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Related Concept Videos

Myocarditis I: Introduction01:21

Myocarditis I: Introduction

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Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
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Rheumatic Heart Disease I: Introduction01:23

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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Myocarditis II: Clinical Features and Diagnostic Tests01:27

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Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
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Cardiomyopathy I: Introduction and Classification01:25

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Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
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Myocarditis III: Medical Management01:14

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Myocarditis: Comprehensive Medical ManagementMyocarditis, the heart muscle inflammation, requires a comprehensive medical management strategy that addresses the underlying cause, provides supportive care, manages symptoms, and reduces cardiac workload.Infections and Autoimmune CausesAdminister appropriate antimicrobial therapy when an infectious agent causes myocarditis. For instance, penicillin treats infections caused by Group A Streptococcus. In cases where autoimmune processes are...
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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

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Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Flow Cytometry-Based Quantification and Analysis of Myocardial B-Cells
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Monogenic autoinflammatory disease-associated cardiac damage.

Qijiao Wei1, Li Sun2

  • 1Department of Rheumatology, Children's Hospital of Fudan University, Shanghai, China.

Inflammation Research : Official Journal of the European Histamine Research Society ... [Et Al.]
|August 10, 2023
PubMed
Summary

Monogenic autoinflammatory diseases (AIDs) can cause various heart problems, including pericarditis and cardiomyopathies. Familial Mediterranean fever (FMF) is particularly associated with cardiac issues, highlighting the need for further research.

Keywords:
Cardiac damageDefects affecting the inflammasomeMonogenic autoinflammatory diseasesNon-inflammasome-related conditionsType 1 interferonopathies

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Area of Science:

  • Immunology
  • Cardiology
  • Genetics

Background:

  • Autoinflammatory diseases (AIDs) involve unprovoked inflammation from a dysregulated innate immune system without autoantibodies or infections.
  • Monogenic AIDs are caused by mutations in single genes affecting inflammation.
  • This review focuses on cardiac manifestations in monogenic AIDs.

Purpose of the Study:

  • To review the spectrum of cardiac manifestations in monogenic autoinflammatory diseases.
  • To highlight specific cardiac conditions associated with these genetic disorders.

Main Methods:

  • Literature review of monogenic autoinflammatory diseases and their cardiac involvement.
  • Analysis of reported cardiac manifestations in affected patient populations.

Main Results:

  • Monogenic AIDs can present with diverse cardiac lesions.
  • Common manifestations include pericarditis, valvular diseases, coronary artery disease, cardiomyopathies, and pulmonary hypertension.
  • Familial Mediterranean fever (FMF) shows a notable association with cardiac involvement, particularly pericardial effusion.

Conclusions:

  • Monogenic AIDs are linked to a range of cardiac pathologies.
  • Pericardial effusion is a frequent cardiac manifestation, potentially stemming from systemic inflammation.
  • Further research is essential to elucidate the precise relationship between monogenic AIDs and cardiac damage.