Clinical characteristics and treatment effect of 15 patients with arrhythmogenic right ventricular dysplasia and cardiomyopathy

AIM: To analyze the clinical characteristics and treatment effect of arrhythmogenic right ventricular dysplasia and cardiomyopathy (ARVD/C). METHODS: Clinical features were reevaluated in 15 patients diagnosed with ARVD/C from 2000 to 2007 in the Department of Cardiology, Nanfang Hospital. Statistical analysis was carried out and treatment effect was evaluated. RESULTS: In a cohort of 15 patients (7 males), mean age at the time of hospitalization was (31±12) years and mean age at initial presentation was (28±11) years. Three patients had a significant family history. Six patients (40%) had a history of syncope. Five cases (33%) had slight palpitations. Only one patient had epsilon waves in right precordial leads (V2-3), with T-wave inversions. Abnormal echocardiography was found in 13 patients (87%), mainly right ventricular (RV) dilation. Four patients received magnetic resonance imaging (MRI) and fat signal intensity of RV wall was found in two cases, thinning of RV wall in three cases, and right ventricular hypertrophy in three cases. For symptomatic patients, ventricular arrhythmias were treated with amiodarone, β-blocker or other antiarrhythmic therapies. Antiarrhythmic drugs were ineffective in 47% (7/15) patients and three patients were therefore treated by ablations. Nevertheless, ventricular tachycardia recurred in one patient. Four patients had implanted cardiac defibrillation (ICD). However, one of them had ICD twice because the battery was exhausted. CONCLUSION: ARVD/C is characterized by ventricular arrhythmias. Diagnosis of ARVD/C may be accomplished by family history, history of syncope, ECG, echocardiography and cardiac MRI. Antiarrhythmic drugs are not very effective. Ablations or ICD can treat ventricular arrhythmias to reduce the incidence of sudden cardiac death.