儿茶酚胺多形性室性心动过速的临床处理：左侧心脏交感神经切除术的作用。
Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia: The Role of Left Cardiac Sympathetic Denervation.
机构信息
From Department of Cardiology and Cardiovascular Clinical Research Center (G.M.D.F., V.D.) and Division of Vascular Surgery (A.O.), Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; Department of Molecular Medicine, University of Pavia, Italy (G.M.D.T., V.D., C.S., L.C.); Center for Cardiac Arrhythmias of Genetic Origin, IRCCS Istituto Auxologico Italiano, Milano, Italy (C.S., L.C., P.J.S.); Departments of Medicine, Pediatrics, and Molecular Pharmacology & Experimental Therapeutics, Divisions of Cardiovascular Diseases and Pediatric Cardiology, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN (J.M.B., C.R.M., M.J.A.); Cardiac Arrhythmia Service, Department of Cardiology, Children's Hospital, Boston, MA (D.J.A.); Division of Cardiology, Children's National Heart Institute, George Washington University, Washington, DC (C.I.B.); Institute of Human Genetics, Helmholtz Zentrum München, Neuherberg, Germany (L.C.); Department of Cardiology, The Royal Children's Hospital, Murdoch Children's Research Institute and Melbourne University, Melbourne, Australia (A.M.D.); Heart Institute, Leviev Heart Center, Sheba Medical Center, Sackler School of Medicine, Tel Aviv University, Tel Hashomer, Israel (M.E.); Department of Clinical Cardiology and Molecular Genetics, National Center for Preventive Medicine, Ministry of Healthcare, Russian Federation, Moscow, Russia (M.K.); Bruce Rappaport Faculty of Medicine; Technion, Haifa, Israel (A.K.); Division of Cardiology, University of British Columbia, Vancouver, Canada (A.D.K.); AP-HP, Hôpital Bichat, Service de Cardiologie et Centre de Référence des Maladies Cardiaques Héréditaires, Paris, France (A.L.); Université Paris Diderot, Sorbonne Paris Cité, Paris, France (A.L.); Heart Centre AMC, Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands (L.O.N., A.A.M.W.); Department of Pediatric Cardiology and Intensive Care Medicine, University Hospital, Georg-August-University,
出版信息
Circulation. 2015 Jun 23;131(25):2185-93. doi: 10.1161/CIRCULATIONAHA.115.015731. Epub 2015 May 27.
BACKGROUND
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Βlockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal.
METHODS AND RESULTS
We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P<0.001) after LCSD, and among 29 patients with a presurgical ICD, the rate of shocks dropped by 93% from 3.6 to 0.6 shocks per person per year (P<0.001). Patients with an incomplete LCSD (n=7) were more likely to experience major cardiac events after LCSD (71% versus 17%; P<0.01) than those with a complete LCSD.
CONCLUSIONS
LCSD is an effective antifibrillatory intervention for patients with CPVT. Whenever syncope occurs despite optimal medical therapy, LCSD could be considered the next step rather than an ICD and could complement ICDs in patients with recurrent shocks.
背景
儿茶酚胺多形性室性心动过速（CPVT）是一种遗传疾病，每当交感神经活动增加时，都会导致危及生命的心律失常。β受体阻滞剂是治疗的主要方法；当它们失效时，植入式心脏复律除颤器（ICD）被使用，但经常导致多次电击。初步的氟卡尼结果令人鼓舞。我们提出左侧心脏交感神经切除术（LCSD）作为有用的辅助治疗，但证据仍然是轶事证据。
方法和结果
我们报告了 63 例接受 LCSD 的 CPVT 患者，其中 54 例为二级预防，9 例为一级预防。LCSD 后中位随访时间为 37 个月。9 例无症状患者无重大心脏事件。在 54 例有既往重大心脏事件的患者中，38 例在最佳药物治疗期间发生，16 例在最佳药物治疗期间未发生，其中 13 例（24%）至少有 1 次复发：0 例发生心脏骤停，2 例仅发生晕厥，10 例发生≥1 次适当的 ICD 放电，1 例患者猝死。1 年和 2 年的无事件生存率分别为 87%和 81%。尽管进行了最佳药物治疗，但接受 LCSD 后，有重大心脏事件的患者比例（n=38）从 100%降至 32%（P<0.001），在 29 例术前接受 ICD 的患者中，电击次数从每人每年 3.6 次降至 0.6 次，下降了 93%（P<0.001）。LCSD 不完全的患者（n=7）在接受 LCSD 后发生重大心脏事件的可能性高于 LCSD 完全的患者（71%比 17%；P<0.01）。
结论
LCSD 是 CPVT 患者有效的抗纤维颤动干预措施。尽管接受了最佳药物治疗，但如果仍出现晕厥，LCSD 可被视为下一步治疗方法，而不是 ICD，并可补充 ICD 在反复发作性电击的患者中。
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