Abstract 15226: High Prevalence of Early Repolarization in Patients With Noncompaction Cardiomyopathy Presenting With Malignant Ventricular Arrhythmias.
Purpose: Early repolarizations are been associated with malignant ventricular arrhythmias, including idiopathic VF and sudden cardiac death (SCD). The possible mechanism include increased trabeculation with deep intramyocardial invagination, carrying the Purkinje system deeper into the mid-myocardium resulting in inhomogenous repolarization. Noncompaction cardiomyopathy (NCCM) is a recently classified, yet rare primary cardiomyopathy with hallmark of excessive trabeculations. Malignant ventricular arrhythmias, including sustained ventricular tachycardia (VT), ventricular fibrillation (VF) has frequently described. The aim of this study was to determine the prevalence early repolarization in NCCM patients, especially in those, primarily presenting with malignant ventricular arrhythmia, including SCD.
Methods: We used the prospective data from our NCCM registry from 2005 to present (n= 74 patients, mean age: 40 ± 14 years, range [17 − 77]). The patients are divided in SCD group (n=11 (15%) with initial presentation of sustained VT or VF and non-SCD group (n=63 (85%), with initial presentation of heart failure or other.
Results: Overall, 18 of the 74 (24%) NCCM patients had early repolarizations; 13 (18%) was located in the lateral leads, two (3%) in inferior leads and four (5%) in both. None had early repolarizations in leads V1 to V3, like in Brugada syndrome. The SCD group had 6/11 (55%) early repolarizations (five in lateral leads, one in inferior leads and one in both), versus 12/63 (19%) in the non-SCD (p=0.02). The median age at first presentation in SCD group was 37 years, range [18–55], with 45 % males; four patients presented with VT and seven patients presented with VF. In the non-SCD group (median age 39 y, range [17–77], 48% male), in eight patients early repolarizations were located in lateral leads, one in inferior leads and three in both leads.
Conclusions: There is a high prevalence of early repolarizations in NCCM patients presenting with malignant ventricular events, including sudden cardiac arrest, with predominant localization in lateral leads. Therefore, early repolarizations may be useful high risk marker for malignant ventricular arrhythmias, including sudden cardiac death in NCCM patients.
- © 2010 by American Heart Association, Inc.