The Many Faces of Early Repolarization Syndrome:A Single Center Case-Series

BACKGROUND Early repolarization syndrome (ERS) is a rare but increasingly recognized cause of malignant ventricular arrhythmias. OBJECTIVE To characterize the presentations and treatments of ERS at our institution. METHODS We performed a retrospective chart review of all patients presenting to our institution between 2008-2019 with ERS. Exclusion criteria included Brugada syndrome, positive provocative testing with class I antiarrhythmics, metabolic disturbances or structural heart disease. RESULTS Of 10 patients identified with ERS, 8 were male with age 30±17 years at diagnosis. Documented arrhythmias included ventricular fibrillation in 7/10, polymorphic ventricular tachycardia (VT) in 3/10 and monomorphic VT in 3/10 patients. Atrial fibrillation was diagnosed in 3/10 and AV block was seen in 2/10. J waves and/or electrocardiographic early repolarization patterns were dynamic in 7/10. Arrhythmias occurred at rest in 8/10 and with exertion in 2/10. Only 1 patient had a family history of sudden death and 4/10 patients had variants of uncertain significance on genetic testing. Quinidine effectively suppressed arrhythmias in 5/5 patients, but required dose escalation to>1g/day in 3/5 patients. Abnormal epicardial electrograms were recorded over the inferolateral left ventricle in 2 patients who underwent mapping and were successfully ablated. Premature ventricular contraction (PVC) triggers were also targeted for ablation in 3 patients. CONCLUSION ERS is a heterogeneous condition and may be associated with both atrial and ventricular arrhythmias, AV block, dynamic electrocardiographic changes and variable triggers. In addition to targeting PVC triggers, mapping and ablation of abnormal epicardial electrograms may be a potential future treatment strategy.
as reported in: Heart Rhythm. 2019 Sep 12 [Epub ahead of print]