24,252 results match your criteria Cardiomyopathy Dilated


Myocarditis: Which Role for Genetics?

Curr Cardiol Rep 2021 May 7;23(6):58. Epub 2021 May 7.

Cardiothoracovascular Department, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI) and University of Trieste, Via Valdoni 7, 34129, Trieste, Italy.

Purpose Of Review: Myocarditis is a polymorphic disease, both in its presentation and clinical course. Recent data suggests that the genetic background, interacting with environmental factors, could be diriment both in the susceptibility and evolution of myocarditis in different clinical presentations. The aim of this paper is to expose the current available evidences and the evolving concepts on this topic, in order to provide insight for improving the clinical management of those patients. Read More

View Article and Full-Text PDF

You cannot ablate the Lernaean Hydra: SCN5A mutation in a patient with multifocal ectopic Purkinje-related premature contractions syndrome treated with Flecainide and an implant of a subcutaneous defibrillator-a case report.

Eur Heart J Case Rep 2021 Apr 30;5(4):ytab158. Epub 2021 Apr 30.

University Hospital of Patras, Rio, Patras, 26504, Greece.

Background: SCN5A mutations may present with different clinical phenotypes such as Brugada syndrome, long QT3 syndrome, sick sinus syndrome, atrial fibrillation, dilated cardiomyopathy, and the least known multifocal ectopic Purkinje-related premature contractions syndrome.

Case Summary: We report a case of a 29-year-old woman with palpitations due to multifocal premature ventricular complexes (PVCs) and a family history of sudden death. The previous electrophysiological study had shown that PVCs arose from Purkinje fibres but catheter ablation was unsuccessful. Read More

View Article and Full-Text PDF

Middle Meningeal Artery Embolization of a Pediatric Patient With Progressive Chronic Subdural Hematoma.

Oper Neurosurg (Hagerstown) 2021 May 6. Epub 2021 May 6.

Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York, USA.

Background And Importance: Evidence suggests middle meningeal artery (MMA) embolization benefits adult patients with chronic subdural hematoma (CSDH) at high risk for recurrence or hemorrhagic complications. Yet, there has not been any report discussing MMA embolization in the pediatric population. Thus, we present a case of an infant with CSDH successfully managed with MMA embolization without surgical management. Read More

View Article and Full-Text PDF

Persistent Cardiac Magnetic Resonance Imaging Features of Myocarditis Detected Months After COVID-19 Infection.

Cureus 2021 Apr 1;13(4):e14250. Epub 2021 Apr 1.

Department of Cardiology, McLaren Health Care, Flint/Michigan State University, Flint, USA.

Acute myocarditis is commonly caused by viral infections resulting from viruses such as adenovirus, enteroviruses, and, rarely, coronavirus. It presents with nonspecific symptoms like chest pain, dyspnea, palpitation, or arrhythmias and can progress to dilated cardiomyopathy or heart failure. Fulminant myocarditis is a potentially life-threatening form of the condition and presents as acute, severe heart failure with cardiogenic shock. Read More

View Article and Full-Text PDF

Experience of cardiac implantable electronic device lead removal from a South African tertiary referral centre.

Cardiovasc J Afr 2021 Apr 19;32:1-5. Epub 2021 Apr 19.

Division of Cardiology, Department of Medicine, Faculty of Health Sciences, University of Cape Town; Cardiac Clinic, Groote Schuur Hospital, Cape Town, South Africa.

Background: The rate of cardiac implantable electronic device (CIED) implantation in low- and middle-income countries is increasing. Patients recieving these devices are frequently older and with multiple co-morbidities, which may later lead to complications requiring CIED removal. CIED removals are associated with life-threatening complications. Read More

View Article and Full-Text PDF

Childhood onset nexilin dilated cardiomyopathy: A heterozygous and a homozygous case.

Am J Med Genet A 2021 May 5. Epub 2021 May 5.

Department of Clinical Genetics, Amsterdam University Medical Centers, Amsterdam, Netherlands.

Pathogenic heterozygous NEXN variants are associated with progressive dilated cardiomyopathy (DCM) usually presenting around 50 years of age. We describe an asymptomatic boy who had transient DCM at 3 months of age, that resolved by 4 months. Presently, at 11 years of age, he has normal cardiac function with signs of mild DCM on cardiac MRI. Read More

View Article and Full-Text PDF

Phenotypic Recapitulation and Correction of Desmoglein-2-deficient Cardiomyopathy using Human Induced Pluripotent Stem Cell-derived Cardiomyocytes.

Hum Mol Genet 2021 May 5. Epub 2021 May 5.

Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Suita, Osaka 565-0871, Japan.

Desmoglein-2, encoded by DSG2, is one of the desmosome proteins that maintains the structural integrity of tissues, including heart. Genetic mutations in DSG2 cause arrhythmogenic cardiomyopathy, mainly in an autosomal dominant manner. Here, we identified a homozygous stop-gain mutations in DSG2 (c. Read More

View Article and Full-Text PDF

Young and early-onset dilated cardiomyopathy with malignant ventricular arrhythmia and sudden cardiac death induced by the heterozygous LDB3, MYH6, and SYNE1 missense mutations.

Ann Noninvasive Electrocardiol 2021 May 5:e12840. Epub 2021 May 5.

Department of Cardiology, The Cardiovascular Center, Interventional Medical Center, Guangdong Provincial Key Laboratory of Biomedical Imaging and Guangdong Provincial Engineering Research Center of Molecular Imaging, The Fifth Affiliated Hospital, Sun Yat-sen University, Zhuhai, China.

Background: The whole exome sequencing (WES) with targeted gene analysis is an effective diagnostic tool for cardiomyopathy. The early-onset sudden cardiac death (SCD) was commonly associated with dilated cardiomyopathy (DCM) induced by pathogenic genetic mutations.

Methods: In a Chinese Han family, the patient of 24 years old occurred with early-onset and DCM and died of SCD associated with ICD storms induced by repetitive ventricular tachycardia/fibrillation (VT/F). Read More

View Article and Full-Text PDF

An Evidence-Based Assessment of Genes in Dilated Cardiomyopathy.

Circulation 2021 May 5. Epub 2021 May 5.

Department of Genetics, University Medical Center Utrecht, University of Utrecht, Utrecht, the Netherlands.

The cardiomyopathies, classically categorized as hypertrophic (HCM), dilated (DCM), and arrhythmogenic right ventricular (ARVC), each have a signature genetic theme. HCM and ARVC are largely understood as genetic diseases of sarcomere or desmosome proteins, respectively. In contrast, >250 genes spanning more than 10 gene ontologies have been implicated in DCM, representing a complex and diverse genetic architecture. Read More

View Article and Full-Text PDF

Nucleoside Analogue Reverse Transcriptase Inhibitors Improve Clinical Outcome in Transcriptional Active Human Parvovirus B19-Positive Patients.

J Clin Med 2021 Apr 29;10(9). Epub 2021 Apr 29.

Institute of Cardiac Diagnostics and Therapy, 12203 Berlin, Germany.

Human parvovirus B19 (B19V) is the predominant cardiotropic virus associated with dilated inflammatory cardiomyopathy (DCMi). Transcriptionally active cardiotropic B19V infection is clinically relevant and triggers adverse long-term mortality. During the study; we evaluated whether antiviral treatment with the nucleoside analogue telbivudine (LTD) is effective in suppressing transcriptional active B19V in endomyocardial biopsies (EMBs) of B19V positive patients and improving clinical outcomes. Read More

View Article and Full-Text PDF

Clinical, biochemical, and echocardiographic evaluation of neonates with vitamin D deficiency due to maternal vitamin D deficiency.

Cardiol Young 2021 May 4:1-6. Epub 2021 May 4.

Faculty of Medicine, Department of Paediatric Endocrinology, Hacettepe University, Sıhhiye/Ankara, Turkey.

Objective: There are a few number of case reports and small-scale case series reporting dilated cardiomyopathy due to vitamin D-deficient rickets. The present study evaluates the clinical, biochemical, and echocardiographic features of neonates with vitamin D deficiency.

Patients And Methods: In this prospective single-arm observational study, echocardiographic evaluation was performed on all patients before vitamin D3 and calcium replacement. Read More

View Article and Full-Text PDF

Genetic analysis using targeted next-generation sequencing of sporadic Chinese patients with idiopathic dilated cardiomyopathy.

J Transl Med 2021 May 3;19(1):189. Epub 2021 May 3.

Department of Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of Coronary Heart Disease Prevention, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.

Background: Inherited dilated cardiomyopathy (DCM) contributes to approximately 25% of idiopathic DCM cases, and the proportion is even higher in familial DCM patients. Most studies have focused on familial DCM, whereas the genetic profile of sporadic DCM in Chinese patients remains unknown.

Methods: Between June 2018 and September 2019, 24 patients diagnosed with idiopathic DCM without a family history were included in the present study. Read More

View Article and Full-Text PDF

Integrated regulatory network based on lncRNA-miRNA-mRNA-TF reveals key genes and sub-networks associated with dilated cardiomyopathy.

Comput Biol Chem 2021 Apr 25;92:107500. Epub 2021 Apr 25.

Data Mining and Text Mining Laboratory, Department of Bioinformatics, Bharathiar University, Coimbatore, Tamilnadu, India. Electronic address:

Dilated Cardiomyopathy (DCM) is a multifactorial condition often leading to heart failure in many clinical cases. Due to the high number of DCMincidence reported as familial, a gene level network based study was conducted utilizing high throughput next generation sequencing data. We exploited the exome and transcriptome sequencing data in NCBI-SRA database to construct a high confidence scale-free regulatory network consisting of lncRNA, miRNA, mRNA and Transcription Factors (TFs). Read More

View Article and Full-Text PDF

The Genetic Pathways Underlying Immunotherapy in Dilated Cardiomyopathy.

Front Cardiovasc Med 2021 14;8:613295. Epub 2021 Apr 14.

Division of Genomics and Translational Biomedicine, College of Health and Life Sciences, Hamad Bin Khalifa University, Doha, Qatar.

Heart failure (HF) is a global public health threat affecting 26 million individuals worldwide with an estimated prevalence increase of 46% by 2030. One of the main causes of HF and sudden death in children and adult is Dilated Cardiomyopathy (DCM). DCM is characterized by dilation and systolic dysfunction of one or both ventricles. Read More

View Article and Full-Text PDF

Identification and Functional Prediction of Long Non-Coding RNAs in Dilated Cardiomyopathy by Bioinformatics Analysis.

Front Genet 2021 16;12:648111. Epub 2021 Apr 16.

The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.

Dilated cardiomyopathy (DCM) is a relatively common cause of heart failure and the leading cause of heart transplantation. Aberrant changes in long non-coding RNAs (lncRNAs) are involved in DCM disorder; however, the detailed mechanisms underlying DCM initiation and progression require further investigation, and new molecular targets are needed. Here, we obtained lncRNA-expression profiles associated with DCM and non-failing hearts through microarray probe-sequence re-annotation. Read More

View Article and Full-Text PDF

Is Occult Genetic Substrate the Missing Link Between Arrhythmic Mitral Annular Disjunction Syndrome and Sudden Cardiac Death?

Can J Cardiol 2021 Apr 29. Epub 2021 Apr 29.

Department of Neuroscience, Imaging and Clinical Sciences, "G.d'Annunzio" University of Chieti-Pescara, Italy. Electronic address:

We present the case of a 28-year-old man with a history of unexplained syncope, frequent ventricular arrhythmias, familial LMNA-related dilated cardiomyopathy (DCM) and mitral annular disjunction (MAD). We provide the first association of a novel truncating LMNA variant serving as a potential vulnerable substrate for arrhythmogenic MAD syndrome. This could suggest a possible synergistic role between concealed genetic variants (resulting in fibrosis as a 'substrate' for arrhythmogenesis) and the presence of mitral annular disjunction (the 'trigger' with mechanical stretch initiating ventricular arrhythmias), which may provide a link between mitral valve prolapse and sudden cardiac death. Read More

View Article and Full-Text PDF

Wearable cardioverter-defibrillator: friend or foe in suspected myocarditis?

ESC Heart Fail 2021 May 1. Epub 2021 May 1.

Department of Internal Medicine and Cardiology, Charité - Universitätsmedizin Berlin, Campus Virchow-Klinikum, Augustenburger Platz 1, Berlin, 13353, Germany.

Aim: Wearable cardioverter defibrillator (WCD, LifeVest, and Zoll) therapy has become a useful tool to bridge a temporarily increased risk for sudden cardiac death. However, despite extensive use, there is a lack of evidence whether patients with myocarditis and impaired LVEF may benefit from treatment with a WCD.

Methods And Results: We conducted a single-centre retrospective observational study analysing patients with a WCD prescribed between September 2015 and April 2020 at our institution. Read More

View Article and Full-Text PDF

Parvovirus B19 DNA detectable in hearts of patients with dilated cardiomyopathy, but absent or inactive in blood.

ESC Heart Fail 2021 May 1. Epub 2021 May 1.

Department of Blood-borne Infections, Donor Medicine Research, Sanquin Blood Supply Foundation, Amsterdam, The Netherlands.

Aims: Parvovirus B19 (B19V) is often assumed to be a cause of dilated cardiomyopathy (DCM), based on the quantification of B19V DNA in endomyocardial biopsies (EMB). Whether the presence of B19V DNA correlates with active infection is still debated. Application of the enzyme endonuclease to blood samples results in degradation of B19V DNA remnants but leaves viral particles intact, which enables differentiation between active and past infection. Read More

View Article and Full-Text PDF

The Unfolded Protein Response as a Compensatory Mechanism and Potential Therapeutic Target in PLN R14del Cardiomyopathy.

Circulation 2021 Apr 30. Epub 2021 Apr 30.

Cardiovascular Institute, Stanford University, Stanford, CA; Department of Cardiothoracic Surgery, Stanford University School of Medicine, Stanford, CA.

Phospholamban (PLN) is a critical regulator of calcium cycling and contractility in the heart. The loss of arginine at position 14 in PLN (R14del) is associated with dilated cardiomyopathy (DCM) with a high prevalence of ventricular arrhythmias. How the R14 deletion causes DCM is poorly understood and there are no disease-specific therapies. Read More

View Article and Full-Text PDF

The combination of PICP blood levels and LGE at CMR provides additional prognostic information in idiopathic Dilated Cardiomyopathy.

Eur J Heart Fail 2021 Apr 30. Epub 2021 Apr 30.

Department of Cardiology, Cardiovascular Research Institute Maastricht (CARIM), Maastricht University Medical Centre, Maastricht, The Netherlands.

Aims: To determine the prognostic value of multilevel assessment of fibrosis in DCM patients.

Methods And Results: We quantified fibrosis in 209 DCM patients at three levels: i. non-invasive late-gadolinium enhancement (LGE) at cardiovascular magnetic resonance (CMR); ii. Read More

View Article and Full-Text PDF

Clinical Identification and Characteristic Analysis of Giant Cell Myocarditis in 12 Cases.

Front Cardiovasc Med 2021 13;8:649094. Epub 2021 Apr 13.

State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Giant cell myocarditis (GCM) is a rare, rapidly progressing cardiomyopathy with high mortality, if not diagnosed and treated in time. We analyzed the progression and clinical manifestations of patients with definitive diagnosis of GCM. We enrolled 12 patients diagnosed with GCM in the explanted heart during heart transplantation (HTx) or by endomyocardial biopsy (EMB) and collected information on demographic data, cardiac structure and function, arrhythmias, preliminary diagnosis, and delay of the diagnosis. Read More

View Article and Full-Text PDF

Skeletal and Cardiac Muscle Disorders Caused by Mutations in Genes Encoding Intermediate Filament Proteins.

Int J Mol Sci 2021 Apr 20;22(8). Epub 2021 Apr 20.

CNR Institute of Molecular Genetics "Luigi Luca Cavalli-Sforza", Unit of Bologna, 40136 Bologna, Italy.

Intermediate filaments are major components of the cytoskeleton. Desmin and synemin, cytoplasmic intermediate filament proteins and A-type lamins, nuclear intermediate filament proteins, play key roles in skeletal and cardiac muscle. Desmin, encoded by the gene (OMIM *125660) and A-type lamins by the gene (OMIM *150330), have been involved in striated muscle disorders. Read More

View Article and Full-Text PDF

Update on the Diagnostic Pitfalls of Autopsy and Post-Mortem Genetic Testing in Cardiomyopathies.

Int J Mol Sci 2021 Apr 16;22(8). Epub 2021 Apr 16.

Department of Health Surveillance and Bioethics, Section of Legal Medicine, Fondazione Policlinico A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.

Inherited cardiomyopathies are frequent causes of sudden cardiac death (SCD), especially in young patients. Despite at the autopsy they usually have distinctive microscopic and/or macroscopic diagnostic features, their phenotypes may be mild or ambiguous, possibly leading to misdiagnoses or missed diagnoses. In this review, the main differential diagnoses of hypertrophic cardiomyopathy (e. Read More

View Article and Full-Text PDF

Beneficial Effect of Left Ventricular Remodeling after Early Change of Sacubitril/Valsartan in Patients with Nonischemic Dilated Cardiomyopathy.

Medicina (Kaunas) 2021 Apr 25;57(5). Epub 2021 Apr 25.

Division of Cardiology, Heart Stroke Vascular Center, Incheon Sejong Hospital, 20, Gyeyangmunhwa-ro, Gyeyang-gu, Incheon 21080, Korea.

Evidence for effectiveness of early change from angiotensin II receptor blockers (ARBs) or angiotensin-converting enzyme inhibitors (ACEIs) to sacubitril/valsartan is lacking. We aimed to investigate whether early changes to sacubitril/valsartan could improve outcomes in patients with nonischemic dilated cardiomyopathy (DCM) in real-world practice. : A total of 296 patients with nonischemic DCM who were treated with ARB or ACEI continuously (group A, = 150) or had their medication switched to sacubitril/valsartan (group S, = 146) were included. Read More

View Article and Full-Text PDF

A Low-Sodium Diet Boosts Ang (1-7) Production and NO-cGMP Bioavailability to Reduce Edema and Enhance Survival in Experimental Heart Failure.

Int J Mol Sci 2021 Apr 14;22(8). Epub 2021 Apr 14.

Department of Medicine, University of Arizona College of Medicine-Phoenix, Phoenix, AZ 85004, USA.

Sodium restriction is often recommended in heart failure (HF) to block symptomatic edema, despite limited evidence for benefit. However, a low-sodium diet (LSD) activates the classical renin-angiotensin-aldosterone system (RAAS), which may adversely affect HF progression and mortality in patients with dilated cardiomyopathy (DCM). We performed a randomized, blinded pre-clinical trial to compare the effects of a normal (human-equivalent) sodium diet and a LSD on HF progression in a normotensive model of DCM in mice that has translational relevance to human HF. Read More

View Article and Full-Text PDF

Role of in the Emery-Dreifuss Muscular Dystrophy Cardiac Phenotype.

Biomolecules 2021 Apr 6;11(4). Epub 2021 Apr 6.

Istituto Nazionale di Genetica Molecolare Romeo ed Enrica Invernizzi, 20122 Milan, Italy.

The locus is one of the most studied tumor suppressor loci in the context of several cancer types. However, in the last years, its expression has also been linked to terminal differentiation and the activation of the senescence program in different cellular subtypes. Knock-out (KO) of the entire locus enhances the capability of stem cells to proliferate in some tissues and respond to severe physiological and non-physiological damages in different organs, including the heart. Read More

View Article and Full-Text PDF

CD24 for Cardiovascular Researchers: A Key Molecule in Cardiac Immunology, Marker of Stem Cells and Target for Drug Development.

J Pers Med 2021 Apr 1;11(4). Epub 2021 Apr 1.

Division of Pediatric Cardiology, Seattle Children's Hospital and the University of Washington School of Medicine, Seattle, WA 98105, USA.

The study of the membrane protein, CD24, and its emerging role in major disease processes, has made a huge leap forward in the past two decades. It appears to have various key roles in oncogenesis, tumor progression and metastasis, stem cell maintenance and immune modulation. First described in the 1980s as the homologous human protein to the mouse HSA (Heat Stable Antigen), it was reported as a surface marker in developing hematopoietic cell lines. Read More

View Article and Full-Text PDF

The Role of Speckle Tracking Echocardiography in the Evaluation of Common Inherited Cardiomyopathies in Children and Adolescents: A Systematic Review.

Diagnostics (Basel) 2021 Apr 1;11(4). Epub 2021 Apr 1.

Population Health Sciences Department, University of Bristol, Bristol BS8 2PS, UK.

Speckle tracking echocardiography (STE) has gained importance in the evaluation of adult inherited cardiomyopathies, but its utility in children is not well characterized. We conducted a systematic review to evaluate the role of STE in pediatric inherited cardiomyopathies. PubMed, EMBASE, Web of Science, Scopus, CENTRAL and CINAHL databases were searched up to May 2020, for terms related to inherited cardiomyopathies and STE. Read More

View Article and Full-Text PDF

Performance, clinical effectiveness, and safety of immunoadsorption in a wide range of indications.

Ther Apher Dial 2021 Apr 29. Epub 2021 Apr 29.

Fresenius Medical Care, Global Medical Office, Bad Homburg, Germany.

Immunoadsorption is well known to selectively remove immunoglobulins and immune complexes from plasma and is applied in a variety of autoimmune diseases and for desensitization before, or at acute rejection after organ transplantation. Performance, safety, and clinical effectiveness of immunoadsorption were the aim of this study. This prospective, noninterventional, multicentre cohort study included patients treated with immunoadsorption (Immunosorba or GLOBAFFIN adsorbers) for any indication. Read More

View Article and Full-Text PDF

Crohn's disease with dilated cardiomyopathy in a child: An experience with heart transplantation.

Pediatr Transplant 2021 Apr 28:e14027. Epub 2021 Apr 28.

Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Michigan, Detroit, MI, USA.

Background: DCM has rarely been reported in children with CD, but is a recognized complication in adults. DCM with uncontrolled heart failure may need heart transplantation, which could be challenging in CD.

Case Description: We present a 9-year-old female with CD who was diagnosed with DCM and a measured Ejection fraction ~25. Read More

View Article and Full-Text PDF