Abstract
Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is an inherited cardiomyopathy characterized clinically by ventricular arrhythmias, sudden death, and structural abnormalities of the right ventricle. Although structural involvement of the right ventricle predominates, a left dominant form (LDAC) has been described. Structural involvement of both ventricles is common in advanced disease. Due to significant heterogeneity in its manifestation, the diagnosis of ARVC/D is challenging and requires a multifaceted approach to patient evaluation. The recently published Revised Task Force Criteria provide clinicians with a validated model to improve the diagnosis of ARVC/D. The management of ARVC/D is primarily aimed at reducing the burden of symptomatic arrhythmias and decreasing the incidence of sudden cardiac death. Automatic implantable cardioverter-defibrillators (AICDs) significantly reduce mortality in patients with ARVC/D. However, accurate risk assessment is needed to avoid exposing low-risk patients to the long-term complications of AICDs. Strenuous exertion increases the rate of disease progression and increases the risk of sudden death.
Original language | English (US) |
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Title of host publication | Electrical Diseases of the Heart |
Subtitle of host publication | Volume 2: Diagnosis and Treatment, Second Edition |
Publisher | Springer-Verlag London Ltd |
Pages | 41-54 |
Number of pages | 14 |
ISBN (Electronic) | 9781447149781 |
ISBN (Print) | 9781447149774 |
DOIs | |
State | Published - Jan 1 2013 |
Keywords
- Arrhythmogenic cardiomyopathy
- Arrhythmogenic right ventricular cardiomyopathy (ARVC/D)
- Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)
- Arrhythmogenic right ventricular dysplasia (ARVD)
- Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)
ASJC Scopus subject areas
- Medicine(all)