Grubb Alex F, Shah Gautam, Aziz Peter F, Krasuski Richard A
Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH.
Department of Internal Medicine, Cleveland Clinic Health System, Cleveland, OH.
J Innov Card Rhythm Manag. 2017 Apr 15;8(4):2658-2664. doi: 10.19102/icrm.2017.080405. eCollection 2017 Apr.
Transposition of the great arteries (TGA) is represented in 5% to 7% of patients with congenital heart disease. These patients face a significant burden of arrhythmia and sudden cardiac death throughout their lives, and many eventually undergo pacemaker or cardiac-defibrillator implantation. Outcomes data following device implantation in this population, however, are limited. From an electrophysiologic database at a large, tertiary care medical center, we identified 63 TGA patients (34 with dextro (d)-TGA and 29 with levo (l)-TGA) with systemic right ventricles receiving an implantable cardiac device from 1996 to 2014. Medical records were reviewed for demographic, echocardiography and device interrogation data. Overall, l-TGA patients were older than d-TGA patients when they underwent initial device implantation (35.6 ± 18.2 versus 17.3 ± 10.6 years, p<0.001), and had more concomitant cardiac defects (55% versus 12%, p<0.001). Survival following initial device implantation was similar between l-TGA and d-TGA (72% versus 74%, p = 1.00), despite the baseline difference in age. Twenty-four patients underwent implantable cardioverter-defibrillator (ICD) implantation: 18 for primary intervention (11 l-TGA and seven d-TGA), and six for secondary prevention (four l-TGA and two d-TGA). Sixty-seven percent of patients in the secondary prevention group had appropriate shocks, compared with 0% of primary prevention patients. Patients with ICD discharge were more likely to have concomitant heart defects (100% versus 30%, p = 0.011). Despite being significantly younger, d-TGA patients had similar survival rates following device implant to l-TGA patients. Patients with TGA and sustained ventricular arrhythmias are at high risk for subsequent events, and typically benefit from ICD implantation. The role of prophylactic ICD implantation in this population, however, remains uncertain.
大动脉转位（TGA）在先天性心脏病患者中占5%至7%。这些患者一生中面临着心律失常和心源性猝死的重大负担，许多人最终接受了起搏器或心脏除颤器植入。然而，该人群植入装置后的结局数据有限。我们从一家大型三级医疗中心的电生理数据库中，识别出63例具有体循环右心室的TGA患者（34例右位（d）-TGA和29例左位（l）-TGA），他们在1996年至2014年期间接受了植入式心脏装置。对病历进行了回顾，以获取人口统计学、超声心动图和装置问询数据。总体而言，l-TGA患者在首次植入装置时比d-TGA患者年龄更大（35.6±18.2岁对17.3±10.6岁，p<0.001），且合并心脏缺陷更多（55%对12%，p<0.001）。尽管年龄存在基线差异，但l-TGA和d-TGA患者首次植入装置后的生存率相似（72%对74%，p = 1.00）。24例患者接受了植入式心律转复除颤器（ICD）植入：18例为一级干预（11例l-TGA和7例d-TGA），6例为二级预防（4例l-TGA和2例d-TGA）。二级预防组67%的患者有适当电击，而一级预防患者为0%。有ICD放电的患者更可能合并心脏缺陷（100%对30%，p = 0.011）。尽管d-TGA患者年龄明显更小，但装置植入后的生存率与l-TGA患者相似。患有TGA和持续性室性心律失常的患者后续事件风险很高，通常从ICD植入中获益。然而，预防性ICD植入在该人群中的作用仍不确定。
