| Objective:To analyze the relationship between the disease extent and the clinical manifestation in patients with the arrhythmogenic right ventricular cardiomyopathy (ARVC).Methods:The study group consisted of 61 subjects with a definite diagnosis of ARVC on the basis of published criteria. We classified patients into 3 groups (Local right ventricular groups, severe right ventricular groups and biventricular groups) according to cardiac magnetic resonance imaging(MRI) results. The clinical data including age, gender, syncope and amaurosis fugax, palpitation and chest discomfort, family history, sustained or non-sustained LBBB-type ventricular tachycardia,24-hour premature ventricular contractions occurred more than 1000 times,12-lead ECGs,24h-Holter parameters, UCG parameters, Electrophysiology Testing parameters. To analyze the relationship between Polymorphic premature ventricular contractions and monomorphic premature ventricular contractions with clinical adverse events in 24-hour ambulatory electrocardiographic monitoring.Results:(1) A group had 19 patients (31%). B group had 28 patients (46%). C group had 14 patients (23%). Right ventricular outflow tract and right ventricular free wall, the base of the right ventricle, right ventricular apex, left ventricle were significantly different among the three groups. (2) Palpitation and chest discomfort, premature ventricular contractions(≥1000 times/24-hour), syncope, sustained or non-sustained LBBB-type ventricular tachycardia, family history showed no significant differences among the three groups. In 41% of patients with amaurosis fugax, there was a significant difference between A group (21%) and B group (79%) (P=0.001); there was a significant difference between B group (36%) and C group (79%) (P=0.009). (3) A, B, C group have one cases of patients with normal electrocardiogram; 24 (39%) patients had Epsilon wave,21 (34%) patients had QRS duration≥110ms(V1-V3),17 (28%) patients had Prolonged S-wave upstroke≥55ms,10 (16%) patients had Complete right branch bundle block,9 (15%) patients had Pathologic Q waves, the incidence of these parameters was increased with the disease extent (A group< B group< C group); QRS duration (V1+V2+V3)/(V4+V5+V6)≥1.2, incomplete right branch bundle block, I°atrioventricular block, left bundle branch block, ST-segment elevation, continuous and non-sustained LBBB-type ventricular tachycardia was no correlation with the disease extent. There was not statistical difference among the three groups. Epsilon wave, QRS duration≥110ms(VI-V3) had statistically significant difference between A group and C group; Prolonged S-wave upstroke≥55ms had statistically difference between A group and C group, B group and C group; First-degree atrioventricular block had statistically difference between the B group and C group; T-wave inversion in V1 leads was often found with A group. T-wave inversion was was more significantly present in inferior leads, V1-V3 leads or beyond V3 with B group and C group. (4) In 24-hour ambulatory electrocardiographic monitoring,9 (28.1%) patients had palpitation in monomorphic ventricular premature contractions, but they did not had adverse event; 23 (71.9%) patients had palpitation and 6 (26.1%) patients had syncope in polymorphic ventricular premature contractions; 6 (26.1%) patients were implanted ICD therapy,2 (8.7%) patients had family history of sudden cardiac death, they were manifested in the patients with pleomorphic ventricular premature contractions. (5) 15 (25%) patients had normal Echocardiography (A group had 13 patients, B group had 2 patients), they diagnosed by cardiac MRI. There was not right atrium dilatation, right ventricular wall thinning, right ventricular wall motion abnormalities in the A group of Local right ventricular in patients with ARVC; B group and C group was significantly higher than the incidence of A group (A group |