| Summary: | Aims: Sudden cardiac death (SCD) is a major cause of mortality in adults with congenital heart disease (ACHD). The role of implantable cardioverter-defibrillator (ICDs) in preventing SCD has been established, however, robust, clinical evidence-based guidelines are lacking in ACHD. The aim of this study was to evaluate the ICD guidelines in ACHD patients. Methods and Results: A total of 131 ACHD patients (male: n = 96 (73.3%), mean age: 42.8 ± 14.7 years, mean follow-up: 40.9 ± 28.3 months) undergoing ICDs implantation between 2010 and 2017 were reviewed. Sixty-nine patients (52.6%) received ICDs for a primary prevention indication. 122 (93.3%) patients had congenital heart disease of moderate to severe complexity. CRT-D (implantable cardiac resynchronization defibrillator) was implanted in 55 (42.0%) patients. During follow-up, 23 patients (17.6%) received appropriate ICD therapy. According to the current guideline (PACES/HRS 2014), 84 (64.1%), 8 (6.1%), and 39 (29.8%) could be classified as Class Ⅰ, Class Ⅱa, and Class Ⅱb indication, respectively. Compared to patients with Class Ⅱa and IIb indication, those with Class Ⅰ indication received more appropriate therapy (P = 0.030). Multivariate analysis showed that age (per 10-years decrease; P = 0.015, HR 1.254 CI; 1.045–1.505) and creatinine (per 100-μmol/L increase; P = 0.019, HR 1.555 CI; 1.076–2.247) were associated with appropriate therapy. Conclusion: Implantation of ICDs for preventing SCD based on current guidelines is reasonable. For patients with a borderline indication, younger age and renal dysfunction may aid in the selection of ICDs candidates.
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