Arrhythmogenic RightVentricular Dysplasia:New Mapping and Ablation Strategy

摘要

Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disease predominantly affecting the right ventricle (RV). It is a form of familial cardiomyopathy with autosomal dominant inheritance (1,2). Pathologically, it is characterized by fibrofatty replacement of RV myocardium, occasionally involving the left ventricle (LV) as well (3-5) and sometimes misdiagnosed as a dilated cardiomyopathy. Ventricular arrhythmia originating from the RV may cause hemodynamic instability resulting in syncope or sudden cardiac death (4,6), which may be the first manifestation of this disease without any preceding symptoms.The clinical manifestation varies greatly, especially in different ethnic groups. In a study from the Veneto region of Italy, up to 20% of sudden death events in young people and athletes were due to ARVD (5). In Naxos Island of Greece, the mode of inheritance of 25 patients from 12 families suffered from ARVD in association with palmoplanar keratosis (Naxos disease) (7) was autosomal recessive and the deletion in plakoglobin accounted for the disorder (8). Familial incidence of premature sudden death in Chinese patients with ARVD appeared to be low and LV involvement in affected individual seemed to be uncommon (9). Seven chromosomal loci were identified by linkage analysis (10). Though the mode of inheritance in most patients is autosomal dominant, the involved genes and the molecular defects causing the common form of this disease are still unknown.