Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA.
Am J Cardiol. 2011 Sep 1;108(5):729-34. doi: 10.1016/j.amjcard.2011.04.020. Epub 2011 Jun 22.
Adults with congenital heart disease are at risk of lethal ventricular arrhythmias and are candidates for implantable cardiac defibrillator (ICD) therapy, yet implant risks, long-term outcomes, and rates of appropriate and inappropriate ICD therapies are not well characterized. We reviewed clinical, implantation, and follow-up data on all transvenous ICDs in adults with congenital heart disease at the Mayo Clinic from 1991 through 2008. Seventy-three adults with congenital heart disease received 85 ICDs. Implantation diagnoses included tetralogy of Fallot (44%) and congenitally corrected transposition of the great arteries (17%). Implantation indication was occurrence of sustained ventricular arrhythmias (secondary prevention) in 36% and prophylactic (primary prevention) in the remainder. There were no major implant-related complications. During follow-up (2.2 ± 2.8 years, range 0 to 15) 11 patients died and 4 patients received heart or heart/lung transplants. An appropriate shock for a ventricular arrhythmia was observed in 19% of patients and an inappropriate shock was observed in 15% of patients. Likelihood of an appropriate shock was associated with increased subpulmonic ventricular pressure. In conclusion, implantation of transvenous ICDs in adults with congenital heart disease is associated with a low risk of implant complications. In this high-risk adult population the rate of inappropriate ICD shocks is low, whereas the likelihood of appropriate therapy for potentially lethal ventricular arrhythmias is high. These data suggest overall benefit of ICD therapy in adults with congenital heart disease.
成人先天性心脏病患者有发生致命性室性心律失常的风险，是植入式心脏复律除颤器（ICD）治疗的候选者，但植入风险、长期结果以及适当和不适当的 ICD 治疗的发生率尚不清楚。我们回顾了 1991 年至 2008 年梅奥诊所成人先天性心脏病患者所有经静脉 ICD 的临床、植入和随访数据。73 名成人先天性心脏病患者接受了 85 个 ICD。植入诊断包括法洛四联症（44%）和先天性矫正型大动脉转位（17%）。植入指征为 36%发生持续性室性心律失常（二级预防），其余为预防性（一级预防）。无重大植入相关并发症。在随访期间（2.2±2.8 年，范围 0 至 15 年），11 名患者死亡，4 名患者接受心脏或心肺移植。19%的患者观察到因室性心律失常而发生的适当电击，15%的患者观察到不适当的电击。适当电击的可能性与亚肺静脉压力增加有关。总之，在成人先天性心脏病患者中植入经静脉 ICD 的并发症风险较低。在这个高危成年人群中，不适当的 ICD 电击率较低，而发生潜在致命性室性心律失常时适当治疗的可能性较高。这些数据表明 ICD 治疗在成人先天性心脏病患者中总体有益。
