Fortunately, the population of adults with congenital heart disease (ACHD) is growing due to improved operation techniques. Life expectancy is continuously rising, nevertheless, sudden cardiac death is one of the leading causes of mortality in ACHD late after initial diagnosis. Risk stratification in ACHD remains challenging as large study results are missing, congenital defects and operation methods differ considerably between individual patients and results from acquired heart diseases are often not conferrable. The purpose of this narrative review is to objectively summarize the current knowledge on arrhythmogenic risk of ACHD and to give an overview on implantable cardioverter-defibrillator (ICD) therapy in this collective. Remarkable progress has been made in electrophysiological understanding of critical areas of slow conduction especially in patients with Tetralogy of Fallot (ToF). In patients with transposition of the great arteries after atrial baffling (Mustard/Senning procedure) atrial arrhythmias play a crucial role in sudden cardiac death. ICD therapy in ACHD may pose special technical challenges due to limited access for intracardiac leads. The introduction of the totally subcutaneous ICD improved therapeutic options for ACHD especially when contraindications for transvenous leads are present. Risk stratification in ACHD has to be seen as a team approach, requires thorough understanding of congenital heart defects and the operation techniques and needs unconventional technical approaches in some cases.
Keywords: Adults with congenital heart diseases (ACHD); ventricular arrhythmia, risk stratification, implantable-cardioverter-defibrillator.
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