MYH7 mutation in a pedigree with familial dilated hypertrophic cardiomyopathy:A case report and review of literature

BACKGROUND Hypertrophic cardiomyopathy(HCM)is one of the most prevalent inherited myocardial disorders and is charac-terized by considerable genetic and phenotypic heterogeneity.A subset of patients with HCM progress to a dilated phase of HCM(DPHCM),which is associated with a poor prognosis;however,the underlying pathogenesis remains inadequately understood.CASE SUMMARY In this study,we present a case involving a pedigree with familial DPHCM and conduct a retrospective review of patients with DPHCM with identified gene mutations.Through panel sequencing targeting the coding regions of 312 genes associated with inherited cardiomyopathy,a heterozygous missense mutation(c.746G>A,p.Arg249Glu)in the MYH7 gene was identified in the proband(III-5).Sanger sequencing subsequently confirmed this pathogenic mutation in three additional family members(II-4,III-4,and IV-3).A total of 26 well-documented patients with DPHCM were identified in the literature.Patients with DPHCM are commonly middle-aged and male.The mean age of patients with DPHCM was 53.43±12.79 years.Heart failure,dyspnoea,and atrial fibrillation were the most prevalent symptoms observed,accompanied by an average left ventricular end-diastolic size of 58.62 mm.CONCLUSION Our findings corroborate the pathogenicity of the MYH7(c.746G>A,p.Arg249Glu)mutation for DPHCM and suggest that the Arg249Gln mutation may be responsible for high mortality.

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

BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro-gressive sensorimotor polyneuropathy,organ enlargement,endocrine disorders,darkening skin,a monoclonal plasma cell proliferative disorder,and lymph node hyperplasia.The organomegaly consists of hepatosplenomegaly and/or lym-phadenopathy;cases of cardiomyopathy are rare.Diagnoses are often delayed because of the atypical nature of the syndrome,exposing patients to possibly severe disability.Therefore,identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.lenalidomide and dexamethasone.CONCLUSION When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin,the POEMS syndrome is the most possible diagnosis.

Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

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.

Correlation analysis between serum NT-proBNP level and left ventricular remodeling in patients with dilated cardiomyopathy

Objective: To study the correlation between serum N-terminal pro-brain natriuretic peptide (NT-proBNP) content and left ventricular remodeling in patients with dilated cardiomyopathy (DCM). Methods: The patients diagnosed with DCM in our hospital between September 2014 and February 2018 were selected as the DCM group, and volunteers receiving physical examination in our hospital during the same period were selected as the control group. Serum NT-proBNP, N-terminal propeptide of procollagen type I (PINP), C-terminal telopeptide of collagen type I (CITP) and matrix metalloproteinase 1 (MMP1) contents as well as echocardiography parameters left ventricular end-diastolic volume (LVEDV), left ventricular end-systolic volume (LVESV), left ventricular ejection fraction (LVEF) and left ventricular mass index (LVMI) were determined. Results: Serum NT-proBNP, PINP, CITP and MMP1 contents as well as LVMI, LVEDV and LVESV levels of DCM group were significantly higher than those of control group while LVEF level was significantly lower than that of control group;serum NT-proBNP content of DCM group was positively correlated with LVMI, LVEDV and LVESV levels as well as serum PINP, CITP and MMP1 contents, and negatively correlated with LVEF level. Conclusion: The increase of serum NT-proBNP in DCM patients is correlated with left ventricular remodeling and NT-proBNP content can be used to evaluate the degree of left ventricular remodeling.

Experiences of a Dilated Cardiomyopathy Patient Suffering to Maintain Life Partnership Based on Margaret Newman’s Theory

Background: Dilated cardiomyopathy is a refractory myocardial disease with a poor prognosis. Mrs. G experienced cardiopulmonary arrest during hospitalization. She constantly struggles with uncertainty and experiences immense mental suffering from her symptoms, treatments, anxiety, and prognosis. This condition requires appropriate nursing care. Purpose: This study, which is based on Margaret Newman’s theory, aims to clarify the experience of illness of Mrs. G who has dilated cardiomyopathy. Methods: This involved interpretive and dialectical praxis research. The researcher and Mrs. G formed a partnership through discussion. Hermeneutical analysis was performed on the discussion content. Results: The experience of illness of Mrs. G consisted of 6 phases: “Looking back on the shock of having an illness and receiving medical treatment”, “Looking back on one’s life until present”, “Confusion resulting from uncertainty”, “Self-insight”, “Recognizing one’s true feelings of wanting to live”, and “Commitment and decision to live”. Mrs. G could find a new self and expand her consciousness. Conclusion: Mrs. G found meaning in coping with her illness and discovered the value of living while accepting her own destiny. She connected to her own life and became more prepared to live with hope. Thus, partnership in nursing care according to Newman’s theory can benefit patients with distress from dilated cardiomyopathy. This nursing support can improve patient outlook.

Non-invasive evaluation of arrhythmic risk in dilated cardiomyopathy:From imaging to electrocardiographic measures

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.

Contemporary characteristics and outcomes of adults with familial dilated cardiomyopathy listed for heart transplantation

BACKGROUND Familial dilated cardiomyopathy(FDCM) account for 20%-30% of non-ischemic cardiomyopathies(NICM). Previous published data showed that some patients with FDCM tend to have rapidly progressive disease; however, five-year mortality was not significantly different in the familial and non-familial forms of NICM with optimal medical therapy.AIM To better define the characteristics and clinical outcomes of FDCM patients listed for heart transplantation(HT).METHODS We queried the United Network for Organ Sharing Registry to identify FDCM patients listed for HT between January 2008 and September 2015 and compared them to NICM and ischemic cardiomyopathy(ICM) patients. We included all patients ≥ 18 years old and we separated patients to three groups: FDCM, NICM and ICM. Chi-square test was used to compare between categorical variables, the t-test was used to compare between continues variables, and Cox-proportional hazards model was used to perform time-dependent survival analyses.RESULTS Of the 24809 adults listed for HT, we identified 677 patients(2.7%) with the diagnosis of FDCM. Compared to patients with NICM and ICM, FDCM patients were younger(FDCM 43.9 ± 13.5 vs NICM 50.9 ± 12.3, P < 0.001, vs ICM 58.5 ±8.1, P < 0.001), more frequently listed as status 2(FDCM 35.2% vs NICM 26.5%, P< 0.001), with significantly lower left ventricular assist device(LVAD) utilization(FDCM 18.4% vs NICM 25.1%, P < 0.001; vs ICM 25.6%, P < 0.001), but higher use of total artificial heart(FDCM 1.3% vs NICM 0.6%, P = 0.039; vs ICM 0.4%, P =0.002). Additionally, patients with FDCM were less frequently delisted for clinical deterioration or death and more likely to be transplanted compared to those with NICM [hazard ratio(HR): 0.617, 95% confidence interval(CI): 0.47-0.81; HR: 1.25,95%CI: 1.14-1.37, respectively], and ICM(HR: 0.5, 95%CI: 0.38-0.66; HR: 1.18,95%CI: 1.08-1.3, respectively). There was more frequent rejection among patients with FDCM(FDCM 11.4% vs NICM 9.8%, P = 0.28; vs ICM 8.4%, P = 0.034). One,three, and five post-transplant survival of patients with FDCM(91%, 88% and80%) was similar to those with NICM(91%, 84%, 79%, P = 0.225), but superior to those with ICM(89%, 82%, 75%, P = 0.008), respectively.CONCLUSION End-stage FDCM patients are more likely to be transplanted, more likely to have early rejection, and have similar or higher survival than patients with other cardiomyopathies.

D-dimer level and long-term outcome in patients with end-stage heart failure secondary to idiopathic dilated cardiomyopathy

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.

Research Progress of Cardiac Myosin Binding Protein C in Dilated Cardiomyopathy and Other Cardiac Conditions

Since its discovery, myosin-binding protein C (cMyBP-C) has become a protein of interest clinically. With emergence of new methodologies and technologies, the structure and functions of cMyBP-C from different aspects can be studied, enabling us to better understand its involvement in certain cardiac conditions. Studying its kinetics of release and clearance from the circulation and by comparing to other conventional biomarkers, it has been reported that cMyBP-C is eligible to be a novel biomarker for several cardiac conditions. Moreover, studying the genetics and their involvement in pathogenic mechanisms has opened the ideas for potential therapeutic strategies. More and more researches are constantly being done to better understand the role of cMyBP-C in dilated cardiomyopathy (DCM). The importance of cMyBP-C to the heart is still actively being investigated. Its presence is however crucial for sarcomere organization and proper regulation of cardiac contraction during systole and complete relaxation during diastole. Genetic mutation in cMyBP-C has been linked to cardiac conditions including hypertrophic and dilated cardiomyopathies. Around 350 types of mutations have already been documented leading to various cardiac conditions and abnormalities. Analyzing human heart samples has enabled us to better understand the importance of cMyBP-C and how its mutations lead to inherited cardiomyopathies. It is therefore necessary to have an update about the research progress of cMyBP-C in relation to DCM and other cardiac conditions.

Modified Batista Procedure for Idiopathic Dilated Cardiomyopathy: Report of a Case

The surgical indications for dilated cardiomyopathy (DCM) remain controversial, not including cardiac transplantation and mechanical circulatory support. We describe a case of idiopathic DCM that underwent successful surgical treatment using a modified left ventriculectomy, modification of the Batista procedure. The patient was a 63-year-old man who suffered from heart failure, New York Heart Association (NYHA) Class IV. Heart failure was derived from idiopathic DCM with a severely compromised left ventricular function complicated by left ventricular thrombosis. He underwent successful surgical treatment, specifically partial left ventriculectomy combined with the papillary muscle approximation, and the postoperative course was uneventful. He has been well with NYHA Class I for 3 years after the operation without heart failure.

Endoventricular Spiral Plication for Ischemic Dilated Cardiomyopathy

Surgical ventricular restoration (SVR) procedures have been developed;however, their long-term effectiveness remains controversial. Although a series of endoventricular spiral plication (ESP) has been rarely reported and its long prognosis is still unknown;this method has a unique concept of left ventricular (LV) restoration without artificial patch materials. Here, we describe the case of a patient with ischemic cardiomyopathy and ischemic mitral regurgitation who successfully underwent ESP, mitral valve repair, and coronary artery bypass grafting. ESP was effective in papillary muscle approximation for avoiding heart failure;however, the noted improvement of LV wall thickening might be temporary.

Correlation of the electrical remodeling of myocardial cells with oxidative stress and inflammatory injury in animal models with dilated cardiomyopathy

Objective:To study the correlation of the electrical remodeling of myocardial cells with oxidative stress and inflammatory injury in animal models with dilated cardiomyopathy. Methods: Male New Zealand white rabbits were selected as experimental animals and divided into model group and control group, model group were made into animal models with dilated cardiomyopathy through ear intravenous injection of adriamycin, and the control group received ear intravenous injection of same dose of saline. 8 weeks later, the myocardial tissue was collected to detect the myocardial cell delay after depolarization (DADs) through the technique of unicell patch clamp, and radioimmunoprecipitation kits and enzyme-linked immunosorbent assay kits were used to determine oxidative stress indexes and inflammatory reaction indexes.Results:The incidence of myocardial cell DADs of animal model group was significantly higher than that of control group, and ROS, MDA, AOPP, CHOP, GRP78, TLR4, NF-kB, IL-1β, IL-6 and TNF-α levels in myocardial tissue were significantly higher than those of control group;ROS, MDA, AOPP, CHOP, GRP78, TLR4, NF-kB, IL-1β, IL-6 and TNF-α levels in myocardial tissue of DADs animals in model group were significantly higher than those of non-DADs animals.Conclusion:Adriamycin-induced dilated cardiomyopathy can lead to myocardial cell delay after depolarization through oxidative stress and inflammatory injury.

Wilms tumor with dilated cardiomyopathy: A case report

BACKGROUND Wilms tumor is the most common renal malignancy in childhood. It occurs primarily between the ages of 2 and 5 years. The usual manifestations are abdominal mass, hypertension, and hematuria. The case presented here had an unusual presentation, with dilated cardiomyopathy and hypertension secondary to the Wilms tumor. CASE SUMMARY A 3-year-old boy presented with a 5-d history of irritability, poor appetite, and respiratory distress. His presenting clinical symptoms were dyspnea, tachycardia, hypertension, and a palpable abdominal mass at the left upper quadrant. His troponin T and pro-B-type natriuretic peptide levels were elevated. Echocardiography demonstrated a dilated hypokinetic left ventricle with an ejection fraction of 29%, and a suspected left renal mass. Computed tomography scan revealed a left renal mass and multiple lung nodules. The definitive diagnosis of Wilms tumor was confirmed histologically. The patient was administered neoadjuvant chemotherapy and underwent radical nephrectomy. After surgery, radiotherapy was administered, and the adjuvant chemotherapy was continued. The blood pressure and left ventricular function normalized after the treatments. CONCLUSION Abdominal mass, dilated cardiomyopathy and hypertension can indicate Wilms tumor in pediatric patients. Chemotherapy and tumor removal achieve successful treatment.

Dhcr24 activates the PI3K/Akt/HKII pathway and protects against dilated cardiomyopathy in mice

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.

Classification of New Biomarkers of Dilated Cardiomyopathy Based on Pathogenesis—An Update

Dilated Cardiomyopathy (DCM) is a complex heart disease affecting the heart musculature and vasculature, involving one or several underlying pathophysiological mechanisms. Identifying potential biomarkers for dilated cardiomyopathy is a challenge owing to various aetiologies involved. Studying the biomarkers involved in DCM will ultimately give a better insight about which pathophysiological pathways are involved in the onset of the disease. Owing to its multifactorial aetiologies, response to treatment is usually poor. If we can find the exact underlying causes, a better treatment approach could be implemented. One way to obtain better insight of DCM is to study the biomarkers released. Through biomarkers, we can know which underlying mechanisms are involved. Biomarkers can provide us with clinical information such as diagnostic, prognostic, risk stratification as well as response to treatment. Underlying mechanisms such as inflammation, stress/strain, myocyte injury, matrix remodelling, oxidative stress, neurohormones involvement, among others, can contribute to the onset of DCM. Different mechanisms will yield different biomarkers. So it would be wise to classify those biomarkers involving in DCM based on their respective pathogenesis. Moreover, most importantly is to be able to make use of the information that biomarker pertains. However, specificity of those biomarkers poses a problem. One way of making these biomarkers clinically useful is to make use of a biomarker modelling score system.

Bioinformatics Analysis of the Relationship between Dilated Cardiomyopathy and Chronic Heart Failure

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.

Comparison Study on Different Quantification Methods of Diffuse Myocardial Fibrosis of Dilated Cardiomyopathy Using Myocardial T1 Value

Purpose: The purpose is to compare several quantification methods and clarify which quantification method is reliable to estimate diffuse myocardial fibrosis with cardiac MRI in patients with dilated cardiomyopathy (DCM) using myocardial T1 value. Methods and Results: Delayed enhancement imaging was performed in 52 patients with DCM and 10 control subjects to identify fibrosis using an inversion time scout sequence. The mean contrast-enhanced myocardial (M) T1 values of the pre and post contrast-enhanced myocardial and left ventricular lumen (L) of control and dilated cardiomyopathy cases were compared. The calculated post M T1 value, pre M T1 value-post M T1 value, and (pre M TI value-post M T1 value)/(pre L T1 value-post L T1 value) were significantly different between the patient group and the control group (344.5 ± 31.6 vs. 390.4 ± 19.3 msec, 239.9 ± 64.2 msec vs. 134.0 ± 28.9 msec, and 0.50 ± 0.11 vs. 0.30 ± 0.60, respectively). (Pre M T1 value-post M T1 value)/(pre L T1 value-post L T1 value) was significantly the most related to the left ventricular ejection fraction (r = 0.66, p Conclusion: (Pre M T1 value-post M T1 value)/(pre L T1 value-post L T1 value) was the most reliable quantification method to estimate the severity of DCM.

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

Dilated cardiomyopathy(DCM)is a common myocardial disease characterized by enlargement of the heart cavity and decreased systolic function,often leading to heart failure(HF)and arrhythmia.The occurrence of atrial fibrillation(AF)is closely related to the progression and prognosis of the disease.In recent years,with the advancement of medical imaging and biomarkers,models for predicting the occurrence of AF in DCM patients have gradually become a research hotspot.This article aims to review the current situation of AF in DCM patients and explore the importance and possible methods of constructing predictive models to provide reference for clinical prevention and treatment.We comprehensively analyzed the risk factors for AF in DCM patients from epidemiological data,pathophysiological mechanisms,clinical and laboratory indicators,electrocardiogram and imaging parameters,and biomarkers,and evaluated the effectiveness of existing predictive models.Through analysis of existing literature and research,this article proposes a predictive model that integrates multiple parameters to improve the accuracy of predicting AF in DCM patients and provide a scientific basis for personalized treatment.

Clinical outcome of cardiac resynchronization therapy in dilated-phase hypertrophic cardiomyopathy

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.

Impact of Left Bundle Branch Block on Left Ventricular Mechanics in Patients with Idiopathic Dilated Cardiomyopathy

Objectives: Left bundle branch block (LBBB) is commonly associated with heart failure. We evaluated the prevalence and impact of LBBB on left ventricular mechanics using 2D strain imaging in patients with idiopathic dilated cardiomyopathy (IDCM). Methods: We included 101 patients with IDCM with mean age 38 ± 18 years: 74% were males and 13.9% of them were in NYHA Class III-IV. LBBB was present in 26 (37%) of included patients. Myocardial mechanics including longitudinal, circumferential strain and rotation and LV synchronization were assessed using two-dimensional strain imaging. Results: LBBB group had higher LV volumes, and PAP compared with non LBBB. Peak LV longitudinal systolic strain (εsys) of the septum and global LV SRsys were significantly lower in LBBB compared to non LBBB group (P 0.01, 0.03). TTP-d was greater in LBBB in comparison to non LBBB group (274.5 ± 116 versus 209.4 ± 139, P 0.02). The electromechanical delay between septal segments was 35 ± 18 ms and between lateral wall segments: 48 ± 24 ms, between anterior wall segments: 21 ± 11 and between inferior wall segments: 41 ± 12. Consequently, LV mechanical dyssynchrony was more evident in IDCM patients with LBBB. QRS width was correlated inversely with LV longitudinal strain and strain rate and electromechanical delay (P 0.0001) in non LBBB group. In LBBB QRS width was not related to cardiac mechanics. Using univariate analysis and after a multiple covariate adjustment, the baseline LBBB was associated with a significantly increased LV dysfunction. Conclusion:After correcting for potential confounders, LBBB was found to be associated with more deterioration of LV mechanics and exaggerated LV dyssynchrony in patients with IDCM.