Clinical features and arrhythmic complications of pediatric-onset arrhythmogenic right ventricular dysplasia

Abstract

Objective:Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial genetic disease that occurs primarily in the right ventricle. Patients with ARVD may present with severe ventricular arrhythmias, syncope, and cardiac arrest. The purpose of this study is to evaluate the clinical features and arrhythmic complications of patients with pediatric-onset ARVD.Methods:Patients diagnosed with ARVD between January 2010 and January 2019 were included in this study.Results:A total of 19 patients with ARVD were evaluated. Of them, 15 patients were male, and their mean age was 12±4 years. The most common symptoms were palpitations (n=6), syncope (n=4), and heart failure symptoms (n=2). Five patients were asymptomatic. Thirteen patients had an epsilon wave; all patients ≥14 years had a T wave inversion in V1–3. Premature ventricular contractions (PVCs) were observed in 15 patients, and ventricular tachycardia (VT) was observed in 9 patients. All patients underwent cardiac magnetic resonance imaging (MRI). Echocardiography and cardiac MRI of two patients were normal at the time of admission; patients were in the concealed phase, and the diagnosis was made by ECG, Holter monitoring, and genetic findings. We administered a beta-blocker in all patients. Two patients underwent an electrophysiological study and ablation because of PVC/VT. An implantable cardiac defibrillator was implanted in 8 patients. The mean follow-up period was 21.5±11 months. Two patients were deceased with incessant VT and heart failure, and one patient was deceased with multiorgan dysfunction after biventricular assist device implantation (n=3).Conclusion:Diagnosis of pediatric-onset ARVD might be much more difficult in children. Sudden cardiac death might be prevented in the early period by raising the awareness of physicians about the disorder. Prevention of sudden death with implantable cardiac defibrillators is crucial in the management of these patients. It should be kept in mind that children with structurally normal hearts may present with an earlier concealed phase and can be diagnosed with ARVD.

A territory-wide study of arrhythmogenic right ventricular cardiomyopathy patients from Hong Kong

“…The model constructed, which included age, male gender, cardiac syncope in the prior 6 months, prior non-sustained VT, number of PVCs in 24 h, number of leads with TWI and right ventricular ejection fraction, demonstrated an improved ability to estimate risk of ventricular arrhythmias and guide decision-making in ICD implantation for such patients (41). A meta-analysis identified the following 11 variables as the most important factor for predicting arrhythmic events: (1) male gender, (2) presyncope, (3) left ventricular dysfunction, (4) TWI in inferior leads, (5) proband status, (6) late potentials, (7) syncope, (8) inducibility at electrophysiological study, (9) right ventricular dysfunction, (10) epsilon waves, and (11) premature ventricular contractions greater than 1000/24 h (42). To our knowledge, such scoring algorithms have not been used to investigate outcomes beyond VT/VF in ARVC/D cohorts.…”

A Territory-Wide Study of Arrhythmogenic Right Ventricular Cardiomyopathy Patients from Hong Kong

“…Disease progression is in turn dominated by diffuse thinning of the right ventricular wall with cardiomyocyte loss and corresponding fibrofatty replacement of the myocardium [ 5 ]. These pathological alterations not only disturb the native electrical conduction system, thereby predisposing affected patients to malignant ventricular arrhythmias and sudden cardiac death (SCD) [ 6 , 7 ], but also potentially induce left ventricular dysfunction and subsequent dual-chamber cardiac failure [ 8 ].…”