Clinical aspects of the idiopathic long QT syndrome.
Clinical aspects of the idiopathic long QT syndrome.
复制标题
特发性长 QT 综合征的临床方面。
DOI：
10.1111/j.1749-6632.1992.tb31006.x
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发表时间：
1992
影响因子：
5.2
通讯作者：
Robinson,JL
中科院分区：
文献类型：
--
作者：
Moss,AJ;Robinson,JL
The idiopathic Long QI' Syndrome (LWS) is an infrequently occurring disorder in which affected family members have an unusual electrocardiographic repolarization abnormality (QT prolongation) and a propensity to syncope and fatal ventricular For the most part, this idiopathic disorder has strong familial patterning suggesting a genetic or hereditary mechanism. Autosomal dominant3 and autosomal recessive4 modes of inheritance were suggested by the pattern of occurrence of this disorder in the first reported LQTS families. However, sporadic non-familial cases continue to appear and represent approximately 10% of the reported cases. The traditional criterion for the diagnosis of W prolongation is a heart rate corrected (Bazett's formula5) QT interval (Wc) greater than 0.44 sec. However, our recent studies on a large normal population6 indicate that the Qr interval is influenced by gender and age as well as heart rate (TABLE l), and these factors should be taken into consideration when diagnosing Qr prolongation. Our own suggested categorization of QTc is to use a three level classification (normal, borderline prolonged, and prolonged QTc) adjusted for age and gender (TABLE 1).In addition to the quantitative prolongation in the QT interval, the electrocardographic profile of the delayed repolarization T-wave is frequently unusual, with a spectrum of configurations. Examples of the delayed repolarization patterns observed in lead I1 of the electrocardiogram in over 300 fimilies with LQTS are schematized in FIGURE 1. The patterns include wide-
