DIAGNOSTIC-CRITERIA FOR THE LONG QT SYNDROME

DOI：

10.1161/01.cir.88.2.782

发表时间：

1993-08-01

期刊：

CIRCULATION

影响因子：

37.8

通讯作者：

中科院分区：

医学1区

文献类型：

作者：

SCHWARTZ, PJ;MOSS, AJ;CRAMPTON, RS

特发性长QT间期综合征(LQTS)是一种家族遗传频繁的先天性疾病，主要特征是QT间期延长和发生危及生命的快速性心律失常，特别是与情绪或身体应激有关。1-5在未经治疗的有症状的患者中，死亡率很高，首次晕厥后第一年死亡率为20%，10年内死亡率约为50%3；然而，不同家庭的死亡风险有所不同。通过使用药物或外科抗肾上腺素能治疗或两者兼用，这种不良的预后得到了显著改善，从而将长期死亡率降至5%。3、4、6这种经常致命的疾病有了有效的治疗方法，强调了早期准确诊断的重要性。不幸的是，LQTS的诊断经常延误，晕厥患者经常被误诊，最常见的是受到癫痫障碍的影响。在其最具特征的表现中，有明显的QT延长和应激性晕厥，对于意识到这种疾病的医生来说，LQTS的诊断相当简单。然而，在临界QT延长和/或没有症状的情况下，正确的诊断可能更困难。正是出于这个原因，1985.3提出了第一套诊断标准(表1)，该建议的主要优点是通过赋予主要和次要标准不同的权重，为LQTS的临床诊断提供了一种合乎逻辑和定量的方法。它的主要局限性是，它使用了传统的、但未用于诊断目的的临界值Qt；&gt；440毫秒‘2。这也导致了一种相当非黑即白的情况，在这种情况下，根据充满困难的测量中的几毫秒的差异，患者被判断为完全正常或异常的心室复极持续时间，例如服务器间的变异性。7.

T he idiopathic long QT syndrome (LQTS) is a congenital disease with frequent familial transmission, characterized primarily by prolongation of the QT interval and by the occurrence of life-threatening tachyarrhythmias, particularly in association with emotional or physical stress. 1-5 Among untreated symptomatic patients, lethality is high, with 20% mortality in the first year after the initial syncope and approximately 50% within 10years3; however, the risk of death varies among different families. This poor prognosis has been significantly improved by the use of pharmacological or surgical antiadrenergic therapy or both, which has reduced long-term mortality to< 5%. 3, 4, 6 The availability of effective therapy for this often lethal disease emphasizes the importance of early and accurate diagnosis. Unfortunately, there is frequently a delay in the diagnosis of LQTS, and patients with syncope are often misdiagnosed, most commonly as affected by a seizure disorder. In its most characteristicpresentation, with obvious QT prolongation and stress-induced syncope, the diagnosis of LQTS is quite straightforward for physicians aware of the disease. In cases of borderline QT prolongation and/or absence of symptoms, however, a correct diagnosis may be more difficult. It was for this reason that a first set of diagnostic criteria (Table 1) was proposed in 1985.3 The major merit of that proposal was that itprovided a logical andquantitative approach to the clinical diagnosis of LQTS by giving a different weight to major and minor criteria. Its major limitation was that it used thetraditional, but untested for diagnostic purposes, cutoff value of QT,> 440 msec'2. This also resulted in a rather black-and-white situation in which patients were judged to have an entirely normal or abnormal duration of ventricular repolarization on the basis of a difference of a few milliseconds in a measurement fraught with difficulties, such as interob-server variability. 7

DIAGNOSTIC-CRITERIA FOR THE LONG QT SYNDROME - AN UPDATE

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