Pharmacotherapy and other therapeutic modalities for managing Arrhythmogenic Right Ventricular Cardiomyopathy.

ABSTRACT

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a genetically determined rare cardiomyopathy (1 in 5000 to 1 in 2000 in the general population), which can lead to ventricular arrhythmias and sudden death (SD). The classic form of the disease has a predilection for the right ventricle (RV), but recognition of left-dominant and biventricular variants led to the broader term "Arrhythmogenic Cardiomyopathy". The disease affects men more frequently than women and becomes clinically overt usually from the second to the fourth decade of life. Treatment consists of restriction of physical exercise, antiarrhythmic drugs, catheter ablation and ICD implantation. These treatments have the potential to change the natural history of the disease by protecting against SD and offering a good-quality and nearly normal life-expectancy. Antiarrhythmic drugs play an important role in terms of reduction of both the number and the complexity of arrhythmias, but they do not reduce the risk of SD. The results of catheter ablation are poor because of the high rate of VT recurrence. ICD should be reserved to selected patients after an accurate risk stratification. The clinical challenge is to improve risk stratification for better identification of those patients who most benefit from the above therapies. Unfortunately, a curative therapy is not yet available.