Short QT syndrome - A familial cause of sudden death
Short QT syndrome - A familial cause of sudden death
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DOI：
10.1161/01.cir.0000085071.28695.c4
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发表时间：
2003-08-26
期刊：
影响因子：
37.8
通讯作者：
Borggrefe, M
中科院分区：
文献类型：
--
作者：
Gaita, F;Giustetto, C;Borggrefe, M
背景- QT间期延长与危及生命事件的风险相关。然而，对于QT间期过短对预后的影响知之甚少。在有心脏性猝死家族史的情况下，2个不同家族的几名成员因晕厥、心悸和复苏的心脏骤停被转诊。尸检没有发现任何结构性心脏病。所有患者心电图显示QT间期均持续短。方法和结果：来自两个家庭的6名患者接受了广泛的无创和有创检查，包括一系列静息心电图、超声心动图、心脏MRI、运动测试、动态心电图和信号平均心电图。6例患者中有4例进行了电生理评估，包括程序性心室刺激。所有受试者均排除结构性心脏病。基线心电图显示，所有患者QT间期小于或等于280 ms （QTc小于或等于300 ms）。在电生理研究中，所有患者的心房和心室不应期均较短，4例患者中有3例心室易感性增加。结论：短QT综合征以家族性猝死、短不应期和诱导性心室颤动为特征。认识到这种心电图模式是很重要的，因为它与年轻健康受试者猝死的高风险有关。
Background - A prolonged QT interval is associated with a risk for life-threatening events. However, little is known about prognostic implications of the reverse - a short QT interval. Several members of 2 different families were referred for syncope, palpitations, and resuscitated cardiac arrest in the presence of a positive family history for sudden cardiac death. Autopsy did not reveal any structural heart disease. All patients had a constantly and uniformly short QT interval at ECG.Methods and Results - Six patients from both families were submitted to extensive noninvasive and invasive work- up, including serial resting ECGs, echocardiogram, cardiac MRI, exercise testing, Holter ECG, and signal-averaged ECG. Four of 6 patients underwent electrophysiological evaluation including programmed ventricular stimulation. In all subjects, a structural heart disease was excluded. At baseline ECG, all patients exhibited a QT interval less than or equal to 280 ms (QTc less than or equal to 300 ms). During electrophysiological study, short atrial and ventricular refractory periods were documented in all and increased ventricular vulnerability to fibrillation in 3 of 4 patients.Conclusions - The short QT syndrome is characterized by familial sudden death, short refractory periods, and inducible ventricular fibrillation. It is important to recognize this ECG pattern because it is related to a high risk of sudden death in young, otherwise healthy subjects.