Sydney Medical School, The University of Sydney, Camperdown, Australia.
Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, Australia.
Pacing Clin Electrophysiol. 2020 Jul;43(7):746-753. doi: 10.1111/pace.13974. Epub 2020 Jun 11.
Sudden cardiac death (SCD) accounts for up to 25% of deaths in the adult congenital heart disease (ACHD) population. Current guidelines for defibrillator implantation are either extrapolated from acquired cardiac disease or are based upon single lesion studies, predominantly Tetralogy of Fallot (TOF). Defibrillator-related morbidity appears to be substantially higher in ACHD patients.
We retrospectively evaluated all patients in our ACHD database who received an implantable cardioverter-defibrillator (ICD) between 2000 and 2019, and who were ≥16 years old at time of implant. Patients were followed for appropriate shocks, inappropriate shocks, and complications.
Of 4748 patients in our database, 59 patients (1.2%) underwent ICD implantation. ICDs were for primary prevention in 63% and secondary prevention in 37%. Over a median follow-up of 6.6 years, 24% received an appropriate shock, 27% an inappropriate shock, and 42% suffered a device-related complication (annualized risks of 3.2%, 3.6%, and 5.7%, respectively). There were no significant predictors of appropriate shocks or inappropriate shocks. All appropriate shocks in primary prevention patients occurred in TOF or transposition of the great arteries (TGA) with atrial switch, typically in the presence of multiple SCD risk factors. The majority of inappropriate shocks were due to supraventricular arrhythmias. Device-related mortality was 1.7% (0.3% per annum).
Appropriate shocks were relatively common in an ACHD ICD population followed in the long term. Device-related morbidity was significant. Although risk factors have been established for TOF, and to a lesser extent TGA, risk stratification for ICD implantation in ACHD remains challenging.
在成人先天性心脏病（ACHD）患者中，心源性猝死（SCD）占死亡人数的 25%。目前的除颤器植入指南要么是从后天性心脏病中推断出来的，要么是基于单一病变研究，主要是法洛四联症（TOF）。在 ACHD 患者中，与除颤器相关的发病率似乎要高得多。
我们回顾性评估了我们的 ACHD 数据库中所有在 2000 年至 2019 年间植入植入式心脏复律除颤器（ICD）的患者，且植入时年龄≥16 岁。对患者进行了适当电击、不适当电击和并发症的随访。
在我们的数据库中，4748 名患者中有 59 名（1.2%）接受了 ICD 植入。ICD 植入的适应证为一级预防 63%，二级预防 37%。在中位随访 6.6 年后，24%的患者发生了适当电击，27%的患者发生了不适当电击，42%的患者发生了与器械相关的并发症（年发生率分别为 3.2%、3.6%和 5.7%）。没有适当电击或不适当电击的显著预测因素。在一级预防患者中，所有适当电击都发生在 TOF 或大动脉转位（TGA）伴心房转换，通常存在多个 SCD 危险因素。大多数不适当电击是由于室上性心律失常引起的。与器械相关的死亡率为 1.7%（0.3%/年）。
在长期随访的 ACHD ICD 患者中，适当电击较为常见。与器械相关的发病率很高。尽管已经确定了 TOF 的危险因素，而且在一定程度上也确定了 TGA 的危险因素，但在 ACHD 中进行 ICD 植入的风险分层仍然具有挑战性。
