Related Experiment Video
Updated: May 29, 2026
10:39
Rat Model of Right-Sided Cardiac Remodeling and Arrhythmia Using Pulmonary Artery Banding
Published on: August 30, 2024
Arrhythmogenic right ventricular cardiomyopathy/dysplasia
Dimitrios Avramides1, Nikos Protonotarios, Angeliki Asimaki
1Cardiology Department, G. Gennimatas Hospital, Athens, Greece. d_avramides@yahoo.com
Hellenic Journal of Cardiology : HJC = Hellenike Kardiologike Epitheorese
|September 24, 2011
Summary
This rare case highlights arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) in a middle-aged woman, suggesting an acquired form. The study reviews ARVC/D diagnosis, management, and prevention strategies.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a heritable heart muscle disease.
- Diagnosis can be challenging, especially in atypical presentations.
Observation:
- A 51-year-old woman presented with a 10-year history of dyspnea, fatigue, presyncope, and ventricular extrasystolic arrhythmia.
- Initial tests excluded coronary artery disease, valvular disease, and left ventricular dysfunction.
- Clinical criteria for ARVC/D were met, confirmed by endomyocardial biopsy.
Findings:
- The patient showed significant left ventricular structural progression over 5 years.
- Diagnosis was made despite a negative family history, negative desmosome mutation tests, and negative myocardial immunohistochemistry.
- This suggests a potential acquired form of ARVC/D.
Implications:
- This case expands the understanding of ARVC/D presentation and potential etiologies.
- It underscores the importance of considering ARVC/D in middle-aged individuals with unexplained arrhythmias and heart failure symptoms.
- The review provides a comprehensive overview for clinicians managing ARVC/D patients.
Related Concept Videos
01:25Cardiomyopathy I: Introduction and Classification
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...
01:29Cardiomyopathy IV: Restrictive Cardiomyopathy
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...
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
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...
01:30Cardiomyopathy II: Dilated Cardiomyopathy
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,...
01:29Disturbances in Heart Rhythm
Arrhythmia or dysrhythmia refers to an abnormal heart rhythm caused by a defect in the heart's conduction system. It can cause the heart to beat irregularly, too quickly, or too slowly, leading to symptoms like chest pain, shortness of breath, and fainting. Factors such as stress, caffeine, alcohol, nicotine, cocaine, certain drugs, congenital defects, diseases, and electrolyte abnormalities can trigger arrhythmias.
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
01:15Dysrhythmias I: Introduction
Dysrhythmias refers to abnormalities in the heart's rhythm. They result from disruptions in the heart's electrical conduction system, which includes the sinoatrial(SA)node, atrioventricular(AV) node, the bundle of His, bundle branches, and Purkinje fibers.Definition and PathophysiologyDysrhythmias result from disorders of impulse formation, impulse conduction, or both. The heart contains specialized cells in the sinoatrial node, atrioventricular node, and the bundle of His and Purkinje fibers...