With the successful application and breakthrough of deep learning technology in image segmentation,there has been continuous development in the field of seismic facies interpretation using convolutional neural network...With the successful application and breakthrough of deep learning technology in image segmentation,there has been continuous development in the field of seismic facies interpretation using convolutional neural networks.These intelligent and automated methods significantly reduce manual labor,particularly in the laborious task of manually labeling seismic facies.However,the extensive demand for training data imposes limitations on their wider application.To overcome this challenge,we adopt the UNet architecture as the foundational network structure for seismic facies classification,which has demonstrated effective segmentation results even with small-sample training data.Additionally,we integrate spatial pyramid pooling and dilated convolution modules into the network architecture to enhance the perception of spatial information across a broader range.The seismic facies classification test on the public data from the F3 block verifies the superior performance of our proposed improved network structure in delineating seismic facies boundaries.Comparative analysis against the traditional UNet model reveals that our method achieves more accurate predictive classification results,as evidenced by various evaluation metrics for image segmentation.Obviously,the classification accuracy reaches an impressive 96%.Furthermore,the results of seismic facies classification in the seismic slice dimension provide further confirmation of the superior performance of our proposed method,which accurately defines the range of different seismic facies.This approach holds significant potential for analyzing geological patterns and extracting valuable depositional information.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
Objective: To screen and analyze the differentially expressed genes between dilated cardiomyopathy (DCM) and chronic heart failure (CHF) based on bioinformatics methods. Methods: The Gene Expression Omnibus (GEO) data...Objective: To screen and analyze the differentially expressed genes between dilated cardiomyopathy (DCM) and chronic heart failure (CHF) based on bioinformatics methods. Methods: The Gene Expression Omnibus (GEO) database was used for data retrieval, and the chip data GSE3585 was downloaded, which was the original data of DCM and normal control group. At the same time, the chip data GSE76701 was downloaded, which was the original data of CHF and control group. Differentially expressed mRNAs (DEmRNAs) were screened by R language limma package, the data were standardized, and the common differentially expressed genes were screened. GO function and KEGG pathway enrichment analysis were performed on the common differentially expressed genes. String11.0 online tool was used for data analysis to obtain differentially expressed genes, and the results were imported into Cytoscape 3.9.1 software. The results were imported into Cytoscape 3.9.1 software, and the common expression gene module was obtained by MOCDE algorithm. Nine Hub genes were obtained by 10 algorithms such as MCC. Results: A total of 248 differentially expressed genes were screened. GO analysis showed that differentially expressed genes were mainly concentrated in 9 different physiological and pathological processes. KEGG analysis showed that the main signaling pathways involved in differentially expressed genes were 2, and 9 key differentially expressed genes were predicted: NPPB, NPPA, MYH6, FRZB, ASPN, SFRP4, RPS4Y1, DDX3Y. Conclusion: This study preliminarily explored the molecular mechanism of DCM and CHF, and obtained the common differentially expressed genes of the two diseases. Further experimental studies are needed to verify the correlation between gene expression and clinicopathological features. Provide new ideas for clinical drug treatment research.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
Backgrounds Clinical trials have demonstrated that cardiac resynchronization therapy (CRT) is effective in patients with "non-is- chemic cardiomyopathy". However, patients with dilated-phase hypertrophic cardiomyo...Backgrounds Clinical trials have demonstrated that cardiac resynchronization therapy (CRT) is effective in patients with "non-is- chemic cardiomyopathy". However, patients with dilated-phase hypertrophic cardiomyopathy (DHCM) have been generally excluded from such trials. We aimed to compare the clinical outcome of CRT in patients with DHCM, idiopathic dilated cardiomyopathy (IDCM), or ischemic cardiomyopathy (ICM). Methods A total of 312 consecutive patients (DHCM: n = 16; IDCM: n = 231; ICM: n = 65) undergoing CRT in Fuwai hospital were studied respectively. Response to CRT was defmed as reduction in left ventricular end-systolic volume (LVESV) _〉 15% at 6-month follow-up. Results Compared with DHCM, IDCM was associated with a lower total mortality (HR: 0.35, 95% CI: 0.13-0.90), cardiac mortality (HR: 0.29; 95% CI: 0.11-0.77), and total mortality or heart failure (HF) hospitalizations (HR: 0.34, 95% CI: 0.17-0.69), independent of known confounders. Compared with DHCM, the total mortality, cardiac mortality and total mortality or HF hospitalizations favored ICM but were not statistically significant (HR: 0.59, 95% CI: 0.22-1.61; HR: 0.59, 95% CI: 0.21-1.63; HR: 0.54, 95% CI: 0.26-1.15; respectively). Response rate to CRT was lower in the DHCM group than the other two groups although the differences didn't reach statistical significance. Conclusions Compared with IDCM, DHCM was associated with a worse outcome after CRT. The clinical outcome of DHCM patients receiving CRT was similar to or even worse than that of ICM patients. These indicate that DHCM behaves very differently after CRT.展开更多
Antibodies against &-adrenoceptor can be detected in serum of patients with dilated cardiomyopathy (DCM), which have 5-agonist-like activity, and induce a positive chronotropic effect on cardiac myocytes by its pe...Antibodies against &-adrenoceptor can be detected in serum of patients with dilated cardiomyopathy (DCM), which have 5-agonist-like activity, and induce a positive chronotropic effect on cardiac myocytes by its persistence at full strength. Effects of the antibodies against Padrenoceptor from sera of patients with DCM on myocardial cytotoxicity and cytoplasmic free Ca2+-concentration (LCa2+ji) were observed in the cultured single layer SD rat ventricular cells by using the cytotoxicity assay and fluorescent Ca2+- indicat0r fura-2/AM. The positive sera of the anti-&adrenoceptor antibodies from patients with DCM markedly enhanced myocardial [Ca2+]i. Betaloc, a 5, -receptor blocker, might inhibit the increase of the antibody-mediated myocardial [Ca2+]i, and the sera from healthy donors had no effect on myocardial [Ca2+]i,. Our results suggest that the anti-β-adrenoceptor antibody might increase myocardial [Ca2+]i, and result in myocardial damage. The antibodies might activate receptor-gating Ca2+-channel, thereby causing myocardial [Ca2+]i, rise and calcium overload. Early use of betaloc is recommended in the treatment of dilated cardiomyopathy.展开更多
Background Previous studies had demonstrated hemostatic abnormalities in patients with heart failure (HF) and several studies have shown that abnormal coagulation indices, represented by elevated D-dimer, had prognost...Background Previous studies had demonstrated hemostatic abnormalities in patients with heart failure (HF) and several studies have shown that abnormal coagulation indices, represented by elevated D-dimer, had prognostic significance in patients with compatible or acute decompensated HF. However, the impact of D-dimer on the outcome in patients with end-stage HF remains unclear. Methods A total of 244 consecutive patients with end-stage HF due to idiopathic dilated cardiomyopathy (DCM) were prospectively enrolled from February 2011 to September 2014. D-dimer levels were measured and its prognostic value was assessed. Primary endpoint was all-cause mortality during the follow-up period. Secondary endpoints were stroke, bleeding, occurrence of sustained ventricular tachycardia or ventricular fibrillation, and major adverse cardiovascular events (MACE). Results D-dimer was significantly elevated in the non-survivors (median: 0.8 vs. 1.1 mg/L, P < 0.001). Traditional markers including B-type natriuretic peptide, troponin I, left ventricular ejection fraction, and left ventricular end-diastolic dimension provided limited prognostic value;but the addition of D-dimer refined the risk stratification. The optimal cut-off value of D-dimer to predict all-cause mortality was 0.84 mg/L by receiver operator characteristic analysis. Elevated D-dimer level was independently associated with increased risk of long-term all-cause mortality (HR = 2.315, 95% CI: 1.570–3.414, P < 0.001) and MACE (HR = 1.256, 95% CI: 1.058–1.490, P = 0.009), and the predictive value was independent of age, sex, atrial fibrillation and anticoagulation status. Conclusions Elevated D-dimer level was independently associated with poor long-term outcome in patients with end-stage HF secondary to idiopathic DCM, and the predictive value was superior to that of traditional prognostic markers.展开更多
AIM To assess for passive expansion of sub-maximally dilated transjugular intrahepatic portosystemic shunts(TIPS) and compare outcomes with maximally dilated TIPS.METHODS Polytetrafluoroethylene covered TIPS(Viatorr) ...AIM To assess for passive expansion of sub-maximally dilated transjugular intrahepatic portosystemic shunts(TIPS) and compare outcomes with maximally dilated TIPS.METHODS Polytetrafluoroethylene covered TIPS(Viatorr) from July 2002 to December 2013 were retrospectively reviewed at two hospitals in a single institution. Two hundred and thirty patients had TIPS maximally dilated to 10 mm(m TIPS), while 43 patients who were at increased risk for hepatic encephalopathy(HE), based on clinical evaluation or low pre-TIPS portosystemic gradient(PSG), had 10 mm TIPS sub-maximally dilated to 8 mm(sm TIPS). Group characteristics(age, gender, Model for End-Stage Liver Disease score, post-TIPS PSG and clinical outcomes were compared between groups, including clinical success(ascites or varices), primary patency,primary assisted patency, and severe post-TIPS HE. A subset of fourteen patients with sm TIPS underwent follow-up computed tomography imaging after TIPS creation, and were grouped based on time of imaging(< 6 mo and > 6 mo). Change in diameter and crosssectional area were measured with 3D imaging software to evaluate for passive expansion.RESULTS Patient characteristics were similar between the sm TIPS and m TIPS groups, except for pre-TIPS portosystemic gradient, which was lower in the sm TIPS group(19.4 mm Hg ± 6.8 vs 22.4 mm Hg ± 7.1, P = 0.01). Primary patency and primary assisted patency between sm TIPS and m TIPS was not significantly different(P = 0.64 and 0.55, respectively). Four of the 55 patients(7%) with sm TIPS required TIPS reduction for severe refractory HE, while this occurred in 6 of the 218 patients(3%) with m TIPS(P = 0.12). For the 14 patients with follow-up computed tomography(CT) imaging, the median imaging follow-up was 373 d. There was an increase in median TIPS diameter, median percent diameter change, median area, and median percent area change in patients with CT follow-up greater than 6 mo after TIPS placement compared to follow-up within 6 mo(8.45 mm, 5.58%, 56.04 mm^2, and 11.48%, respectively, P = 0.01).CONCLUSION Passive expansion of sm TIPS does occur but clinical outcomes of sm TIPS and m TIPS were similar. Sub-maximal dilation can prevent complications related to overshunting in select patients.展开更多
Myocardial fiber deformation measurements have been reported to be associated with adverse outcomes in patients with acute heart failure and those with myocardial infarction.However,few studies have addressed the prog...Myocardial fiber deformation measurements have been reported to be associated with adverse outcomes in patients with acute heart failure and those with myocardial infarction.However,few studies have addressed the prognostic value of global circumferential strain(GCS)in dilated cardiomyopathy(DCM)patients with severely impaired systolic function.This study aimed to evaluate the prognostic value of cardiac magnetic resonance(CMR)-derived GCS in DCM patients with severely reduced ejection.Consecutive DCM patients with severely reduced ejection fraction(EF<35%)who underwent CMR were included.GCS was calculated from CMR cine images.The clinical endpoint was a composite of all-cause mortality,heart transplantation,implantable cardioverter defibrillator(ICD)implantation and aborted sudden cardiac death(SCD).A total of 129 patients with a mean EF of 15.33%(11.36%–22.27%)were included.During a median follow-up of 518 days,endpoint events occurred in 50 patients.Patients with GCS≥the median(−5.17%)had significantly reduced event-free survival as compared with those with GCS<the median(P<0.01).GCS was independently associated with adverse events after adjusting for clinical and imaging risk factors including extent of late gadolinium enhancement(LGE)(P<0.05).Adding GCS into the model including the extent of LGE resulted in significant improvements in the C-statistic(from 0.706 to 0.742;P<0.05)with a continuous net reclassification improvement(NRI)of 29.71%.It was concluded that GCS derived from CMR could be useful for risk stratification in DCM patients with severely reduced EF,which may increase common imaging risk factors including LGE.展开更多
Background Contemporary heart failure medications have led to considerable improvement in the survival of patients with heart failure. However,limited evidence is available regarding the effect of those medications in...Background Contemporary heart failure medications have led to considerable improvement in the survival of patients with heart failure. However,limited evidence is available regarding the effect of those medications in patients with idiopathic dilated cardiomyopathy (IDCM),particularly in China. We sought to analyze the trends in clinical characteristics and the prescription rate of recommended therapies and its prognostic impact in patients with IDCM. Methods From 2009 to 2016,1441 consecutive patients (age: 55±14 years,68% men,LVEF: 33%± 12%) fulfilling World Health Organization criteria for IDCM were enrolled in the current retrospective cohort study. Temporal trends of baseline clinical characteristics,treatment and prognosis were analyzed,and potential influential factors were explored. Results Rates of patients receiving angiotensin-converting enzyme inhibitors/angiotensin II receptors blockers,β-blockers,aldosterone receptor antagonists and diuretics increased from 55%,45%,58%,51% in 2009 to 67%,69%,71%,64% in 2016,respectively (P < 0.05);whereas,the proportion of patients receiving digoxin decreased from 39% in 2009 to 28% in 2016 (P < 0.05). The overall proportion of patients with optimal guideline-directed medical therapy (GDMT) was 44.6%;however,that rate increased from 33% in 2009 to 41%,49% and 56% in 2012,2014 and 2016 respectively (P < 0.05). Patients with optimal GDMT had a better outcome than those without,but there was no temporal trend toward improvement in the overall long-term prognosis of IDCM patients with the years. There was a trend towards admission of patients with milder disease and toward increased admission to a cardiology ward with the years. Conclusions An improvement in prescription rates of guideline-recommended medications in IDCM patients was observed. However,it remains suboptimal,and there is still some room for improvement. The prognosis of patients with optimal GDMT was better than those without. Moreover,the following patient category also had an improved prognosis: patients with LVEF ≥ 40%,with device therapy,and those admitted to a cardiology ward.展开更多
Malignant ventricular arrhythmias are a major adverse event and worsen the prognosis of patients affected by ischemic and non-ischemic dilated cardiomyopathy.The main parameter currently used to stratify arrhythmic ri...Malignant ventricular arrhythmias are a major adverse event and worsen the prognosis of patients affected by ischemic and non-ischemic dilated cardiomyopathy.The main parameter currently used to stratify arrhythmic risk and guide decision making towards the implantation of a cardioverter defibrillator is the evaluation of the left ventricular ejection fraction.However,this strategy is characterized by several limitations and consequently additional parameters have been suggested in order to improve arrhythmic risk stratification.The aim of this review is to critically revise the prognostic significance of non-invasive diagnostic tools in order to better stratify the arrhythmic risk prognosis of dilated cardiomyopathy patients.展开更多
Emotion recognition from speech data is an active and emerging area of research that plays an important role in numerous applications,such as robotics,virtual reality,behavior assessments,and emergency call centers.Re...Emotion recognition from speech data is an active and emerging area of research that plays an important role in numerous applications,such as robotics,virtual reality,behavior assessments,and emergency call centers.Recently,researchers have developed many techniques in this field in order to ensure an improvement in the accuracy by utilizing several deep learning approaches,but the recognition rate is still not convincing.Our main aim is to develop a new technique that increases the recognition rate with reasonable cost computations.In this paper,we suggested a new technique,which is a one-dimensional dilated convolutional neural network(1D-DCNN)for speech emotion recognition(SER)that utilizes the hierarchical features learning blocks(HFLBs)with a bi-directional gated recurrent unit(BiGRU).We designed a one-dimensional CNN network to enhance the speech signals,which uses a spectral analysis,and to extract the hidden patterns from the speech signals that are fed into a stacked one-dimensional dilated network that are called HFLBs.Each HFLB contains one dilated convolution layer(DCL),one batch normalization(BN),and one leaky_relu(Relu)layer in order to extract the emotional features using a hieratical correlation strategy.Furthermore,the learned emotional features are feed into a BiGRU in order to adjust the global weights and to recognize the temporal cues.The final state of the deep BiGRU is passed from a softmax classifier in order to produce the probabilities of the emotions.The proposed model was evaluated over three benchmarked datasets that included the IEMOCAP,EMO-DB,and RAVDESS,which achieved 72.75%,91.14%,and 78.01%accuracy,respectively.展开更多
Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are someti...Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.展开更多
In ocean explorations,side-scan sonar(SSS)plays a very important role and can quickly depict seabed topography.As-sembling the SSS to an autonomous underwater vehicle(AUV)and performing semantic segmentation of an SSS...In ocean explorations,side-scan sonar(SSS)plays a very important role and can quickly depict seabed topography.As-sembling the SSS to an autonomous underwater vehicle(AUV)and performing semantic segmentation of an SSS image in real time can realize online submarine geomorphology or target recognition,which is conducive to submarine detection.However,because of the complexity of the marine environment,various noises in the ocean pollute the sonar image,which also encounters the intensity inhomogeneity problem.In this paper,we propose a novel neural network architecture named dilated convolutional neural network(DcNet)that can run in real time while addressing the above-mentioned issues and providing accurate semantic segmentation.The proposed architecture presents an encoder-decoder network to gradually reduce the spatial dimension of the input image and recover the details of the target,respectively.The core of our network is a novel block connection named DCblock,which mainly uses dilated convolution and depthwise separable convolution between the encoder and decoder to attain more context while still retaining high accuracy.Furthermore,our proposed method performs a super-resolution reconstruction to enlarge the dataset with high-quality im-ages.We compared our network to other common semantic segmentation networks performed on an NVIDIA Jetson TX2 using our sonar image datasets.Experimental results show that while the inference speed of the proposed network significantly outperforms state-of-the-art architectures,the accuracy of our method is still comparable,which indicates its potential applications not only in AUVs equipped with SSS but also in marine exploration.展开更多
Objective To evaluate the efficacy and safety of long-term treatment with arotinolol in patients with idiopathic dilated cardiomyopathy(IDCM).Methods Sixty-three patients with IDCM were evaluated at baseline and after...Objective To evaluate the efficacy and safety of long-term treatment with arotinolol in patients with idiopathic dilated cardiomyopathy(IDCM).Methods Sixty-three patients with IDCM were evaluated at baseline and after 12-month therapy with arotinolol.The conventional therapy for congestive heart failure was continued throughout the study with arotinolol as the only β-blocker.Left ventricular function was assessed with the New York Heart Association functional class and two-dimensional echocardiography.Results After 12-month arotinolol treatment,there was a significant improvement in left ventricular systolic function.Left ventricular end-systolic dimension significantly decreased from 59.52±8.83 mm to 50.89±8.17 mm(P<0.001).Left ventricular ejection fraction significantly increased from 27.39%±7.94% to 41.13%±9.45%(P<0.001).Left ventricular mass index decreased from 150.47±42.42 g/m2 to 141.58±34.36 g/m2(P<0.01).No adverse events leading to premature discontinuation of study drug occurred.Conclusion In this preliminary study,12-month arotinolol treatment has a favorable effect on left ventricular function in patients with IDCM,and it is safe and well tolerated.展开更多
Clinical, electrocardiographic and echocardiographic findings in 64 patients with dilated cardiomyopathy were retrospectively studied. Compared with 51 patients without complete left bundle branch block (CLBBB), 13 pa...Clinical, electrocardiographic and echocardiographic findings in 64 patients with dilated cardiomyopathy were retrospectively studied. Compared with 51 patients without complete left bundle branch block (CLBBB), 13 patients with CLBBB had higher New York Heart Association (NYHA) functional class (P<0. 05), increased left ventricular end-diastolic and end-systolic diameters (P<0. 002) and myocardial mass (P<0. 02). severe mitral regurgitation (P<0. 01) and higher mortality rate (P<0. 04). Multivariate stepwise regression analysis revealed that the presence of CLBBB was an independent prognostic factor for patients with dilated cardiomyopathy.展开更多
BACKGROUND Nemaline myopathy(NM)is a rare type of congenital myopathy,with an incidence of 1:50000.Patients with NM often exhibit hypomyotonia and varying degrees of muscle weakness.Skeletal muscles are always affecte...BACKGROUND Nemaline myopathy(NM)is a rare type of congenital myopathy,with an incidence of 1:50000.Patients with NM often exhibit hypomyotonia and varying degrees of muscle weakness.Skeletal muscles are always affected by this disease,while myocardial involvement is uncommon.However,with improvements in genetic testing technology,it has been found that NM with a mutation in the myopalladin(MYPN)gene not only causes slow,progressive muscle weakness but also results in dilated or hypertrophic cardiomyopathy.CASE SUMMARY A 3-year-old pre-school boy was admitted to our hospital with cough,edema,tachypnea,and an increased heart rate.The patient was clinically diagnosed with severe dilated cardiomyopathy and heart failure,and subsequent gene examination confirmed the diagnosis of NM with a mutation in MYPN.Captopril,diuretics,low-dose digoxin,and dobutamine were administered.After 22 d of hospitalization,the patient was discharged due to the improvement of clinical symptoms.During the follow-up period,the patient died of refractory heart failure.CONCLUSION Decreased muscular tone and dilated cardiomyopathy are common features of MYPN-mutated NM.Heart transplantation may be a solution to this type of cardiomyopathy.展开更多
Objective: To determine whether a mutation of mitochondrial DNA induces familial dilated cardiomyopathy in Chinese families with cardiomyopathy, and analyzed the correlation between the genotype and phenotype. Method...Objective: To determine whether a mutation of mitochondrial DNA induces familial dilated cardiomyopathy in Chinese families with cardiomyopathy, and analyzed the correlation between the genotype and phenotype. Methods: Affected members in three Chinese families of the familial dilated cardiomyopathy underwent clinical evaluation and DNA analysis. Polymerase chain reaction and direct DNA sequencing were used to screen for mitochondrial DNA mutation. The type of mtDNA variations and clinical situation were analysed on the patients with mitochondrial DNA mutation. Results: The mitochondrial A3434G mutation was identified in one of the three families,the 3434 th nucleotide A was replaced by G, which led to change of amino acid. No mutations were identified in the clinically unaffected members of the family and all members of the other two families. Conclusion: This study indicates that the mitochondrial A3434G mutation maybe related with familial dilated cardiomyopathy and deafness.展开更多
Objective Dilated cardiomyopathy(DCM) is generally considered to be accompanied by both left and right ventricular dysfunction,but most studies only analyze the left ventricular function. In this study,we evaluated th...Objective Dilated cardiomyopathy(DCM) is generally considered to be accompanied by both left and right ventricular dysfunction,but most studies only analyze the left ventricular function. In this study,we evaluated the effect of arotinolol on right ventricular function in patients with DCM. Methods Right ventricular ejection fraction(RVEF) and right ventricular diameter(RVD) were measured by two-dimensional echocardiography(2-DE) in 33 DCM patients;RVEF measured by first-pass radionuclide angiography(FPRA) was compared with that by 2-DE. Results The treatment with arotinolol for one year resulted in a reduction in the right ventricular diameter(baseline,23.0 ± 8.3 mm vs after one-year treatment,20.7 ± 5.4 mm;P=0.004 ) and an associated increase in ejection fraction(baseline,36.9 ± 10.3% vs after one-year treatment,45.8 ± 9.6%;P < 0.001 ) ;there is a high correlation between the 2-DE method and radionuclide ventriculographic method. The correlation coefficient is 0.933(P<0.001) . Conclusion Arotinolol therapy could not only improve left ventricular function,but also improve right ventricular function in DCM patients.展开更多
Background: 24-dehydrocholesterol reductase(Dhcr24) catalyzes the last step of cholesterol biosynthesis, which is required for normal development and antiapoptotic activities of tissues. We found that Dhcr24 expressio...Background: 24-dehydrocholesterol reductase(Dhcr24) catalyzes the last step of cholesterol biosynthesis, which is required for normal development and antiapoptotic activities of tissues. We found that Dhcr24 expression decreased in the cTnTR141 Wdilated cardiomyopathy(DCM) transgenic mice. Therefore, we tested whether rescued expression of Dhcr24 could prevent the development of DCM and its possible mechanism.Methods: Heart tissue specific transgenic overexpression mice of Dhcr24 was generated, then was crossed to c TnTR141 Wmouse to obtain the double transgenic mouse(DTG). The phenotypes were demonstrated by the survival, cardiac geometry and function analysis, as well as microstructural and ultrastructural observations based on echocardiography and histology examination. The pathway and apoptosis were analysed by western blotting and TUNEL assay in vivo and in vitro.Results: We find that Dhcr24 decreased in hearts tissues of cTnTR141 Wand LMNAE82 KDCM mice. The transgenic overexpression of Dhcr24 significantly improves DCM phenotypes in cTnTR141 Wmice, and activates PI3K/Akt/HKII pathway, followed by a reduction of the translocation of Bax and release of cytochrome c, caspase-9 and caspase-3 activation and myocyte apoptosis. Knockdown the expression of Dhcr24 reduces the activation of PI3K/Akt/HKII pathway and inhibition of the mitochondrial-dependent apoptosis. The anti-apoptotic effect of Dhcr24 could be completely removed by the inhibition of PI3K pathway and partly removed by the HKII inhibitor in H9c2 cell line.Conclusion: Compensatory expression of Dhcr24 protect against DCM through activated PI3K/Akt/HKII pathway and reduce Bax translocation. This is the first investigation for the molecular mechanism of Dhcr24 participate in development of DCM.展开更多
基金funded by the Fundamental Research Project of CNPC Geophysical Key Lab(2022DQ0604-4)the Strategic Cooperation Technology Projects of China National Petroleum Corporation and China University of Petroleum-Beijing(ZLZX 202003)。
文摘With the successful application and breakthrough of deep learning technology in image segmentation,there has been continuous development in the field of seismic facies interpretation using convolutional neural networks.These intelligent and automated methods significantly reduce manual labor,particularly in the laborious task of manually labeling seismic facies.However,the extensive demand for training data imposes limitations on their wider application.To overcome this challenge,we adopt the UNet architecture as the foundational network structure for seismic facies classification,which has demonstrated effective segmentation results even with small-sample training data.Additionally,we integrate spatial pyramid pooling and dilated convolution modules into the network architecture to enhance the perception of spatial information across a broader range.The seismic facies classification test on the public data from the F3 block verifies the superior performance of our proposed improved network structure in delineating seismic facies boundaries.Comparative analysis against the traditional UNet model reveals that our method achieves more accurate predictive classification results,as evidenced by various evaluation metrics for image segmentation.Obviously,the classification accuracy reaches an impressive 96%.Furthermore,the results of seismic facies classification in the seismic slice dimension provide further confirmation of the superior performance of our proposed method,which accurately defines the range of different seismic facies.This approach holds significant potential for analyzing geological patterns and extracting valuable depositional information.
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.
文摘Objective: To screen and analyze the differentially expressed genes between dilated cardiomyopathy (DCM) and chronic heart failure (CHF) based on bioinformatics methods. Methods: The Gene Expression Omnibus (GEO) database was used for data retrieval, and the chip data GSE3585 was downloaded, which was the original data of DCM and normal control group. At the same time, the chip data GSE76701 was downloaded, which was the original data of CHF and control group. Differentially expressed mRNAs (DEmRNAs) were screened by R language limma package, the data were standardized, and the common differentially expressed genes were screened. GO function and KEGG pathway enrichment analysis were performed on the common differentially expressed genes. String11.0 online tool was used for data analysis to obtain differentially expressed genes, and the results were imported into Cytoscape 3.9.1 software. The results were imported into Cytoscape 3.9.1 software, and the common expression gene module was obtained by MOCDE algorithm. Nine Hub genes were obtained by 10 algorithms such as MCC. Results: A total of 248 differentially expressed genes were screened. GO analysis showed that differentially expressed genes were mainly concentrated in 9 different physiological and pathological processes. KEGG analysis showed that the main signaling pathways involved in differentially expressed genes were 2, and 9 key differentially expressed genes were predicted: NPPB, NPPA, MYH6, FRZB, ASPN, SFRP4, RPS4Y1, DDX3Y. Conclusion: This study preliminarily explored the molecular mechanism of DCM and CHF, and obtained the common differentially expressed genes of the two diseases. Further experimental studies are needed to verify the correlation between gene expression and clinicopathological features. Provide new ideas for clinical drug treatment research.
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Backgrounds Clinical trials have demonstrated that cardiac resynchronization therapy (CRT) is effective in patients with "non-is- chemic cardiomyopathy". However, patients with dilated-phase hypertrophic cardiomyopathy (DHCM) have been generally excluded from such trials. We aimed to compare the clinical outcome of CRT in patients with DHCM, idiopathic dilated cardiomyopathy (IDCM), or ischemic cardiomyopathy (ICM). Methods A total of 312 consecutive patients (DHCM: n = 16; IDCM: n = 231; ICM: n = 65) undergoing CRT in Fuwai hospital were studied respectively. Response to CRT was defmed as reduction in left ventricular end-systolic volume (LVESV) _〉 15% at 6-month follow-up. Results Compared with DHCM, IDCM was associated with a lower total mortality (HR: 0.35, 95% CI: 0.13-0.90), cardiac mortality (HR: 0.29; 95% CI: 0.11-0.77), and total mortality or heart failure (HF) hospitalizations (HR: 0.34, 95% CI: 0.17-0.69), independent of known confounders. Compared with DHCM, the total mortality, cardiac mortality and total mortality or HF hospitalizations favored ICM but were not statistically significant (HR: 0.59, 95% CI: 0.22-1.61; HR: 0.59, 95% CI: 0.21-1.63; HR: 0.54, 95% CI: 0.26-1.15; respectively). Response rate to CRT was lower in the DHCM group than the other two groups although the differences didn't reach statistical significance. Conclusions Compared with IDCM, DHCM was associated with a worse outcome after CRT. The clinical outcome of DHCM patients receiving CRT was similar to or even worse than that of ICM patients. These indicate that DHCM behaves very differently after CRT.
文摘Antibodies against &-adrenoceptor can be detected in serum of patients with dilated cardiomyopathy (DCM), which have 5-agonist-like activity, and induce a positive chronotropic effect on cardiac myocytes by its persistence at full strength. Effects of the antibodies against Padrenoceptor from sera of patients with DCM on myocardial cytotoxicity and cytoplasmic free Ca2+-concentration (LCa2+ji) were observed in the cultured single layer SD rat ventricular cells by using the cytotoxicity assay and fluorescent Ca2+- indicat0r fura-2/AM. The positive sera of the anti-&adrenoceptor antibodies from patients with DCM markedly enhanced myocardial [Ca2+]i. Betaloc, a 5, -receptor blocker, might inhibit the increase of the antibody-mediated myocardial [Ca2+]i, and the sera from healthy donors had no effect on myocardial [Ca2+]i,. Our results suggest that the anti-β-adrenoceptor antibody might increase myocardial [Ca2+]i, and result in myocardial damage. The antibodies might activate receptor-gating Ca2+-channel, thereby causing myocardial [Ca2+]i, rise and calcium overload. Early use of betaloc is recommended in the treatment of dilated cardiomyopathy.
文摘Background Previous studies had demonstrated hemostatic abnormalities in patients with heart failure (HF) and several studies have shown that abnormal coagulation indices, represented by elevated D-dimer, had prognostic significance in patients with compatible or acute decompensated HF. However, the impact of D-dimer on the outcome in patients with end-stage HF remains unclear. Methods A total of 244 consecutive patients with end-stage HF due to idiopathic dilated cardiomyopathy (DCM) were prospectively enrolled from February 2011 to September 2014. D-dimer levels were measured and its prognostic value was assessed. Primary endpoint was all-cause mortality during the follow-up period. Secondary endpoints were stroke, bleeding, occurrence of sustained ventricular tachycardia or ventricular fibrillation, and major adverse cardiovascular events (MACE). Results D-dimer was significantly elevated in the non-survivors (median: 0.8 vs. 1.1 mg/L, P < 0.001). Traditional markers including B-type natriuretic peptide, troponin I, left ventricular ejection fraction, and left ventricular end-diastolic dimension provided limited prognostic value;but the addition of D-dimer refined the risk stratification. The optimal cut-off value of D-dimer to predict all-cause mortality was 0.84 mg/L by receiver operator characteristic analysis. Elevated D-dimer level was independently associated with increased risk of long-term all-cause mortality (HR = 2.315, 95% CI: 1.570–3.414, P < 0.001) and MACE (HR = 1.256, 95% CI: 1.058–1.490, P = 0.009), and the predictive value was independent of age, sex, atrial fibrillation and anticoagulation status. Conclusions Elevated D-dimer level was independently associated with poor long-term outcome in patients with end-stage HF secondary to idiopathic DCM, and the predictive value was superior to that of traditional prognostic markers.
文摘AIM To assess for passive expansion of sub-maximally dilated transjugular intrahepatic portosystemic shunts(TIPS) and compare outcomes with maximally dilated TIPS.METHODS Polytetrafluoroethylene covered TIPS(Viatorr) from July 2002 to December 2013 were retrospectively reviewed at two hospitals in a single institution. Two hundred and thirty patients had TIPS maximally dilated to 10 mm(m TIPS), while 43 patients who were at increased risk for hepatic encephalopathy(HE), based on clinical evaluation or low pre-TIPS portosystemic gradient(PSG), had 10 mm TIPS sub-maximally dilated to 8 mm(sm TIPS). Group characteristics(age, gender, Model for End-Stage Liver Disease score, post-TIPS PSG and clinical outcomes were compared between groups, including clinical success(ascites or varices), primary patency,primary assisted patency, and severe post-TIPS HE. A subset of fourteen patients with sm TIPS underwent follow-up computed tomography imaging after TIPS creation, and were grouped based on time of imaging(< 6 mo and > 6 mo). Change in diameter and crosssectional area were measured with 3D imaging software to evaluate for passive expansion.RESULTS Patient characteristics were similar between the sm TIPS and m TIPS groups, except for pre-TIPS portosystemic gradient, which was lower in the sm TIPS group(19.4 mm Hg ± 6.8 vs 22.4 mm Hg ± 7.1, P = 0.01). Primary patency and primary assisted patency between sm TIPS and m TIPS was not significantly different(P = 0.64 and 0.55, respectively). Four of the 55 patients(7%) with sm TIPS required TIPS reduction for severe refractory HE, while this occurred in 6 of the 218 patients(3%) with m TIPS(P = 0.12). For the 14 patients with follow-up computed tomography(CT) imaging, the median imaging follow-up was 373 d. There was an increase in median TIPS diameter, median percent diameter change, median area, and median percent area change in patients with CT follow-up greater than 6 mo after TIPS placement compared to follow-up within 6 mo(8.45 mm, 5.58%, 56.04 mm^2, and 11.48%, respectively, P = 0.01).CONCLUSION Passive expansion of sm TIPS does occur but clinical outcomes of sm TIPS and m TIPS were similar. Sub-maximal dilation can prevent complications related to overshunting in select patients.
基金the National Natural Science Foundation of China(Nos.81701653 and 81570348).
文摘Myocardial fiber deformation measurements have been reported to be associated with adverse outcomes in patients with acute heart failure and those with myocardial infarction.However,few studies have addressed the prognostic value of global circumferential strain(GCS)in dilated cardiomyopathy(DCM)patients with severely impaired systolic function.This study aimed to evaluate the prognostic value of cardiac magnetic resonance(CMR)-derived GCS in DCM patients with severely reduced ejection.Consecutive DCM patients with severely reduced ejection fraction(EF<35%)who underwent CMR were included.GCS was calculated from CMR cine images.The clinical endpoint was a composite of all-cause mortality,heart transplantation,implantable cardioverter defibrillator(ICD)implantation and aborted sudden cardiac death(SCD).A total of 129 patients with a mean EF of 15.33%(11.36%–22.27%)were included.During a median follow-up of 518 days,endpoint events occurred in 50 patients.Patients with GCS≥the median(−5.17%)had significantly reduced event-free survival as compared with those with GCS<the median(P<0.01).GCS was independently associated with adverse events after adjusting for clinical and imaging risk factors including extent of late gadolinium enhancement(LGE)(P<0.05).Adding GCS into the model including the extent of LGE resulted in significant improvements in the C-statistic(from 0.706 to 0.742;P<0.05)with a continuous net reclassification improvement(NRI)of 29.71%.It was concluded that GCS derived from CMR could be useful for risk stratification in DCM patients with severely reduced EF,which may increase common imaging risk factors including LGE.
基金supported by grants from the National Natural Science Foundation of China (No.81400267 & No.81370219)the Sichuan Provincial Department of Science and Technology (2016FZ0084)
文摘Background Contemporary heart failure medications have led to considerable improvement in the survival of patients with heart failure. However,limited evidence is available regarding the effect of those medications in patients with idiopathic dilated cardiomyopathy (IDCM),particularly in China. We sought to analyze the trends in clinical characteristics and the prescription rate of recommended therapies and its prognostic impact in patients with IDCM. Methods From 2009 to 2016,1441 consecutive patients (age: 55±14 years,68% men,LVEF: 33%± 12%) fulfilling World Health Organization criteria for IDCM were enrolled in the current retrospective cohort study. Temporal trends of baseline clinical characteristics,treatment and prognosis were analyzed,and potential influential factors were explored. Results Rates of patients receiving angiotensin-converting enzyme inhibitors/angiotensin II receptors blockers,β-blockers,aldosterone receptor antagonists and diuretics increased from 55%,45%,58%,51% in 2009 to 67%,69%,71%,64% in 2016,respectively (P < 0.05);whereas,the proportion of patients receiving digoxin decreased from 39% in 2009 to 28% in 2016 (P < 0.05). The overall proportion of patients with optimal guideline-directed medical therapy (GDMT) was 44.6%;however,that rate increased from 33% in 2009 to 41%,49% and 56% in 2012,2014 and 2016 respectively (P < 0.05). Patients with optimal GDMT had a better outcome than those without,but there was no temporal trend toward improvement in the overall long-term prognosis of IDCM patients with the years. There was a trend towards admission of patients with milder disease and toward increased admission to a cardiology ward with the years. Conclusions An improvement in prescription rates of guideline-recommended medications in IDCM patients was observed. However,it remains suboptimal,and there is still some room for improvement. The prognosis of patients with optimal GDMT was better than those without. Moreover,the following patient category also had an improved prognosis: patients with LVEF ≥ 40%,with device therapy,and those admitted to a cardiology ward.
文摘Malignant ventricular arrhythmias are a major adverse event and worsen the prognosis of patients affected by ischemic and non-ischemic dilated cardiomyopathy.The main parameter currently used to stratify arrhythmic risk and guide decision making towards the implantation of a cardioverter defibrillator is the evaluation of the left ventricular ejection fraction.However,this strategy is characterized by several limitations and consequently additional parameters have been suggested in order to improve arrhythmic risk stratification.The aim of this review is to critically revise the prognostic significance of non-invasive diagnostic tools in order to better stratify the arrhythmic risk prognosis of dilated cardiomyopathy patients.
基金supported by the National Research Foundation of Korea funded by the Korean Government through the Ministry of Science and ICT under Grant NRF-2020R1F1A1060659 and in part by the 2020 Faculty Research Fund of Sejong University。
文摘Emotion recognition from speech data is an active and emerging area of research that plays an important role in numerous applications,such as robotics,virtual reality,behavior assessments,and emergency call centers.Recently,researchers have developed many techniques in this field in order to ensure an improvement in the accuracy by utilizing several deep learning approaches,but the recognition rate is still not convincing.Our main aim is to develop a new technique that increases the recognition rate with reasonable cost computations.In this paper,we suggested a new technique,which is a one-dimensional dilated convolutional neural network(1D-DCNN)for speech emotion recognition(SER)that utilizes the hierarchical features learning blocks(HFLBs)with a bi-directional gated recurrent unit(BiGRU).We designed a one-dimensional CNN network to enhance the speech signals,which uses a spectral analysis,and to extract the hidden patterns from the speech signals that are fed into a stacked one-dimensional dilated network that are called HFLBs.Each HFLB contains one dilated convolution layer(DCL),one batch normalization(BN),and one leaky_relu(Relu)layer in order to extract the emotional features using a hieratical correlation strategy.Furthermore,the learned emotional features are feed into a BiGRU in order to adjust the global weights and to recognize the temporal cues.The final state of the deep BiGRU is passed from a softmax classifier in order to produce the probabilities of the emotions.The proposed model was evaluated over three benchmarked datasets that included the IEMOCAP,EMO-DB,and RAVDESS,which achieved 72.75%,91.14%,and 78.01%accuracy,respectively.
文摘Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.
基金This work is partially supported by the Natural Key Research and Development Program of China(No.2016YF C0301400).
文摘In ocean explorations,side-scan sonar(SSS)plays a very important role and can quickly depict seabed topography.As-sembling the SSS to an autonomous underwater vehicle(AUV)and performing semantic segmentation of an SSS image in real time can realize online submarine geomorphology or target recognition,which is conducive to submarine detection.However,because of the complexity of the marine environment,various noises in the ocean pollute the sonar image,which also encounters the intensity inhomogeneity problem.In this paper,we propose a novel neural network architecture named dilated convolutional neural network(DcNet)that can run in real time while addressing the above-mentioned issues and providing accurate semantic segmentation.The proposed architecture presents an encoder-decoder network to gradually reduce the spatial dimension of the input image and recover the details of the target,respectively.The core of our network is a novel block connection named DCblock,which mainly uses dilated convolution and depthwise separable convolution between the encoder and decoder to attain more context while still retaining high accuracy.Furthermore,our proposed method performs a super-resolution reconstruction to enlarge the dataset with high-quality im-ages.We compared our network to other common semantic segmentation networks performed on an NVIDIA Jetson TX2 using our sonar image datasets.Experimental results show that while the inference speed of the proposed network significantly outperforms state-of-the-art architectures,the accuracy of our method is still comparable,which indicates its potential applications not only in AUVs equipped with SSS but also in marine exploration.
文摘Objective To evaluate the efficacy and safety of long-term treatment with arotinolol in patients with idiopathic dilated cardiomyopathy(IDCM).Methods Sixty-three patients with IDCM were evaluated at baseline and after 12-month therapy with arotinolol.The conventional therapy for congestive heart failure was continued throughout the study with arotinolol as the only β-blocker.Left ventricular function was assessed with the New York Heart Association functional class and two-dimensional echocardiography.Results After 12-month arotinolol treatment,there was a significant improvement in left ventricular systolic function.Left ventricular end-systolic dimension significantly decreased from 59.52±8.83 mm to 50.89±8.17 mm(P<0.001).Left ventricular ejection fraction significantly increased from 27.39%±7.94% to 41.13%±9.45%(P<0.001).Left ventricular mass index decreased from 150.47±42.42 g/m2 to 141.58±34.36 g/m2(P<0.01).No adverse events leading to premature discontinuation of study drug occurred.Conclusion In this preliminary study,12-month arotinolol treatment has a favorable effect on left ventricular function in patients with IDCM,and it is safe and well tolerated.
文摘Clinical, electrocardiographic and echocardiographic findings in 64 patients with dilated cardiomyopathy were retrospectively studied. Compared with 51 patients without complete left bundle branch block (CLBBB), 13 patients with CLBBB had higher New York Heart Association (NYHA) functional class (P<0. 05), increased left ventricular end-diastolic and end-systolic diameters (P<0. 002) and myocardial mass (P<0. 02). severe mitral regurgitation (P<0. 01) and higher mortality rate (P<0. 04). Multivariate stepwise regression analysis revealed that the presence of CLBBB was an independent prognostic factor for patients with dilated cardiomyopathy.
基金the Sichuan Province Science and Technology Support Program of China。
文摘BACKGROUND Nemaline myopathy(NM)is a rare type of congenital myopathy,with an incidence of 1:50000.Patients with NM often exhibit hypomyotonia and varying degrees of muscle weakness.Skeletal muscles are always affected by this disease,while myocardial involvement is uncommon.However,with improvements in genetic testing technology,it has been found that NM with a mutation in the myopalladin(MYPN)gene not only causes slow,progressive muscle weakness but also results in dilated or hypertrophic cardiomyopathy.CASE SUMMARY A 3-year-old pre-school boy was admitted to our hospital with cough,edema,tachypnea,and an increased heart rate.The patient was clinically diagnosed with severe dilated cardiomyopathy and heart failure,and subsequent gene examination confirmed the diagnosis of NM with a mutation in MYPN.Captopril,diuretics,low-dose digoxin,and dobutamine were administered.After 22 d of hospitalization,the patient was discharged due to the improvement of clinical symptoms.During the follow-up period,the patient died of refractory heart failure.CONCLUSION Decreased muscular tone and dilated cardiomyopathy are common features of MYPN-mutated NM.Heart transplantation may be a solution to this type of cardiomyopathy.
基金Guangxi province science research grants(GKQ0542049)
文摘Objective: To determine whether a mutation of mitochondrial DNA induces familial dilated cardiomyopathy in Chinese families with cardiomyopathy, and analyzed the correlation between the genotype and phenotype. Methods: Affected members in three Chinese families of the familial dilated cardiomyopathy underwent clinical evaluation and DNA analysis. Polymerase chain reaction and direct DNA sequencing were used to screen for mitochondrial DNA mutation. The type of mtDNA variations and clinical situation were analysed on the patients with mitochondrial DNA mutation. Results: The mitochondrial A3434G mutation was identified in one of the three families,the 3434 th nucleotide A was replaced by G, which led to change of amino acid. No mutations were identified in the clinically unaffected members of the family and all members of the other two families. Conclusion: This study indicates that the mitochondrial A3434G mutation maybe related with familial dilated cardiomyopathy and deafness.
文摘Objective Dilated cardiomyopathy(DCM) is generally considered to be accompanied by both left and right ventricular dysfunction,but most studies only analyze the left ventricular function. In this study,we evaluated the effect of arotinolol on right ventricular function in patients with DCM. Methods Right ventricular ejection fraction(RVEF) and right ventricular diameter(RVD) were measured by two-dimensional echocardiography(2-DE) in 33 DCM patients;RVEF measured by first-pass radionuclide angiography(FPRA) was compared with that by 2-DE. Results The treatment with arotinolol for one year resulted in a reduction in the right ventricular diameter(baseline,23.0 ± 8.3 mm vs after one-year treatment,20.7 ± 5.4 mm;P=0.004 ) and an associated increase in ejection fraction(baseline,36.9 ± 10.3% vs after one-year treatment,45.8 ± 9.6%;P < 0.001 ) ;there is a high correlation between the 2-DE method and radionuclide ventriculographic method. The correlation coefficient is 0.933(P<0.001) . Conclusion Arotinolol therapy could not only improve left ventricular function,but also improve right ventricular function in DCM patients.
基金The present work was supported by the National Science and Technology Support Project(2015BAI08B01)CAMS Innovation Fund for Medical Sciences(CAMS-I2M,2016-I2M-1-015)Beijing Natural Science Foundation(5172027)
文摘Background: 24-dehydrocholesterol reductase(Dhcr24) catalyzes the last step of cholesterol biosynthesis, which is required for normal development and antiapoptotic activities of tissues. We found that Dhcr24 expression decreased in the cTnTR141 Wdilated cardiomyopathy(DCM) transgenic mice. Therefore, we tested whether rescued expression of Dhcr24 could prevent the development of DCM and its possible mechanism.Methods: Heart tissue specific transgenic overexpression mice of Dhcr24 was generated, then was crossed to c TnTR141 Wmouse to obtain the double transgenic mouse(DTG). The phenotypes were demonstrated by the survival, cardiac geometry and function analysis, as well as microstructural and ultrastructural observations based on echocardiography and histology examination. The pathway and apoptosis were analysed by western blotting and TUNEL assay in vivo and in vitro.Results: We find that Dhcr24 decreased in hearts tissues of cTnTR141 Wand LMNAE82 KDCM mice. The transgenic overexpression of Dhcr24 significantly improves DCM phenotypes in cTnTR141 Wmice, and activates PI3K/Akt/HKII pathway, followed by a reduction of the translocation of Bax and release of cytochrome c, caspase-9 and caspase-3 activation and myocyte apoptosis. Knockdown the expression of Dhcr24 reduces the activation of PI3K/Akt/HKII pathway and inhibition of the mitochondrial-dependent apoptosis. The anti-apoptotic effect of Dhcr24 could be completely removed by the inhibition of PI3K pathway and partly removed by the HKII inhibitor in H9c2 cell line.Conclusion: Compensatory expression of Dhcr24 protect against DCM through activated PI3K/Akt/HKII pathway and reduce Bax translocation. This is the first investigation for the molecular mechanism of Dhcr24 participate in development of DCM.