Zareba W, Moss A J, le Cessie S, Locati E H, Robinson J L, Hall W J, Andrews M L
Department of Medicine, University of Rochester School of Medicine and Dentistry, New York 14642, USA.
J Am Coll Cardiol. 1995 Dec;26(7):1685-91. doi: 10.1016/0735-1097(95)60383-2.
This study sought to identify risk factors for cardiac events (syncope, aborted cardiac arrest or sudden cardiac death) in family members of patients with the long QT syndrome.
Patients with the long QT syndrome are known to be at high risk for cardiac events. Whenever the first member of a family is identified as having the long QT syndrome (proband), there is concern regarding the likelihood of cardiac events in other family members.
A multivariate logistic regression model was used to evaluate the risk of cardiac events in 637 family members who were first- and second-degree relatives of 151 probands with the long QT syndrome and in a subset of 513 family members who were not receiving beta-adrenergic blocking agents. There were 293 first-degree (46%) and 344 second-degree relatives (54%) (293 men [46%], 344 women [54%]). Fifteen percent of the family members had a corrected QT interval (QTc) > 0.44 s, and relative tachycardia and bradycardia were observed in 12% and 25%, respectively.
The risk of cardiac events occurring before age 40 in family members not taking beta-blockers was influenced by the QTc interval (odds ratio [OR] 1.18/0.01 increase in QTc value; 95% confidence interval [CI] 1.12 to 1.24), relative tachycardia (OR 2.21, 95% CI 0.97 to 5.02) or bradycardia (OR 2.24, 95% CI 1.10 to 4.56) and an interaction term combining gender and closeness of the relationship to the proband (OR for female first-degree relative 3.23 vs. all second-degree relatives, 95% CI 1.67-6.22).
Female first-degree relatives of patients with the long QT syndrome have a higher risk of cardiac events than male first- or second-degree relatives, independent of recorded electrocardiographic findings. Not only bradycardia, but also tachycardia increases risk of cardiac events in family members of patients with the long QT syndrome.
本研究旨在确定长QT综合征患者家庭成员发生心脏事件（晕厥、心脏骤停未遂或心源性猝死）的危险因素。
已知长QT综合征患者发生心脏事件的风险很高。每当一个家庭中的首个成员被确诊为长QT综合征（先证者）时，人们就会担心其他家庭成员发生心脏事件的可能性。
采用多因素逻辑回归模型，评估637名151例长QT综合征先证者的一级和二级亲属发生心脏事件的风险，以及513名未接受β肾上腺素能阻滞剂治疗的家庭成员亚组的风险。其中有293名一级亲属（46%）和344名二级亲属（54%）（293名男性[46%]，344名女性[54%]）。15%的家庭成员校正QT间期（QTc）>0.44秒，分别有12%和25%的人出现相对心动过速和心动过缓。
未服用β受体阻滞剂的家庭成员在40岁前发生心脏事件的风险受QTc间期（QTc值每增加0.01，优势比[OR]为1.18；95%置信区间[CI]为1.12至1.24）、相对心动过速（OR为2.21，95%CI为0.97至5.02）或心动过缓（OR为2.24，95%CI为1.10至4.56）以及性别与与先证者关系亲疏的交互项影响（女性一级亲属的OR为3.23，与所有二级亲属相比，95%CI为1.67 - 6.22）。
长QT综合征患者的女性一级亲属发生心脏事件的风险高于男性一级或二级亲属，与记录的心电图结果无关。不仅心动过缓，心动过速也会增加长QT综合征患者家庭成员发生心脏事件的风险。
