Wang L X
School of Biomedical Sciences, Charles Sturat University, Wagga Wagga, NSW 2678, Australia.
J Postgrad Med. 2003 Apr-Jun;49(2):179-81.
Congenital long QT syndrome (LQTS) is a rare but life-threatening disorder affecting cardiac electrophysiology. It occurs due to mutation in genes encoding for the ion channels in ventricular cell membrane. Syncopal attacks and cardiac arrest are the main symptoms of the disease. Anti-adrenergic therapy with oral beta-blockers has been the mainstay of treatment for LQTS. However, up to 30% of patients fail to respond to medical therapy and remain symptomatic. An alarming 10% of patients still experience cardiac arrest or sudden cardiac death during the course of therapy. Left cardiac sympathetic denervation (LCSD) has been used as an alternative therapy in patients who are resistant to beta-blockers. Although LCSD appears effective in reducing the frequency of syncopal attacks and improving the survival rate in both the short and long-term, its use has not gained popularity. The recent advent of minimally invasive thoracoscopic sympathectomy may improve the acceptance of LCSD by physicians and patients in the future. The primary objective of this article was to review the current evidence of the clinical efficacy and safety of LCSD in the management of LQTS. The review was based on Medline search of articles published between 1966 and 2002.
先天性长QT综合征（LQTS）是一种罕见但危及生命的疾病，影响心脏电生理。它是由于心室细胞膜离子通道编码基因的突变而发生的。晕厥发作和心脏骤停是该疾病的主要症状。口服β受体阻滞剂的抗肾上腺素能治疗一直是LQTS治疗的主要方法。然而，高达30%的患者对药物治疗无反应且仍有症状。令人担忧的是，10%的患者在治疗过程中仍会发生心脏骤停或心源性猝死。左心交感神经去神经支配术（LCSD）已被用作对β受体阻滞剂耐药患者的替代治疗方法。尽管LCSD在短期和长期内似乎都能有效降低晕厥发作频率并提高生存率，但其应用并不广泛。最近出现的微创胸腔镜交感神经切除术可能会在未来提高医生和患者对LCSD的接受度。本文的主要目的是回顾LCSD在LQTS治疗中临床疗效和安全性的现有证据。该综述基于对1966年至2002年间发表的文章进行的Medline检索。
