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Related Concept Videos

Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

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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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Cardiomyopathy II: Dilated Cardiomyopathy01:30

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Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

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Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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Imbalances in Cardiac Output01:26

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The heart's primary function is to pump blood throughout the body, maintaining a balance between blood sent out (cardiac output) and blood returning (venous return). If this balance is disrupted, it can result in congestive heart failure (CHF), a severe condition where the heart becomes an inefficient pump, leading to inadequate blood circulation.
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...
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Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

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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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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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Rat Model of Right-Sided Cardiac Remodeling and Arrhythmia Using Pulmonary Artery Banding
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IgG4 related cardiac disease.

Hector Carbajal1, Lindsay Waters, Jovan Popovich

  • 1Houston Methodist Hospital, Houston, Texas ; Weill Cornell Medical College, New York, New York.

Methodist Debakey Cardiovascular Journal
|December 4, 2013
PubMed
Summary
This summary is machine-generated.

This case report details a patient with IgG4-related disease presenting with cardiac involvement, including heart block. Positron emission tomography (PET) scans may aid in identifying myocardial involvement in this condition.

Keywords:
IgG4inflammatory pseudotumor

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Area of Science:

  • Immunology
  • Cardiology
  • Pathology

Background:

  • Immunoglobulin G4 (IgG4)-related systemic disease is a multisystemic inflammatory condition.
  • Inflammatory pseudotumors with IgG4 pathology can affect various organs.

Observation:

  • A patient initially diagnosed with an IgG4-related inflammatory pseudotumor experienced visual symptoms responsive to prednisone.
  • Cardiac complications, including chest pain, bradycardia, and third-degree heart block, developed years later, requiring pacemaker implantation.
  • Positron emission tomography (PET) revealed extensive IgG4-related disease involvement in multiple organs, including the myocardium and sinoatrial (SA) node.

Findings:

  • While direct cardiac pathology confirmation was absent, the clinical presentation and extent of organ involvement strongly suggested IgG4-related cardiac disease.
  • This case highlights the potential for IgG4-related disease to manifest with significant cardiac complications.

Implications:

  • The findings suggest that IgG4-related systemic disease can affect the heart, potentially leading to conduction abnormalities.
  • Positron emission tomography (PET) scanning may be a valuable tool for detecting myocardial and SA node involvement in IgG4-related disease.
  • Further research is warranted to understand the mechanisms and long-term cardiac implications of IgG4-related disease.