Abstract
: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive inherited heart disease characterized by ventricular arrhythmias and sudden cardiac death especially in the young. ARVC has been traditionally associated with the Mediterranean basin, as many seminal studies on the disease have originated from research groups of this region. Today, however, numerous ARVC registries from all over the world emphasize that the disease does not have a specific racial or geographical predilection. This work provides a review on the global perspective of ARVC.
Highlights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable cardiac muscle disease characterized pathologically by fibrofatty replacement –usually but not exclusively- of the right ventricular (RV) myocardium
The disease shows a familial pattern in 40–70% of the cases and molecular genetic studies demonstrated that ARVC is a desmosomal disease resulting from genetically defective cell adhesion proteins such as plakoglobin, desmoplakin, plaokophillin-2, desmoglein-2, and desmocollin [2,3]
Clinical manifestations are related to electrical instability, including either ventricular tachycardia of RV origin or ventricular fibrillation, which may lead to sudden cardiac death (SCD), mostly in young people and athletes [4 –6]
Summary
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable cardiac muscle disease characterized pathologically by fibrofatty replacement –usually but not exclusively- of the right ventricular (RV) myocardium. All the cases had T wave inversion on the right precordial surface ECG at rest and left bundle branch block QRS pattern during ventricular tachycardia [11] This was the first time ARVC was acknowledged as a major cause of death in the young. This screening program has shown marked efficacy in detecting cardiac pathologies leading to SCD in the young, with a drop of mortality rates among athletes in the Veneto region from 3.5/100,000 in 1979 to 0.5/100,000 in 2003 (Figure 3) [28] This impressive drop was largely attributable to the successful detection of ARVC and hypertrophic cardiomyopathy among the screened individuals, as early reports accounted ARVC as the second most common cause of death in the young in the Veneto region [11]. It was found that cardiomyopathies are the most common cause of SCD in young British athletes accounting for 62% with ARVC attributed to 14% of deaths [31]
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