Abstract
Background
In arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), implantable cardioverter-defibrillators (ICD) after an episode of sustained monomorphic ventricular tachycardia (MVT) are currently recommended in most situations. However, radiofrequency catheter ablation (RCA) is effective in reducing recurrent VT and whether MVT is a surrogate of sudden cardiac death is debated when other risk factors are lacking.
Purpose
To report the outcomes of patients with ARVC/D who underwent RCA of well-tolerated MVT without a back-up ICD.
Methods
Patients with a definite ARVC/D diagnosis according to the 2010 Task Force revised criteria who underwent RCA of well-tolerated MVT at 9 tertiary centers across 5 countries, without an ICD prior to RCA and in the 3 following months were retrospectively included. Patients presenting with syncope or electrical storm, and patients with left ventricular ejection fraction <50% were excluded. Similar patients implanted with an ICD prior or without RCA in the same period served as controls.
Results
Sixty-five patients [median age 46.1 years, range (19.5–73.8), 75% males] underwent RCA of MVT between 2003 and 2016. Familial history of ARVC/D was found in 11% of patients. Epsilon-waves were present in 19% and T-waves inversion beyond V2 in 43%. A right ventricular (RV) ejection fraction ≤40% or fractional area change ≤33% was found in 14 (25%) patients. Median left ventricular ejection fraction was 61% (50–70). Clinical presentation was palpitations in 81% of patients and near-syncope in 14%. Prior to RCA, patients were on beta-blockers alone in 18%, class I drugs in 37% and amiodarone in 9%, while 15% of patients were free any antiarrhythmic medication. Only 1 patient (2%) had >1 clinical VT morphology. Median VT rate was 180 (110–270). An epicardial approach was used in 31% patients. The clinical VT was inducible in 84% of patients. The median number of targeted RV site was 1 (1–3) (RV outflow tract in 72%). Full acute success defined inability to induce any VT was achieved in 72% of patients. During a median follow-up time of 49 month (1.4–162), there was no death or aborted cardiac arrest. Survival without VT recurrence was estimated at 82%, 71% and 60%, 12-, 36- and 60-months after RCA. No VT recurrence was observed among patient who had undergone an epicardial ablation. Among patients with VT recurrence, 6 (35%) did not receive an ICD, and 14 (70%) underwent redo RCA. An ICD was implanted in 10 patients, including 5 for VT recurrence. Fifty-eight patients constituted the control group, and 64% had appropriate ICD interventions during follow-up.
Conclusions
Despite a significant rate of VT recurrence, selected patients with ARVC/D who underwent RCA for stable MVT without an ICD did not experience any arrhythmic death. Further prospective studies are mandatory to precise the respective places of ICD and RCA in the management of ARVC/D patients with well-tolerated MVT.
Acknowledgement/Funding
None
Two Novel Variants in Genes of Arrhythmogenic Right Ventricular Cardiomyopathy – a Case Report
