Age-corrected QRS duration was significantly associated with lethal and cardiac event-free survival in patients with the SCN5A-E1784K variant, among whom 6% experienced a lethal event.
Observational (n=231)
Yes
Ventricular conduction parameters, specifically age-corrected QRS duration, serve as important risk markers for lethal and cardiac events in patients with the SCN5A-E1784K variant.
Abstract Background and Aims SCN5A-E1784K (c.5350GA) is the most common variant associated with the long QT (LQTS) and Brugada syndromes (BrS). It can manifest variably as LQTS, BrS and/or conduction disorders. This presents a challenge for risk stratification. We aimed to describe clinical and ECG characteristics and identify risk markers that associate with arrhythmic events. Methods We undertook a retrospective observational multicentre study of a large cohort of 231 subjects with SCN5A-E1784K from Europe, USA and Japan. Comprehensive demographic and clinical data, including initial presentation ECG and follow-up, were collected. ‘Lethal events’ were defined as sudden death, non-fatal cardiac arrest, and documented sustained VT or VF. ‘Cardiac events’ were defined as arrhythmic syncope plus any lethal events. Clinical characteristics and ECG parameters corrected for age were investigated for association with lethal and/or cardiac events. Results Fourteen (6%) subjects experienced a lethal event and 45 (19%) a cardiac event. PR interval and QRS duration were associated with lethal and cardiac events. In multivariable models, both PR interval and QRS duration were associated with lethal events but only QRS duration was associated with cardiac events. Only age-corrected QRS (rQRS) was associated with lethal and cardiac event-free survival from birth after correction for multiple testing. Conclusion Ventricular myocardial conduction appears likely to play a role in the risk of arrhythmic events in patients with SCN5A-E1784K. This provides an important opportunity for the personalisation of management and has the potential to guide preventative therapies.
Tanck et al. (Sat,) conducted a observational in SCN5A-E1784K overlap sodium channel disease (n=231). PR interval and QRS duration was evaluated on Lethal events (sudden death, non-fatal cardiac arrest, sustained VT/VF) and cardiac events. Age-corrected QRS duration was significantly associated with lethal and cardiac event-free survival in patients with the SCN5A-E1784K variant, among whom 6% experienced a lethal event.
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