Clinical manifestations of arrhythmogenic right ventricular cardiomyopathy in Korean patients

Abstract

Background: The clinical manifestations of the Korean patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are not well known.

Methods: The clinical data of Korean patients who met the Task Force Criteria for ARVC were analyzed.

Results: Thirty-seven patients (41.2+/-14.8 years old, 19 males) were diagnosed with ARVC. The commonest presenting symptoms were palpitations (30%), syncope/presyncope (30%) and no symptoms (30%). Four patients had a family history of premature sudden death or ARVC. Most patients with no symptoms were evaluated due to ECG abnormalities or asymptomatic ventricular arrhythmias. Ventricular tachycardia, ventricular fibrillation and frequent premature ventricular contractions only were observed in 35%, 5% and 24%, respectively. Wall motion abnormalities of the right and left ventricles were detected in 92% and 41%, respectively. Fatty or fibrofatty infiltration was observed in 26 of the 32 (81%) patients who underwent an endomyocardial biopsy. Two patients had signs of heart failure. Two patients with syncope/presyncope were diagnosed with vasovagal syncope and another was due to side effects from a medication. Most of the patients with ventricular arrhythmias were treated with beta-blockers and/or amiodarone. Implantable cardioverter-defibrillators (ICDs) were implanted in 3 patients. During a mean follow-up of 27.4+/-26.5 months no syncope or sudden death developed except for in one patient with an ICD who suffered from recurrent shocks due to ventricular fibrillation.

Conclusions: ARVC may be an important cause of syncope, ventricular arrhythmias, and ECG and wall motion abnormalities of the ventricles in Koreans. The Korean patients with ARVC exhibited various clinical manifestations.