Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden cardiac death in young people. Implantable cardioverter defibrillator (ICD) is the optimal therapy in patients with HCM, both for primary or secondary prevention of sudden death. Left ventricular systolic function in HCM is usually normal. However, in few patients, HCM has been reported to progress to a state that is character...

Background Cardiovascular magnetic resonance (CMR) imaging is the gold standard imaging modality for characterization of dilated cardiomyopathy (DCM). We recently identified genetic variants that truncate the giant protein Titin (TTN) accounting for up to 25% of DCM cases: a discovery that will increase the utility of genetic testing and family screening in DCM. However, the clinical consequenc...

Dilated cardiomyopathy (DCM) is a highly prevalent and often lethal disease in Irish wolfhounds. Complex segregation analysis indicated different loci involved in pathogenesis. Linear fixed and mixed models were used for the genome-wide association study. Using 106 DCM cases and 84 controls we identified one SNP significantly associated with DCM on CFA37 and five SNPs suggestively associated wi...

Background The underlying myocardial injury in early forms of dilated cardiomyopathy (DCM) is unclear. Cardiovascular magnetic resonance (CMR) myocardial T1-mapping monitors the enlargement of the cardiac extra-cellular space and thus offers a non-invasive tool of quantitative measurement of myocardial fibrosis. We used T1mapping techniques to explore the hypothesis that diffuse myocardial fibr...

Background Interstitial myocardial fibrosis is a histological hallmark of non-ischemic dilated cardiomyopathy (DCM), and may play an important role in adverse remodelling and progressive systolic dysfunction. T1-mapping enables noninvasive assessment of diffuse fibrosis by quantification of myocardial extracellular volume fraction (ECV). We hypothesized that CMR would identify a raised ECV in D...

Background Severe right ventricular dysfunction (RVD) is an important prognostic parameter in dilated cardiomyopathy (DCM). The relationship between myocardial fibrosis of the left ventricle (LV) and particularly the septum and right ventricular (RV) function remains poorly understood. We sought to investigate this relationship in a large cohort of DCM patients using cardiovascular magnetic res...

We have shown cardiac protection by metallothionein (MT) in the development of diabetic cardiomyopathy (DCM) via suppression of cardiac cell death in cardiac-specific MT-overexpressing transgenic (MT-TG) mice. The present study was undertaken to define whether diabetes can induce cardiac endoplasmic reticulum (ER) stress and whether MT can prevent cardiac cell death via attenuating ER stress. D...

An integrative analysis of miRNA and mRNA expression profiles in left ventricle (LV) of diabetes-induced rats was performed to elucidate the role of miRNAs and their mRNAs target in diabetic cardiomyopathy (DCM). mRNA (GSE4745) and miRNA (GSE44179) datasets were downloaded from Gene Expression Omnibus 2R (GEO2R) and differentially expressed mRNAs and miRNAs were selected. Cardiotoxicity-related...

β-adrenergic signaling pathways mediate key aspects of cardiac function. Its dysregulation is associated with a range of cardiac diseases, including dilated cardiomyopathy (DCM). Previously, we established an iPSC model of familial DCM from patients with a mutation in TNNT2, a sarcomeric protein. Here, we found that the β-adrenergic agonist isoproterenol induced mature β-adrenergic signaling in...

Peripartum cardiomyopathy (PPCM) is a severe cardiac disease occurring in the last month of pregnancy or in the first 5 months after delivery and shows many similar clinical characteristics as dilated cardiomyopathy (DCM) such as ventricle dilation and systolic dysfunction. While PPCM was believed to be DCM triggered by pregnancy, more and more studies show important differences between these d...