Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy.
Department of Biomedical Informatics, University of Arizona College of Medicine, Phoenix, Arizona; AZCERT, Inc., Oro Valley, Arizona.
J Am Coll Cardiol. 2016 Apr 5;67(13):1639-1650. doi: 10.1016/j.jacc.2015.12.063.
Drug-induced long QT syndrome (diLQTS) and congenital LQTS (cLQTS) share many features, and both syndromes can result in life-threatening torsades de pointes (TdP). Our understanding of their mechanistic and genetic similarities has led to their improved clinical management. However, our inability to prevent diLQTS has resulted in removal of many medicines from the market and from development. Genetic and clinical risk factors for diLQTS and TdP are well known and raise the possibility of TdP prevention. Clinical decision support systems (CDSS) can scan the patient's electronic health records for clinical risk factors predictive of diLQTS and warn when a drug that can cause TdP is prescribed. CDSS have reduced prescriptions of QT-prolonging drugs, but these relatively small changes lack the power to reduce TdP. The growing genetic evidence linking diLQTS to cLQTS suggests that prevention of TdP in the future may require inclusion of both genetic and clinical predictors into CDSS.
药物诱导的长 QT 综合征（diLQTS）和先天性长 QT 综合征（cLQTS）有许多共同特征，这两种综合征均可导致危及生命的尖端扭转型室性心动过速（TdP）。我们对其机制和遗传相似性的理解，使得它们的临床管理得到了改善。然而，我们无法预防 diLQTS，导致许多药物从市场上和研发中被撤出。diLQTS 和 TdP 的遗传和临床危险因素众所周知，这增加了 TdP 预防的可能性。临床决策支持系统（CDSS）可以扫描患者的电子健康记录，寻找预测 diLQTS 的临床危险因素，并在开具可能导致 TdP 的药物时发出警告。CDSS 减少了 QT 延长药物的处方，但这些相对较小的变化缺乏降低 TdP 的能力。越来越多的遗传证据将 diLQTS 与 cLQTS 联系起来，这表明未来预防 TdP 可能需要将遗传和临床预测因素纳入 CDSS。
