Adamos Georgios, Iacovidou Nicoletta, Xanthos Theodoros
National and Kapodistrian University of Athens, Medical School, Athens, Greece.
Hellenic Society of Cardiopulmonary Resuscitation.
Mini Rev Med Chem. 2018;18(6):495-506. doi: 10.2174/1389557517666170707110000.
Long QT syndrome (LQTS) is an arrhythmogenic disorder characterized by repolarization abnormalities with a tendency to cause life threatening cardiac events. The first manifestation of the syndrome may be sudden death, therefore, early diagnosis and therapy is of great importance. LQTS can be congenital or acquired. The latter is most commonly seen in hospitalized patients and such individuals have an easily recognizable and reversible precipitating factor (electrolyte disturbances, certain drugs etc.). Congenital LQTS, a less prevalent condition, is considered to be part of the greater family of disorders called channelopathies. Extensive research over the past decade allowed better understanding of the underlying molecular mechanisms and unmasking of the genetic substrate of these patients. Both pharmacological and interventional therapies (left cardiac sympathetic denervation, ICDs) have been used in the treatment of congenital long-QT syndrome. This review will focus solely on the pharmacologic therapy of this potentially lethal inherited disorder summarizing current knowledge regarding b-blocker use, taking into account novel and more targeted approaches such as gene-specific therapy.
长QT综合征（LQTS）是一种致心律失常性疾病，其特征为复极异常，易引发危及生命的心脏事件。该综合征的首发表现可能是猝死，因此，早期诊断和治疗至关重要。LQTS可分为先天性和后天性。后者在住院患者中最为常见，这类患者有易于识别且可逆转的诱发因素（电解质紊乱、某些药物等）。先天性LQTS相对少见，被认为是一大类称为离子通道病的疾病家族的一部分。过去十年的广泛研究使人们对其潜在分子机制有了更好的理解，并揭示了这些患者的遗传基础。药物治疗和介入治疗（左心交感神经去神经术、植入式心脏复律除颤器）均已用于先天性长QT综合征的治疗。本综述将仅聚焦于这种潜在致命性遗传性疾病的药物治疗，总结关于β受体阻滞剂使用的现有知识，并考虑基因特异性治疗等新颖且更具针对性的方法。
