Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome
Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome
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DOI：
10.1161/circulationaha.107.701243
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发表时间：
2008-04-29
期刊：
影响因子：
37.8
通讯作者：
Zhang, Li
中科院分区：
文献类型：
--
作者：
Goldenberg, Ilan;Moss, Arthur J.;Zhang, Li
先天性长QT综合征（LQTS）是无器质性心脏病儿童心脏性猝死的重要原因。然而，这种遗传性disorder.Methods和结果-考克斯比例风险回归模型的儿童危及生命的心脏事件的具体危险因素尚未被确定，以确定流产的心脏骤停或心脏性猝死的危险因素在3015 LQTS儿童从国际LQTS注册谁被随访1至12岁。男孩联合终点的累积概率（5%）显著高于女孩（1%; P < 0.001）。儿童期心脏骤停或心源性猝死的危险因素包括校正的QT间期[QTc]> 500 ms（风险比[HR]; 2.72; 95%置信区间[CI]，1.50 - 4.92; P = 0.001）和既往晕厥（近期晕厥[< 2年]：HR，6.16; 95% CI，3.41 - 11.15; P < 0.001;远期晕厥[>= 2年]：HR，2.67; 95% CI，1.22 - 5.85; P = 0.01），而既往晕厥是女孩中唯一显著的危险因素（近期晕厥：HR，27.82; 95% CI，9.72 ~ 79.60; P < 0.001;远期晕厥：HR，12.04; 95% CI，3.79 ~ 38.26; P < 0.001）。β-受体阻滞剂治疗与心脏骤停或心脏性猝死风险显著降低53%相关（P = 0.01）。结论- LQTS男孩在儿童期发生致命或近致命心脏事件的比率显著高于女孩。QTc间期> 500 ms和既往晕厥史可识别男孩的风险，而既往晕厥是女孩中唯一重要的风险因素。β受体阻滞剂治疗与儿童期危及生命的心脏事件风险显著降低相关。
Background - The congenital long-QT syndrome (LQTS) is an important cause of sudden cardiac death in children without structural heart disease. However, specific risk factors for life-threatening cardiac events in children with this genetic disorder have not been identified.Methods and Results - Cox proportional-hazards regression modeling was used to identify risk factors for aborted cardiac arrest or sudden cardiac death in 3015 LQTS children from the International LQTS Registry who were followed up from 1 through 12 years of age. The cumulative probability of the combined end point was significantly higher in boys (5%) than in girls (1%; P < 0.001). Risk factors for cardiac arrest or sudden cardiac death during childhood included corrected QT interval [QTc] duration > 500 ms (hazard ratio [HR]; 2.72; 95% confidence interval [CI], 1.50 to 4.92; P = 0.001) and prior syncope (recent syncope [< 2 years]: HR, 6.16; 95% CI 3.41 to 11.15; P < 0.001; remote syncope [>= 2 years]: HR, 2.67; 95% CI, 1.22 to 5.85; P = 0.01) in boys, whereas prior syncope was the only significant risk factor among girls (recent syncope: HR, 27.82; 95% CI, 9.72 to 79.60; P < 0.001; remote syncope: HR, 12.04; 95% CI, 3.79 to 38.26; P < 0.001). beta-Blocker therapy was associated with a significant 53% reduction in the risk of cardiac arrest or sudden cardiac death (P = 0.01).Conclusions - LQTS boys experience a significantly higher rate of fatal or near-fatal cardiac events than girls during childhood. A QTc duration > 500 ms and a history of prior syncope identify risk in boys, whereas prior syncope is the only significant risk factor among girls. beta-Blocker therapy is associated with a significant reduction in the risk of life-threatening cardiac events during childhood.