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Aims: Early repolarization (ER) has been linked to poorer outcomes in idiopathic ventricular fibrillation (IVF). The role of family screening in IVF is not clear. Our aim was to review predictors for poorer outcomes and evaluate the role of family screening in IVF., Methods and Results: This was a retrospective multicentre cohort study including all patients diagnosed with IVF. Data were collected on baseline characteristics, ECG findings, and recurrence of ventricular arrhythmia (VA) during follow-up. Electrocardiogram findings were reviewed in first-degree relatives that were screened. A total of 66 patients were included with male predominance (42/66, 64%) and Caucasian ethnicity (47/66, 71%). Mean age at cardiac arrest was 38 years ± 11. Thirty-one patients had ER (47%) predominantly with J-point amplitude ≥2 mm and horizontal ST segments (18/31, 58%). Recurrent VA was seen in 13 patients (20%). Horizontal ST segments were associated with increased rates of VA recurrence (OR 11, 95% CI 2.7-43.7; P = 0.0007). Early repolarization was seen in 20% of the 72 first-degree relatives and was more common if the proband had persistent ER pattern (OR 10.7, 95% CI 2.2-51.5; P = 0.003)., Conclusion: Ventricular arrhythmia recurrence was lower than previously reported. Early repolarization was common in this IVF cohort, and horizontal ST segments were suggestive predictor for poorer outcomes. Persistent ER in proband was associated with ER in first-degree relatives. With better understanding of its predictive value and the relationship to IVF, this information could potentially be used to guide family screening and identify new mutations using family members with persistent ER., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions, please email: journals.permissions@oup.com.)

Background: LQTS may cause sudden cardiac death (SCD), but the mechanisms linking gene mutations to ventricular fibrillation (VF) are unclear., Objective: To determine whether ventricular activation delays in congenital long QT syndrome (LQTS) are associated with VF and to describe these delays clinically by measuring activation through ventricular myocardium after a premature extrastimulus., Methods: Forty-six patients with LQTS, including 16 with VF (LQTS VF) were investigated, and the results were compared with those from 24 patients with hypertrophic cardiomyopathy and VF (HCM VF). Electrograms in response to premature stimuli were analyzed for increases in electrogram duration (DeltaED) and the S1S2 coupling intervals at which electrogram latency starts to increase (S1S2(delay)). Two piecewise continuous straight line segments were fitted to the last electrogram deflection as a function of S1S2 interval in the LQTS and HCM VF populations, and the difference in their gradient (alpha) was taken as an index of the abruptness of the onset of this delay., Results: Thirteen LQTS VF and six LQTS non-VF patients had values of DeltaED and S1S2(delay) comparable to those in HCM VF patients, while the remainder (three LQTS VF and 24 LQTS non-VF) had lower values (P<.001). There was only a weak correlation between delay and the corrected QT interval. The HCM and LQTS VF patients could be separated by the value of alpha (P<.01), with the LQTS patients having a more abrupt onset of delay., Conclusions: Large delays in ventricular activation after an extrastimulus occur in patients with the LQTS, especially those with VF. The change in delay is abrupt in the LQTS, indicating sudden block to activation creating a dynamic substrate for arrhythmogenesis.

Aims: Early repolarization (ER) has been linked to poorer outcomes in idiopathic ventricular fibrillation (IVF). The role of family screening in IVF is not clear. Our aim was to review predictors for poorer outcomes and evaluate the role of family screening in IVF.Methods and Results: This was a retrospective multicentre cohort study including all patients diagnosed with IVF. Data were collected on baseline characteristics, ECG findings, and recurrence of ventricular arrhythmia (VA) during follow-up. Electrocardiogram findings were reviewed in first-degree relatives that were screened. A total of 66 patients were included with male predominance (42/66, 64%) and Caucasian ethnicity (47/66, 71%). Mean age at cardiac arrest was 38 years ± 11. Thirty-one patients had ER (47%) predominantly with J-point amplitude ≥2 mm and horizontal ST segments (18/31, 58%). Recurrent VA was seen in 13 patients (20%). Horizontal ST segments were associated with increased rates of VA recurrence (OR 11, 95% CI 2.7-43.7; P = 0.0007). Early repolarization was seen in 20% of the 72 first-degree relatives and was more common if the proband had persistent ER pattern (OR 10.7, 95% CI 2.2-51.5; P = 0.003).Conclusion: Ventricular arrhythmia recurrence was lower than previously reported. Early repolarization was common in this IVF cohort, and horizontal ST segments were suggestive predictor for poorer outcomes. Persistent ER in proband was associated with ER in first-degree relatives. With better understanding of its predictive value and the relationship to IVF, this information could potentially be used to guide family screening and identify new mutations using family members with persistent ER. [ABSTRACT FROM AUTHOR]

Ventricular pre-excitation causing reversible left ventricular (LV) systolic dysfunction has been reported in adults and children. We describe severe heart failure secondary to ventricular pre-excitation in a 3-month-old girl who presented with echocardiographic evidence of marked intraventricular dyssynchrony. The patient was initially considered for transplantation, but dual chamber left atrioventricular epicardial pacing initiated at 4.5 months of age resulted in a marked clinical and echocardiographic improvement. Five years later, successful radiofrequency ablation resulted in loss of pre-excitation and reversal of LV dyssynchrony, thus allowing pacemaker explantation. To our knowledge, this is one of very few reported cases of pre-excitation–induced cardiomyopathy affecting a young infant and the only case palliated with resynchronization pacing. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of this study was to assess the prevalence of J-point elevation among the relatives of sudden arrhythmic death syndrome (SADS) probands. Background: J-point elevation is now known to be associated with idiopathic ventricular fibrillation. We hypothesized that this early repolarization phenomenon is an inherited trait responsible for a proportion of otherwise unexplained SADS cases. Methods: Families of SADS probands were evaluated in an inherited arrhythmia clinic. Twelve-lead electrocardiograms were analyzed for J-point elevation defined as >0.1 mV from baseline present in 2 or more of the inferior (II, III, and aVF) or lateral (1, aVL, V4 to V6) leads. Electrocardiographic data were compared with those of 359 controls of a similar age, sex, and ethnic distribution. Results: A total of 363 first-degree relatives from 144 families were evaluated. J-point elevation in the inferolateral leads was present in 23% of relatives and 11% of control subjects (odds ratio: 2.54, 95% confidence interval: 1.66 to 3.90; p < 0.001). Conclusions: J-point elevation is more prevalent in the relatives of SADS probands than in controls. This indicates that early repolarization is an important potentially inheritable pro-arrhythmic trait or marker of pro-arrhythmia in SADS. [Copyright &y& Elsevier]