Mönnig Gerold, Köbe Julia, Löher Andreas, Eckardt Lars, Wedekind Horst, Scheld Hans H, Haverkamp Wilhelm, Milberg Peter, Breithardt Günter, Schulze-Bahr Eric, Böcker Dirk
Department of Cardiology and Angiology, University Hospital Münster, Münster, Germany.
Heart Rhythm. 2005 May;2(5):497-504. doi: 10.1016/j.hrthm.2005.02.008.
The purpose of this study was to evaluate retrospectively a single-center experience with the use of ICDs in patients with long QT syndrome (LQTS) concerning outcome, complications, and optimal programming.
Use of implantable cardioverter-defibrillator (ICD) in patients with congenital LQTS is controversial but is generally accepted in high-risk patients.
We enrolled 27 symptomatic patients with LQTS undergoing ICD therapy (QTc 540 +/- 64 ms(1/2); 85% female, 63% cardiac arrest; 33% syncope despite beta-blockers; 4% with severe phenotype) and 81 genotyped patients with LQTS undergoing conventional drug therapy (28 LQT1, 39 LQT2, 1 LQT3, 13 LQT5). During a mean follow-up of 65 +/- 34 months, one death occurred in the ICD group that was not LQTS related. A total of 178 appropriate shocks were observed in 10/27 patients (37%), mostly in survivors of cardiac arrest (in 58% of cardiac arrest patients vs. in 20% of non-cardiac arrest patients).
In a logistic regression analysis, only QTc interval (121/178 shocks (68%) for QTc > 500 ms(1/2)) and "survived cardiac arrest" were prognostic for ICD shocks. In 30% of patients in the ICD group, multiple shocks occurred and could be reduced after increase of antibradycardia pacing rate, adding beta-blocker therapy, or starting the rate-smoothing algorithm (average 7.1 shocks before to 0.75 shocks after additional intervention annually).
ICD therapy is a safe and useful tool in high-risk patients with LQTS. QTc interval and cardiac arrest survivors were prognostic factors for appropriate ICD shocks. The results of this large single-center experience suggest that beta-blockers should always be added to ICD therapy. In addition, some patients might benefit from additional antibradycardia pacing, prolonged detection time, and a rate-smoothing algorithm to prevent recurrent episodes.
本研究旨在回顾性评估单中心使用植入式心脏复律除颤器（ICD）治疗长QT综合征（LQTS）患者的疗效、并发症及最佳程控情况。
先天性LQTS患者使用植入式心脏复律除颤器（ICD）存在争议，但在高危患者中普遍被接受。
我们纳入了27例接受ICD治疗的有症状LQTS患者（QTc为540±64毫秒（1/2）；85%为女性，63%曾发生心脏骤停；33%尽管使用了β受体阻滞剂仍有晕厥；4%为严重表型）以及81例接受传统药物治疗的基因分型LQTS患者（28例LQT1型，39例LQT2型，1例LQT3型，13例LQT5型）。在平均65±34个月的随访期间，ICD组有1例死亡，与LQTS无关。共观察到10/27例患者（37%）发生了178次恰当电击，多数发生在心脏骤停幸存者中（58%的心脏骤停患者 vs. 20%的非心脏骤停患者）。
在逻辑回归分析中，只有QTc间期（QTc>500毫秒（1/2）时，178次电击中有121次（68%））和“曾发生心脏骤停”是ICD电击的预后因素。ICD组30%的患者发生了多次电击，在提高抗心动过缓起搏频率、加用β受体阻滞剂治疗或启动心率平滑算法后（每年额外干预前平均7.1次电击，干预后为0.75次电击），电击次数可减少。
ICD治疗是高危LQTS患者安全有效的工具。QTc间期和心脏骤停幸存者是恰当ICD电击的预后因素。这项大型单中心研究结果表明，ICD治疗应始终联合β受体阻滞剂。此外，一些患者可能受益于额外的抗心动过缓起搏、延长检测时间及心率平滑算法以预防复发。
