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Clinical implications for patients with long QT syndrome who experience a cardiac event during infancy
JACC - Journal of the American College of Cardiology, 08/24/09
Spazzolini C et al. – Pts with long QT syndrome (LQTS) and aborted cardiac arrest (ACA) during the first year of life are at very high risk for subsequent ACA or death during the next 10 yrs, and beta-blockers may not prevent fatal or near-fatal cardiac events in this small but high-risk subset.
Methods- Study of clinical and prognostic aspects of LQTS-related cardiac events in first year of life (infancy)
- Study population: 3,323 pts with QT interval corrected for heart rate (QTc) ≥450 ms in International LQTS Registry
- Assessment of 20 pts with sudden cardiac death (SCD), 16 with ACA, 34 with syncope, and 3,253 asymptomatic during infancy
- Risk factors for cardiac event among 212 pts with electrocardiogram in infancy: QTc ≥500 ms, heart rate ≤100 beats/min, and female sex
- ACA before age 1 yr associated with hazard ratio of 23.4 for ACA or SCD during ages 1-10 yrs
- During 10-yr follow-up after infancy, beta-blocker therapy associated with significant reduction in ACA/SCD only in pts with syncopal episode within 2 yrs before ACA/SCD but not for pts who survived ACA in infancy
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