Long‐term follow‐up of arrhythmogenic right ventricular cardiomyopathy patients with an implantable cardioverter‐defibrillator for prevention of sudden cardiac death

Abstract

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited cardiomyopathy with a high burden of ventricular arrhythmia, which is an important cause of sudden cardiac death (SCD). Implantable cardioverter‐defibrillator (ICD) is believed to be the most reliable management against SCD. Hypothesis Ventricular arrhythmia does not necessarily confer a poor prognosis in ARVC patients with an ICD. Methods A total of 39 ARVC patients (34 male) implanted with an ICD at our electrophysiology center and followed up continuously were included in this study. The mean age at diagnosis was 42.1 ± 14.8 years. Results Thirty‐three patients (84.6%) had suffered ventricular arrhythmia with hemodynamic compromise before ICD implantation. During a median follow‐up of 48.6 months (interquartile range, 32.3–73.3), 3 patients (7.7%) died, 1 of sudden death, 1 of heart failure, and 1 of cerebral infarction. Twenty‐eight patients (71.8%) experienced 540 appropriate ICD interventions. The first appropriate ICD intervention occurred more than 2 years after initial ICD implantation in 5 patients (12.8%). Twelve patients (30.8%) suffered from electrical storm. The event‐free period was significantly shorter in patients who did not have broad precordial T wave inversion ≥V1–V3 (hazard ratio = 0.39, 95% confidence interval: 0.16‐0.96). No significant difference was shown in antiarrhythmic drugs and radiofrequency catheter ablation before ICD implantation between patients with and without appropriate ICD therapies (P > 0.05). Conclusions Recurrence of sustained ventricular tachycardia/ventricular fibrillation is frequent in high‐risk patients with ARVC. The prognosis is favorable for ARVC patients treated with an ICD for prevention of SCD.

Blockade of the renin‐angiotensin‐aldosterone system in patients with arrhythmogenic right ventricular dysplasia: A double‐blind, multicenter, prospective, randomized, genotype‐driven study (BRAVE study)

“…An ICD is an efficient treatment for prevention of SCD. 4 Long-term follow-up studies are rare. Magnetic resonance imaging (MRI) and echocardiograms are the best examinations to monitor progression of ARVD.…”

Cardiomyopathies in China: A 2018–2019 state‐of‐the‐art review

“…Broad precordial T-wave inversion ≥V1–V3 was associated with a shorter event-free period. 115 In a study of 48 adults with ARVC undergoing radiofrequency catheter ablation with a minimum follow-up of 2 years, long-term freedom from VT was 56.3% at 71.4 ± 45.7 months after the last procedure. Only 37.5% of the patients had VT-free survival after a single procedure.…”

Arrhythmogenic right ventricular cardiomyopathy

“…Usual medication consist of β‐blockers, amiodarone, and sotalol . Although some studies showed beneficial effects of sotalol and amiodarone, most studies suggested no significant effects of those drugs in reducing the rate of life‐threatening arrhythmic events . Despite those limited data, β‐blockers are currently recommended for both prevention of arrhythmias and reduction in ventricular wall stress.…”

“…Catheter ablation is a therapeutic option for patients with sustained monomorphic VT. Although no significant reduction in life‐threatening arrhythmic events during the follow‐up was observed, catheter ablation results in symptomatic improvement by a significant reduction in the burden of VT . Additional antiarrhythmic drug therapy, repeated ablation, and backup ICD implantations are required to provide suppression of VTs and SCD prevention …”