Intentional Non-Therapy in Long QT Syndrome (LQTS).
Intentional Non-Therapy in Long QT Syndrome (LQTS).
复制标题
长 QT 综合征 (LQTS) 的故意不治疗。
DOI：
10.1016/j.hrthm.2020.02.017
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发表时间：
2020
期刊：
影响因子：
5.5
通讯作者：
M. Ackerman
中科院分区：
文献类型：
--
作者：
C. Macintyre;Ram K. Rohatgi;A. Sugrue;J. Bos;M. Ackerman
背景国际指南建议对长QT综合征的治疗采用I类(有症状或QTcTc470ms)或II类(无症状和QTc470ms)。目的本研究的目的是评估采用仅观察(有意非治疗)策略的长QT间期综合征(≥)患者的疗效。结果在661例LQTS患者中，55例(8.3%)无症状患者(女性53%；年龄16岁；18岁)接受了有意的非治疗。只建议采取预防措施。平均发病年龄为37.8±21.2岁。平均QTc448±30ms。在平均7.5±4.3年的随访期内，无一例发生LQTS引发的心脏事件。与较大的治疗队列相比，这个故意未治疗的队列症状较少，确诊时年龄较大，静息QTc值较低(P<；.0001)。结论在仔细的临床评估、风险评估和预防措施制定后，可以考虑在临床特征包括无症状状态、诊断时年龄较大和QTC<；470ms的LQTS患者中考虑仅观察策略，与使用β-受体阻滞剂治疗的LQTS患者相比，该患者具有良好的结果和更好的生活质量。具有这种低风险特征的LQTS患者不应接受预防性植入性心律转复除颤器。
BackgroundInternational guidelines advise universal beta-blocker therapy as either a class I (symptomatic or QTc ≥470 ms) or class II (asymptomatic and QTc <470 ms) recommendation for treatment of long QT syndrome (LQTS).ObjectiveThe purpose of this study was to evaluate the outcomes of a highly selected cohort of patients with LQTS managed with an observation-only (intentional nontreatment) strategy.MethodsThe cohort was derived from a comprehensive retrospective registry of patients with LQTS. Clinical phenotype and genotype data were collected via review of electronic health records.ResultsAmong 661 patients with LQTS, 55 (8.3%) asymptomatic patients (53% female; 16 age <18 years) were managed with intentional nontherapy. Only preventative measures were advised. Mean age at diagnosis was 37.8 ± 21.2 years. Mean QTc was 448 ± 30 ms. None of the patients experienced an LQTS-triggered cardiac event over mean follow-up of 7.5 ± 4.3 years. Compared to the larger treated cohort, this intentionally untreated cohort was less symptomatic, was older at diagnosis, and had lower resting QTc values (P<.0001).ConclusionAfter careful clinical evaluation, risk assessment, and institution of precautionary measures, an observation-only strategy may be considered in a highly selected group of LQTS patients with a clinical profile that includes asymptomatic status, older age at diagnosis, and QTc <470 ms, with excellent outcomes and better quality of life than LQTS patients treated with beta-blocker. LQTS patients with this low-risk profile should not receive a prophylactic implantable cardioverter–defibrillator.
影响因子：
37.8
作者：
Moss, AJ;Zareba, W;Andrews, ML
通讯作者：
Andrews, ML
影响因子：
24
作者：
Goldenberg I;Horr S;Moss AJ;Lopes CM;Barsheshet A;McNitt S;Zareba W;Andrews ML;Robinson JL;Locati EH;Ackerman MJ;Benhorin J;Kaufman ES;Napolitano C;Platonov PG;Priori SG;Qi M;Schwartz PJ;Shimizu W;Towbin JA;Vincent GM;Wilde AA;Zhang L
通讯作者：
Zhang L