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International Journal of Cardiology Mar 2024Gene therapy is a technique to correct genetic abnormalities, through introduction of a functional gene or through direct genome editing. Adeno-associated virus... (Review)
Review
Gene therapy is a technique to correct genetic abnormalities, through introduction of a functional gene or through direct genome editing. Adeno-associated virus (AAV)-mediated gene replacement shows promise for targeted therapies in treatment of inherited cardiomyopathies and is the most used approach in clinical trials. However, immune responses from the host to the virus and gene product pose delivery and safety challenges. This review explores the immunological reactions to AAV-based gene therapy, their potential toxic effects, with a focus on myocarditis, and future directions for gene therapy.
Topics: Humans; Myocarditis; Genetic Therapy; Genetic Vectors; Dependovirus
PubMed: 38030043
DOI: 10.1016/j.ijcard.2023.131617 -
Journal of Cardiology Mar 2024Although most cases of myocarditis are self-limiting with a gradual improvement in cardiac function, the involvement of myocarditis in sudden cardiac death among... (Review)
Review
Although most cases of myocarditis are self-limiting with a gradual improvement in cardiac function, the involvement of myocarditis in sudden cardiac death among children and young adults remains substantial, with rates of 3-17 % and 8.6-12 %, respectively. Moreover, the risk of developing chronic dilated cardiomyopathy ranges from 21 % to 30 % in all cases confirmed by biopsy. Current therapeutic strategies for myocarditis and its complications range from standard supportive care for heart failure and arrhythmias to etiologically oriented, case-based therapeutic options. For example, immunosuppression is indicated only in certain forms of acute myocarditis with clinical or endomyocardial biopsy evidence of immune checkpoint inhibitor-induced myocarditis and autoimmune diseases, including giant cell myocarditis, eosinophilic myocarditis, vasculitis, or cardiac sarcoidosis. However, our views on myocarditis treatment have changed considerably over the past two decades, thanks to the emergence of regenerative cells/tissues as well as drug and gene delivery systems. Cell-based therapies are now growing in popularity in any field of medicine. Studies evaluating the therapeutic efficacy of different stem cells in the treatment of acute myocarditis and its chronic complications have shown that although the experimental characteristics varied from study to study, in general, these strategies reduced inflammation and myocardial fibrosis while preventing myocarditis-induced systolic dysfunction and adverse remodeling in animal models.
Topics: Child; Animals; Humans; Myocarditis; Myocardium; Cardiomyopathies; Cardiomyopathy, Dilated; Inflammation; Biopsy
PubMed: 37597837
DOI: 10.1016/j.jjcc.2023.08.009 -
Cardiology Clinics Aug 2023Systemic diseases can cause heart block owing to the involvement of the myocardium and thereby the conduction system. Younger patients (<60) with heart block should be... (Review)
Review
Systemic diseases can cause heart block owing to the involvement of the myocardium and thereby the conduction system. Younger patients (<60) with heart block should be evaluated for an underlying systemic disease. These disorders are classified into infiltrative, rheumatologic, endocrine, and hereditary neuromuscular degenerative diseases. Cardiac amyloidosis owing to amyloid fibrils and cardiac sarcoidosis owing to noncaseating granulomas can infiltrate the conduction system leading to heart block. Accelerated atherosclerosis, vasculitis, myocarditis, and interstitial inflammation contribute to heart block in rheumatologic disorders. Myotonic, Becker, and Duchenne muscular dystrophies are neuromuscular diseases involving the myocardium skeletal muscles and can cause heart block.
Topics: Humans; Muscular Dystrophies; Arrhythmias, Cardiac; Heart Block; Myocardium; Myocarditis; Arthritis, Rheumatoid; Heart Diseases
PubMed: 37321693
DOI: 10.1016/j.ccl.2023.03.008 -
Myocarditis in Patients With Idiopathic Inflammatory Myopathies: Clinical Presentation and Outcomes.The Journal of Rheumatology Aug 2023To determine the clinical phenotype and outcomes of patients with idiopathic inflammatory myopathies (IIMs) and myocarditis.
OBJECTIVE
To determine the clinical phenotype and outcomes of patients with idiopathic inflammatory myopathies (IIMs) and myocarditis.
METHODS
Using the Johns Hopkins Myositis Center Research Registry, we identified 31 adult patients with IIM-out of a total of 3082 with confirmed or suspected muscle disease-with an encounter code of myocarditis from 2004 to 2021. Of these, 14 adult patients with IIM were adjudicated to have clinical myocarditis. Information about demographics, autoantibodies, and clinical outcomes was retrospectively collected and analyzed.
RESULTS
Of 14 patients with IIM with clinical myocarditis, the median age at IIM diagnosis was 49 (IQR 35-56) years, and the median age at myocarditis diagnosis was 54 (IQR 36-61) years. The median duration between IIM diagnosis and myocarditis was 3 (IQR 2-9) years. The majority of patients were female (8/14, 57%) and Black (10/14, 71%). Antisynthetase syndrome was the most common IIM subtype (9/14, 64%). Anti-Jo1 (n = 4) and anti-PL12 (n = 3) were the most frequent autoantibodies. At myocarditis diagnosis, most patients (11/14, 79%) had active myositis, defined as elevated creatine kinase and/or muscle weakness; required hospitalization (13/14, 93%); and had reduced left ventricular ejection fraction (LVEF < 50%; 10/14, 71%). Despite intensification of immunosuppression, the 5-year overall survival rate from IIM diagnosis was 84%, and the 5-year overall survival rate from myocarditis diagnosis was 53%. Systolic dysfunction (LVEF < 40%) at final evaluation was observed in all expired patients (n = 6).
CONCLUSION
Clinical presentations of myocarditis in this select cohort of patients with IIM were severe and heterogeneous with poor outcomes despite intensification of immunosuppression, potentially reflecting late detection of myocarditis.
Topics: Humans; Male; Female; Retrospective Studies; Myocarditis; Stroke Volume; Ventricular Function, Left; Myositis; Autoantibodies
PubMed: 37003604
DOI: 10.3899/jrheum.220989 -
Emergency Medicine Practice Jul 2023Pericarditis and myocarditis represent a challenging set of diseases to diagnose and treat. These diseases typically present with chest pain and dyspnea in previously... (Review)
Review
Pericarditis and myocarditis represent a challenging set of diseases to diagnose and treat. These diseases typically present with chest pain and dyspnea in previously healthy young people, often in the weeks following a viral illness, including COVID-19. Nonetheless, the etiologies can be very diverse, including infectious, noninfectious, drug-induced, and autoimmune causes. This review focuses on the evaluation, diagnosis, and management of emergency department patients presenting with pericarditis and myocarditis and summarizes current guidelines and best-practice medical management strategies in order to avoid potential life-threatening cardiac complications.
Topics: Humans; Adolescent; Myocarditis; COVID-19; Pericarditis; Emergency Service, Hospital; Diagnosis, Differential; COVID-19 Testing
PubMed: 37352407
DOI: No ID Found -
European Journal of Pediatrics Nov 2023Coronavirus disease 2019 in children presents with distinct phenotype in comparison to adults. Overall, the pediatric infection with a generally milder clinical course... (Review)
Review
Coronavirus disease 2019 in children presents with distinct phenotype in comparison to adults. Overall, the pediatric infection with a generally milder clinical course of the acute infection compared to adults still faces several unknown aspects. Specifically, the presence of a wide range of inflammatory manifestations, including multisystem inflammatory syndrome in children (MIS-C), myocarditis, and long COVID in the period after infection suggests a particular susceptibility of some children upon severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Albeit peculiar complications such as long covid are less frequent in children compared to adults, research on the relationship between inflammatory syndromes and SARS-CoV-2 is rapidly evolving. Conclusions: new studies and findings continue to emerge, providing further insights into the underlying mechanisms and potential therapeutic strategies. In the present work, we revised current knowledge of the main factors accounting for such variability upon SARS-CoV-2 infection over the pediatric age group. What is Known: • COVID19 in children overall showed a milder course compared to adults during the acute phase of the infection. • Children showed to be susceptible to a wide range of post infectious complications including multisystem inflammatory syndrome in children (MIS-C), myocarditis, neuroinflammation, and long COVID. What is New: • Mechanisms underlying susceptibility to a severe course of the infection were recently shown to pertain to the host. • A specific combination of HLA was recently shown to be associated to higher susceptibility to MIS-C in children.
Topics: Adult; Child; Humans; COVID-19; Post-Acute COVID-19 Syndrome; RNA, Viral; SARS-CoV-2; Myocarditis
PubMed: 37702769
DOI: 10.1007/s00431-023-05184-w -
JAMA Aug 2023
Topics: Humans; Myocarditis; Diagnosis, Differential; Acute Disease
PubMed: 37552301
DOI: 10.1001/jama.2023.5526 -
Hellenic Journal of Cardiology : HJC =... 2023Paediatric cardiomyopathies form a heterogeneous group of disorders characterized by structural and electrical abnormalities of the heart muscle, commonly due to a gene... (Review)
Review
Paediatric cardiomyopathies form a heterogeneous group of disorders characterized by structural and electrical abnormalities of the heart muscle, commonly due to a gene variant of the myocardial cell structure. Mostly inherited as a dominant or occasionally recessive trait, they might be part of a syndromic disorder of underlying metabolic or neuromuscular defects or combine early developing extracardiac abnormalities (i.e., Naxos disease). The annual incidence of 1 per 100,000 children appears higher during the first two years of life. Dilated and hypertrophic cardiomyopathy phenotypes share an incidence of 60% and 25%, respectively. Arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy, and left ventricular noncompaction are less commonly diagnosed. Adverse events such as severe heart failure, heart transplantation, or death usually appear early after the initial presentation. In ARVC patients, high-intensity aerobic exercise has been associated with worse clinical outcomes and increased penetrance in at-risk genotype-positive relatives. Acute myocarditis in children has an incidence of 1.4-2.1 cases/per 100,000 children per year, with a 6-14% mortality rate during the acute phase. A genetic defect is considered responsible for the progression to dilated cardiomyopathy phenotype. Similarly, a dilated or arrhythmogenic cardiomyopathy phenotype might emerge with an episode of acute myocarditis in childhood or adolescence. This review provides an overview of childhood cardiomyopathies focusing on clinical presentation, outcome, and pathology.
Topics: Adolescent; Humans; Child; Myocarditis; Cardiomyopathies; Myocardium; Arrhythmogenic Right Ventricular Dysplasia; Phenotype
PubMed: 36870438
DOI: 10.1016/j.hjc.2023.02.007 -
Physical Medicine and Rehabilitation... Aug 2023Cardiovascular complications associated with the severe acute respiratory syndrome coronavirus 2 infection are common and lead to high mortality in the acute phase and... (Review)
Review
Cardiovascular complications associated with the severe acute respiratory syndrome coronavirus 2 infection are common and lead to high mortality in the acute phase and high morbidity in the chronic phase impacting an individual's quality of life and health outcomes. Patients afflicted with coronavirus disease-2019 (COVID-19) infection display an increased risk for myocarditis, dysrhythmia, pericarditis, ischemic heart disease, heart failure, and thromboembolism. Although cardiovascular complications are reported across all patients with COVID-19, hospitalized patients with severe infection are most vulnerable. The underline pathobiology remains poorly defined albeit complex. Following current guidelines in decision-making for evaluation and management in addition to the beginning or returning exercise is recommended.
Topics: Humans; COVID-19; SARS-CoV-2; Quality of Life; Myocarditis; Cardiovascular Diseases
PubMed: 37419531
DOI: 10.1016/j.pmr.2023.03.003 -
Clinical Cardiology Jul 2023
Topics: Humans; Antipyretics; Myocarditis
PubMed: 37249254
DOI: 10.1002/clc.24026