The many faces of early repolarization syndrome: A single-center case series.
Adolescent
Adult
Cardiac Resynchronization Therapy
/ methods
Catheter Ablation
/ methods
Electrocardiography
Female
Follow-Up Studies
Heart Conduction System
/ physiopathology
Heart Ventricles
/ physiopathology
Humans
Male
Middle Aged
Retrospective Studies
Syndrome
Tachycardia, Ventricular
/ physiopathology
Time Factors
Young Adult
Cardiac arrest
Catheter ablation
Early repolarization syndrome
J wave
Ventricular fibrillation
Journal
Heart rhythm
ISSN: 1556-3871
Titre abrégé: Heart Rhythm
Pays: United States
ID NLM: 101200317
Informations de publication
Date de publication:
02 2020
02 2020
Historique:
received:
30
07
2019
pubmed:
16
9
2019
medline:
28
4
2021
entrez:
16
9
2019
Statut:
ppublish
Résumé
Early repolarization syndrome (ERS) is a rare but increasingly recognized cause of malignant ventricular arrhythmias. The purpose of this study was to characterize the presentations and treatments of ERS at our institution. We performed a retrospective chart review of all patients presenting to our institution between 2008 and 2019 with ERS. Exclusion criteria included Brugada syndrome, positive provocative testing with class I antiarrhythmic drugs, metabolic disturbances, or structural heart disease. Of 10 patients identified with ERS, 8 were men with a mean age of 30 ± 17 years at diagnosis. Documented arrhythmias included ventricular fibrillation in 7 of 10, polymorphic ventricular tachycardia in 3 of 10, and monomorphic ventricular tachycardia in 3 of 10 patients. Atrial fibrillation was diagnosed in 3 of 10, and atrioventricular block was seen in 2 of 10. J waves and/or electrocardiographic early repolarization patterns were dynamic in 7 of 10. Arrhythmias occurred at rest in 8 of 10 and with exertion in 2 of 10. Only 1 patient had a family history of sudden death, and 4 of 10 patients had variants of uncertain significance on genetic testing. Quinidine effectively suppressed arrhythmias in 5 of 5 patients but required dose escalation to >1 g/d in 3 of 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 triggers were also targeted for ablation in 3 patients. ERS is a heterogeneous condition and may be associated with both atrial and ventricular arrhythmias, atrioventricular block, dynamic electrocardiographic changes, and variable triggers. In addition to targeting premature ventricular contraction triggers, mapping and ablation of abnormal epicardial electrograms may be a potential future treatment strategy.
Sections du résumé
BACKGROUND
Early repolarization syndrome (ERS) is a rare but increasingly recognized cause of malignant ventricular arrhythmias.
OBJECTIVE
The purpose of this study was 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 and 2019 with ERS. Exclusion criteria included Brugada syndrome, positive provocative testing with class I antiarrhythmic drugs, metabolic disturbances, or structural heart disease.
RESULTS
Of 10 patients identified with ERS, 8 were men with a mean age of 30 ± 17 years at diagnosis. Documented arrhythmias included ventricular fibrillation in 7 of 10, polymorphic ventricular tachycardia in 3 of 10, and monomorphic ventricular tachycardia in 3 of 10 patients. Atrial fibrillation was diagnosed in 3 of 10, and atrioventricular block was seen in 2 of 10. J waves and/or electrocardiographic early repolarization patterns were dynamic in 7 of 10. Arrhythmias occurred at rest in 8 of 10 and with exertion in 2 of 10. Only 1 patient had a family history of sudden death, and 4 of 10 patients had variants of uncertain significance on genetic testing. Quinidine effectively suppressed arrhythmias in 5 of 5 patients but required dose escalation to >1 g/d in 3 of 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 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, atrioventricular block, dynamic electrocardiographic changes, and variable triggers. In addition to targeting premature ventricular contraction triggers, mapping and ablation of abnormal epicardial electrograms may be a potential future treatment strategy.
Identifiants
pubmed: 31521808
pii: S1547-5271(19)30834-3
doi: 10.1016/j.hrthm.2019.09.013
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
273-281Informations de copyright
Copyright © 2019 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.