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Evolution in Managing Long QT Syndrome,Journal of the American College of Cardiology
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SEE PAGE 453 F orty years ago, the number of patients diagnosed as affected by long QT syndrome (LQTS) was very small (1), and even the best cardiology centers had seen only a handful of them. Almost nothing was known about the natural history and the response to therapy. As evidence, we may recall that Dirk Durrer (a pioneer of cardiac electrophysiology) wrote to Conor Ward (of the Romano-Ward syndrome) “I am pessimistic about the possibility to control the syndrome in any way,” and that in the early 1970s, one of the initially favored therapies was digitalis (1). Therefore, the only way to understand these aspects, critical for any “newly discovered” disease, was to establish an International Registry (2–4). Besides paving the way to the genetic discoveries (4), the fruits of the LQTS registry were truly significant. They showed, for instance, that the risk for major cardiac events (syncope, cardiac arrest, and sudden death) is higher when the QT interval is longer, when a syncope has already occurred, and among women especially after puberty, and that ßblocker therapy is very effective (5,6). The registry was the only way to make progress at a time when the diagnosed cases were so few, but it contained the germ of a problem. Moss in the United States and Schwartz in Europe, but covering also Asia and Africa, were sending out forms to the many physicians who had either published 1 or a few cases or had inquired
中文翻译：
参见第 453 页 四十年前，被诊断为长 QT 综合征 (LQTS) 的患者数量非常少 (1)，即使是最好的心脏病学中心也只见过少数患者。关于自然史和对治疗的反应几乎一无所知。作为证据，我们可能记得 Dirk Durrer（心脏电生理学的先驱）写信给 Conor Ward（Romano-Ward 综合征）“我对以任何方式控制该综合征的可能性持悲观态度”，并且在 1970 年代初期，最初受青睐的疗法之一是洋地黄 (1)。因此，了解这些对任何“新发现”疾病至关重要的方面的唯一方法是建立国际登记处 (2-4)。除了为基因发现 (4) 铺平道路之外，LQTS 登记的成果也确实意义重大。例如，他们展示了 当 QT 间期较长时，当晕厥已经发生时，尤其是在青春期后的女性中，主要心脏事件（晕厥、心脏骤停和猝死）的风险更高，并且 ß 阻滞剂治疗非常有效（5， 6). 在诊断出的病例很少的时候，登记是取得进展的唯一途径，但它包含了问题的根源。美国的 Moss 和欧洲的 Schwartz（但也覆盖亚洲和非洲）正在向许多发表了 1 个或几个病例或咨询过的医生发送表格 在诊断出的病例很少的时候，登记是取得进展的唯一途径，但它包含了问题的根源。美国的 Moss 和欧洲的 Schwartz（但也覆盖亚洲和非洲）正在向许多发表了 1 个或几个病例或咨询过的医生发送表格 在诊断出的病例很少的时候，登记是取得进展的唯一途径，但它包含了问题的根源。美国的 Moss 和欧洲的 Schwartz（但也覆盖亚洲和非洲）正在向许多发表了 1 个或几个病例或咨询过的医生发送表格 |
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