Key result
Arrhythmogenic right ventricular cardiomyopathy/dysplasia is a progressive disease characterized by fibrofatty replacement of the right ventricle, requiring high clinical suspicion for diagnosis and management to prevent sudden cardiac death.
A high clinical suspicion and extensive multimodality evaluation are required to establish the diagnosis of ARVC/D and guide optimal management, including ICD therapy and heart failure treatment.
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by progressive, fibrofatty replacement of the myocardium, ventricular arrhythmia, sudden death, and progressive heart failure. ARVC/D may be an important cause of syncope, ventricular arrhythmias, electrocardiogram (ECG) abnormalities and/or non-ischemic wall motion abnormalities. Some patients, however, do not have a typical clinical presentation. Thus, a high clinical suspicion and extensive studies may be needed to establish the diagnosis of ARVC/D. Recent progress in diagnostic modalities and a better understanding of the clinical manifestations of ARVC/D may lead to optimal management of affected patients. (Korean Circ J 2008;38:514-523)
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Yongkeun Cho (2008) conducted a review in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. Arrhythmogenic right ventricular cardiomyopathy/dysplasia is a progressive disease characterized by fibrofatty replacement of the right ventricle, requiring high clinical suspicion for diagnosis and management to prevent sudden cardiac death.
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