopathy is a difficult entity to treat in children as most cases are idiopathic and the therapy remains mostly supportive. Hypovitaminosis-D and hypocalcemia are common treatable causes of dilated cardiomyopathy in children and must be ruled out in all cases of dilated cardiomyopathy. Patients refractory to medical therapy should be considered for ventricular assist devices and orthotopic heart transplantation.

Respondent or non-respondent comparison post cardiac resynchronisation therapy implantation in patients with dilated cardiomyopathy

Background: Dilated cardiomyopathy can be treated using cardiac resynchronisation therapy (CRT) effectively. In our study, we compared the clinical and biochemical profile of responders and non-responders to CRT device (CRTD) implantation suffering from DCM. Methods: A cross-sectional observational study was performed in 47 patients with dilated cardiomyopathy for CRTD implantation for a period of 18 months. The tools used for the study include electrocardiography 12 lead, echocardiography: 2D, M mode, Doppler, strain echo, Holter monitoring, coronary angiography and CRTD implantation. Statistical analysis was performed using Epi Info (TM) 7.2.2.2. Results: The proportion of responders (68.1%) was significantly higher than non-responder (31.9%). Almost 60% of patients in non-responder group had smoking as a risk factor. Around 60% were suffering from hypertension and 33% from T2DM in non-respondent group. Parameters of dyssynchrony has significantly improved in responder group than in non-responder group. LVEDV, LVESV has shown an increase and EF has decreased considerably in DCM patients. Many patients in non-responder category have shown mitral and tricuspid regurgitation. Strain echocardiography parameters-GLS, GRS and GCS were significantly decreased. Post CRTD echocardiographic parameter has improved considerably and LVESV was reduced in more than 15% of responders. Conclusions: The CRTD implantation improves patients’ clinical and Echocardiographic data which can help in better patient management, improving quality of life and decreased healthcare cost. By this study we can improve patients’ selection and predict accordingly for CRT responders and non-responders and can take necessary measures for better patient’s management.

Trasplante cardíaco por hemocromatosis. Caso pediátrico / Cardiac transplantation due to hemochromatosis. A pediatric case report

La hemocromatosis es una enfermedad caracterizada por el excesivo depósito de hierro en múltiples órganos, entre ellos hígado, páncreas, piel y corazón. La infiltración de este último es un importante factor en morbilidad y mortalidad. Presentamos un caso de un paciente pediátrico con insuficiencia cardíaca terminal que ameritó trasplante cardíaco, que resultó sin complicaciones. Posterior a la cirugía, mostró mejoría bioquímica y clínica, lo que influyó positivamente en su calidad de vida y prolongó su supervivencia.

Hemochromatosis is a disease characterized by excess iron stores in multiple organs, including the liver, pancreas, skin, and heart. The infiltration of the heart is an important factor in morbidity and mortality. Here we describe the case of a pediatric patient with end-stage heart failure who required a heart transplantation, with no complications. After the surgery, she showed biochemical and clinical improvement, with a positive impact on her quality of life and a prolonged survival.

Dilated Cardiomyopathy with Biventricular Thrombi: A Severe Manifestation of Behcet Disease

Behcet’s disease is a systemic vasculitis of the vessels for all calibers, touching arterial and venous territories. The causes of disease are unknow. BD reaches young age subjects from 10 to 45 years and affects both men and women. BD is ubiquitous but more frequent in patients from Mediterranean basin, the middle East and Asia. The diagnosis of BD is essentially clinical. The diagnostic criteria make it possible to carry the diagnosis with good sensitivity and specifity. BD evolves by recurrent inflammatory attack. BD can affect all of the organs; cardiacs manifestations are dominated by intracardiac thrombosis, the damage of three tunics, coronaryarteritis with or without myocardial infarction, coronaries aneurysms and endomyocardial fibrosis. The vascular manifestations are dominated by arterial or venous thrombosis. The presence of dilated cardiomyopathy with reduced left ventricular ejection fraction is rare. It can be explained by ischemic or inflammatory origin by cytokines. We report a case of young woman aged of 33 years to the history of 3 episodes of bipolar aphtae which presented dilated cardiomyopathy with reduced left ventricular function, biventricular thrombosis, bilateral distal pulmonary embolism with pulmonary infarction.

Evaluación de resultados en una serie de pacientes con trasplante cardiaco: experiencia en el Instituto Nacional de Cardiología

Exponemos la experiencia del Instituto Nacional de Cardiología de una serie de casos de pacientes sometidos a trasplante cardiaco entre mayo de 2016 y junio 2022. Se realizaron 14 trasplantes, 13 fueron del sexo masculino. La edad osciló entre 19 y 62 años. Las etiologías fueron cardiopatías de tipo idiopática en 57% y valvular en 21%. El 50% se trasplantó en INTERMACS 4 (Interagency Registry for Mechanically Assisted Circulatory Support), 21% INTERMACS 3 y solo 28% en INTERMACS 2. Tres pacientes se trasplantaron con asistencia circulatoria tipo membrana circulación extracorpórea. Las complicaciones más frecuentes fueron las infecciosas. La mortalidad hospitalaria fue 35,7%. Hubo un fallecido en el seguimiento tras 5 años de trasplante.

We present the experience of the National Institute of Cardiology of a series of cases of patients undergoing heart transplantation between May 2016 and June 2022. Fourteen transplants were performed, 13 of the patients were male. The age ranged between 19 and 62 years. The etiologies were idiopathic heart disease in 57% and valvular heart disease in 21%. Fifty percent was transplanted in INTERMACS 4 (Interagency Registry for Mechanically Assisted Circulatory Support), 21% in INTERMACS 3 and only 28% in INTERMACS 2. Three patients were transplanted with membrane type extracorporeal circulation circulatory support. The most frequent complications were infectious. Hospital mortality was 35.7%. There was one patient who died during follow-up after 5 years of transplantation.

Formononetin regulates dilated cardiomyopathy-mediated heart failure in rats via HSP90/AKT cardiomyocyte apoptosis and mechanism / 西安交通大学学报(医学版)

【Objective】 To investigate the effects of formononetin (FMN) on cardiomyocyte apoptosis and HSP90/AKT in rats with dilated cardiomyopathy-mediated heart failure. 【Methods】 Echocardiography, ELISA, histological staining, and TUNEL staining were used to observe the protective effect of different doses of FMN on dilated cardiomyopathy-mediated heart failure in rats and the apoptosis of cardiomyocytes. The potential targets of formononetin on dilated cardiomyopathy-mediated heart failure were obtained from TCMSP, DisGeNet, GeneCards, and other databases, the key targets were obtained according to the protein-protein interaction (PPI) network, and the key targets were verified by molecular docking. Western blotting was used to further verify the regulatory role of key targets in the treatment of dilated cardiomyopathy-mediated heart failure with formononetin. 【Results】 Formononetin could reduce the levels of LVIDS, LVIDD, NT-pro BNP, cTn-T, CK, CK-MB, and LDH in rats with dilated cardiomyopathy-mediated heart failure, increase the levels of EF and FS, and reduce the apoptosis of cardiomyocytes. FMN had a strong binding effect on 10 key targets (AKT1, HSP90AA1, CASP3, MAPK1, MMP9, SRC, ALB, HRAS, IGF1, and EGFR) screened by network pharmacology, with HSP90AA1 and AKT1 having the strongest binding effect. Formononetin decreased the expression of HSP90, AKT and downstream CASP3 protein, but increased the expression of p-AKT in myocardial tissue. 【Conclusion】 Formononetin may inhibit the expression of HSP90, promote phosphorylation of AKT to p-AKT, and inhibit the expression of CASP3, thereby reducing the apoptosis of cardiomyocytes and improving myocardial tissue damage, so as to achieve the purpose of treating dilated cardiomyopathy-mediated heart failure.

Clinical and genetic characteristics of children with primary dilated cardiomyopathy / 中国当代儿科杂志

OBJECTIVES@#To study the genetic characteristics, clinical characteristics, and prognosis of children with primary dilated cardiomyopathy (DCM).@*METHODS@#A retrospective analysis was performed on the medical data of 44 children who were diagnosed with DCM in Hebei Children's Hospital from July 2018 to February 2023. According to the genetic testing results, they were divided into two groups: gene mutation-positive group (n=17) and gene mutation-negative group (n=27). The two groups were compared in terms of clinical data at initial diagnosis and follow-up data.@*RESULTS@#Among the 44 children with DCM, there were 21 boys (48%) and 23 girls (52%). Respiratory symptoms including cough and shortness of breath were the most common symptom at initial diagnosis (34%, 15/44). The detection rate of gene mutations was 39% (17/44). There were no significant differences between the two groups in clinical characteristics, proportion of children with cardiac function grade Ⅲ or Ⅳ, brain natriuretic peptide levels, left ventricular ejection fraction, and left ventricular fractional shortening at initial diagnosis (P>0.05). The median follow-up time was 23 months, and 9 children (20%) died, including 8 children from the gene mutation-positive group, among whom 3 had TTN gene mutation, 2 had LMNA gene mutation, 2 had TAZ gene mutation, and 1 had ATAD3A gene mutation. The gene mutation-positive group had a significantly higher mortality rate than the gene mutation-negative group (P<0.05).@*CONCLUSIONS@#There is no correlation between the severity of DCM at initial diagnosis and gene mutations in children. However, children with gene mutations may have a poorer prognosis.

Recent studies on dilated cardiomyopathy caused by TTN mutations in children / 中国当代儿科杂志

The mutations of TTN gene that encodes titin are the most common mutation type among the genetic causes of dilated cardiomyopathy (DCM). This article reviews the worldwide studies on potential molecular pathogenesis (transcription, post-translational modification, etc.), clinical phenotypes, and gene therapies of pediatric DCM caused by TTN mutations, with the hope of providing a reference for the precision treatment of pediatric DCM caused by TTN mutations.

Study on Myocardial Lymphangiogenesis Disorder in Dilated Cardiomyopathy Mice Induced by Doxorubicin and the Protective Mechanism of Kuoxin Decoction / 世界科学技术-中医药现代化

Objective To observe the dynamic changes of cardiac lymphangiogenesis in Doxorubicin(DOX)-induced dilated cardiomyopathy(DCM)model mice,and to study the the protective mechanism of Kuoxin Decoction.Methods The DCM mouse model was established by intraperitoneal injection of DOX,and the dynamic observation was performed every week.On this basis,60 C57BL/6 mice were randomly divided into 6 groups(n=10):control group,Model group,L-KXD,M-KXD and H-KXD groups and Captopril group.After successful modeling,the KXD and the positive control drug Captopril were administered continuously for 28 days.Echocardiography was used to detect cardiac function in mice,HE staining and Masson staining were used to observe pathological and morphological changes of the heart,Whole-mount immunofluorescent staining was used to detect the expression of LYVE-1 and Podoplanin in epicardial lymphatic vessels,Western blot was used to detect the expression of VEGFR-3 protein,and qPCR was used to detect the expression of VEGFR-3 mRNA.Results DCM mice induced by DOX showed significant cardiac function decline from the third week(DOX:15 mg·kg-1,P<0.05),and significant ventricular remodeling at the fifth week(DOX:15 mg·kg-1,P<0.01);The lymphatic vessel area of the mouse heart decreased significantly from the fourth week(DOX:20 mg·kg-1,P<0.0001),and the expression of VEGFR-3 decreased significantly from the third week(DOX:15 mg·kg-1,P<0.01).Conclusion KXD can improve ventricular remodeling and cardiac function in DOX-induced DCM mice,promote cardiac lymphangiogenesis,and upregulate the expression of VEGFR-3 at protein and mRNA levels,with a better effect than captopril.DOX-induced cardiac lymphangiogenesis in DCM mice leads to severe myocardial fibrosis and weakened cardiac function,which gradually worsens with the accumulation of modeling time and dose.KXD can promote cardiac lymphangiogenesis and improve cardiac function in DOX-induced DCM mice.The mechanism may be related to the up-regulation of VEGFR-3 expression.

Value of four-dimensional automatic right ventricular quantitative analysis in evaluating right ventricular function in patients with dilated cardiomyopathy / 中华超声影像学杂志

Objective:To evaluate the right ventricular function in patients with dilated cardiomyopathy (DCM) by four-dimensional automatic right ventricular quantitative analysis (4D Auto RVQ), and compare with the right ventricular ejection fraction measured by cardiac magnetic resonance (CMR-RVEF), and to explore the clinical application value of 4D Auto RVQ technique in evaluating the right ventricular function of patients with DCM.Methods:A prospective study was conducted to select 52 patients with DCM who were treated in Fuwai Central China Cardiovascular Hospital of Zhengzhou University from March to October 2022 as DCM group, and 52 healthy volunteers were selected as the control group during the same period. The four-dimensional right ventricular ejection fraction (4D-RVEF), right ventricular stroke volume index (RVSVI), right ventricular end-diastolic volume index (RVEDVI), right ventricular end-systolic volume index (RVESVI), four-dimensional right ventricular basal diameter (4D-RVDd-base), four-dimensional right ventricular middle diameter (4D-RVDd-mid), four-dimensional right ventricular long axis diameter (4D-RVLd), four-dimensional tricuspid annular plane systolic excursion (4D-TAPSE) and four-dimensional right ventricular fractional area change (4D-RVFAC) were obtained by 4D Auto RVQ technique. The differences of the above parameters between DCM group and control group were compared.Pearson linear correlation analysis was used to evaluate the correlation between echocardiographic parameters and CMR-RVEF. The ROC curve was used to find the most sensitive parameters for evaluating right ventricular function, and the area under the ROC curve ( AUC ) was calculated and compared.Results:Compared with the control group, RVEDVI, RVESVI, 4D-RVDd-base and 4D-RVDd-mid in the DCM group were increased, and the absolute values of 4D-RVEF, 4D-TAPSE, 4D-RVFAC, right ventricular global longitudinal strain(RVGLS) and right ventricular free wall longitudinal strain(RVFWLS) were decreased (all P<0.05). Correlation analysis showed that 4D-RVEF was positively correlated with CMR-RVEF ( r=0.711, P<0.05). ROC curve analysis showed that 4D-RVEF was superior to other parameters in evaluating right ventricular function in DCM patients (AUC: 0.916). Conclusions:4D Auto RVQ technique can quantitatively evaluate right ventricular function in DCM patients. 4D-RVEF has a significant correlation with CMR-RVEF, and 4D-RVEF has the best efficacy in evaluating right ventricular function in DCM patients.