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Related Concept Videos

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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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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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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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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Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
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Arrhythmias are irregular heart rhythms occurring when the heart's electrical impulses become abnormal. These disturbances can lead to various symptoms, depending on their severity and the underlying cause. Some common factors contributing to arrhythmias include hypoxia, ischemia, electrolyte imbalances, excessive catecholamine exposure, drug toxicity, and muscle overstretching. Arrhythmias can be classified into two main types based on the rate and site of origin of abnormal heart rhythms.
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Arrhythmogenic left ventricular cardiomyopathy.

Seyedeh Mojdeh Mirmomen1, Andrew Jay Bradley1, Andrew Ernest Arai1

  • 1National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA.

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Arrhythmogenic ventricular cardiomyopathy (AVC) is a genetic heart condition causing fibrofatty tissue in the heart muscle. Primarily affecting the right ventricle, AVC can rarely involve the left ventricle, posing diagnostic challenges.

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Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Arrhythmogenic ventricular cardiomyopathy (AVC) is an inherited myocardial disorder.
  • It involves fibrofatty infiltration of the heart muscle, leading to arrhythmias and sudden cardiac death.

Observation:

  • The classic form, arrhythmogenic right ventricular cardiomyopathy (ARVC), primarily affects the right ventricle.
  • Left ventricular involvement can occur in advanced ARVC cases.

Findings:

  • Predominantly left ventricular AVC is rare.
  • Diagnosis is challenging due to lack of specific criteria and potential cardiotoxicity.

Implications:

  • Understanding predominantly left ventricular AVC is crucial for accurate diagnosis and management.
  • Further research is needed to develop diagnostic criteria for this rare subtype.