title: 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.
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Rigato
authors.forename: Domenico
authors.initials: D
authors.lastname: Corrado
authors.forename: Cristina
authors.initials: C
authors.lastname: Basso
authors.forename: Alessandro
authors.initials: A
authors.lastname: Zorzi
authors.forename: Kalliopi
authors.initials: K
authors.lastname: Pilichou
authors.forename: Barbara
authors.initials: B
authors.lastname: Bauce
authors.forename: Gaetano
authors.initials: G
authors.lastname: Thiene
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D017115:Catheter Ablation / Q000379:methods*; D017147:Defibrillators, Implantable; D015438:Health Behavior; D016027:Heart Transplantation; D006801:Humans
chemical_list: D000319:Adrenergic beta-Antagonists; D000889:Anti-Arrhythmia Agents; D000925:Anticoagulants