TY - JOUR
T1 - Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy
AU - Spazzolini, Carla
AU - Mullally, Jamie
AU - Moss, Arthur J.
AU - Schwartz, Peter J.
AU - McNitt, Scott
AU - Ouellet, Gregory
AU - Fugate, Thomas
AU - Goldenberg, Ilan
AU - Jons, Christian
AU - Zareba, Wojciech
AU - Robinson, Jennifer L.
AU - Ackerman, Michael J.
AU - Benhorin, Jesaia
AU - Crotti, Lia
AU - Kaufman, Elizabeth S.
AU - Locati, Emanuela H.
AU - Qi, Ming
AU - Napolitano, Carlo
AU - Priori, Silvia G.
AU - Towbin, Jeffrey A.
AU - Vincent, G. Michael
PY - 2009/8/25
Y1 - 2009/8/25
N2 - Objectives: This study was designed to evaluate the clinical and prognostic aspects of long QT syndrome (LQTS)-related cardiac events that occur in the first year of life (infancy). Background: The clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously. Methods: The study population of 3,323 patients with QT interval corrected for heart rate (QTc) ≥450 ms enrolled in the International LQTS Registry involved 20 patients with sudden cardiac death (SCD), 16 patients with aborted cardiac arrest (ACA), 34 patients with syncope, and 3,253 patients who were asymptomatic during the first year of life. Results: The risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc ≥500 ms, heart rate ≤100 beats/min, and female sex. An ACA before age 1 year was associated with a hazard ratio of 23.4 (p <0.01) for ACA or SCD during ages 1 to 10 years. During the 10-year follow-up after infancy, beta-blocker therapy was associated with a significant reduction in ACA/SCD only in those with a syncopal episode within 2 years before ACA/SCD but not for those who survived ACA in infancy. Conclusions: Patients with LQTS who experience ACA during the first year of life are at very high risk for subsequent ACA or death during their next 10 years of life, and beta-blockers might not be effective in preventing fatal or near-fatal cardiac events in this small but high-risk subset.
AB - Objectives: This study was designed to evaluate the clinical and prognostic aspects of long QT syndrome (LQTS)-related cardiac events that occur in the first year of life (infancy). Background: The clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously. Methods: The study population of 3,323 patients with QT interval corrected for heart rate (QTc) ≥450 ms enrolled in the International LQTS Registry involved 20 patients with sudden cardiac death (SCD), 16 patients with aborted cardiac arrest (ACA), 34 patients with syncope, and 3,253 patients who were asymptomatic during the first year of life. Results: The risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc ≥500 ms, heart rate ≤100 beats/min, and female sex. An ACA before age 1 year was associated with a hazard ratio of 23.4 (p <0.01) for ACA or SCD during ages 1 to 10 years. During the 10-year follow-up after infancy, beta-blocker therapy was associated with a significant reduction in ACA/SCD only in those with a syncopal episode within 2 years before ACA/SCD but not for those who survived ACA in infancy. Conclusions: Patients with LQTS who experience ACA during the first year of life are at very high risk for subsequent ACA or death during their next 10 years of life, and beta-blockers might not be effective in preventing fatal or near-fatal cardiac events in this small but high-risk subset.
KW - genetics
KW - infants
KW - long QT syndrome
KW - risk stratification
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U2 - 10.1016/j.jacc.2009.05.029
DO - 10.1016/j.jacc.2009.05.029
M3 - Article
C2 - 19695463
AN - SCOPUS:68749107301
VL - 54
SP - 832
EP - 837
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
SN - 0735-1097
IS - 9
ER -