Tab. 1.

Recommendations for management of ventricular arrhythmias in patients with congenital heart disease. Adapted from the 2015 European Society of Cardiology guidelines for the management of patients with ventricular arrhythmias (Priori et al.) [42]

Class	Clinical indication	Level of evidence
Class I	After evaluation to define the cause of the event and exclude any reversible causes, ICD implantation is recommended for patients with CHD who are survivors of an aborted cardiac arrest	B
	ICD implantation is recommended for patients with CHD with symptomatic sustained VT who have undergone haemodynamic and electrophysiological evaluation	B
	Catheter ablation is recommended as additional therapy or an alternative to ICD in patients with CHD who have recurrent monomorphic VT or appropriate ICD therapies that are not manageable by device reprogramming or drug therapy	C
	ICD therapy is recommended in adults with CHD and a systemic LVEF < 35 %, biventricular physiology, symptomatic HF despite optimal medical treatment and NYHA functional class II or III	C
Class IIa	ICD implantation should be considered in patients with CHD with syncope of unknown origin in the presence of either advanced ventricular dysfunction or inducible sustained VT or VF on PVS	B
	ICD implantation should be considered in selected patients with tetralogy of Fallot and multiple risk factors for SCD, including LV dysfunction, non-sustained VT, QRS duration > 180 ms or inducible sustained VT on PVS	B
	Catheter ablation should be considered as an alternative to drug therapy for symptomatic sustained monomorphic VT in patients with CHD and an ICD	B
Class IIb	ICD therapy may be considered in patients with advanced single or systemic RV dysfunction in the presence of other risk factors such as non-sustained VT, NYHA functional class II or III or severe systemic AV valve regurgitation	B
	PVS may be considered for risk stratification of SCD in patients with tetralogy of Fallot who have one or more risk factors among LV dysfunction, non-sustained VT and QRS duration > 180 ms	B
	PVS may be considered in patients with CHD and non-sustained VT to determine the risk of sustained VT	C
	Surgical ablation guided by electrophysiological mapping may be considered in patients with CHD undergoing cardiac surgery, with clinical sustained VT and with inducible sustained monomorphic VT with an identified critical isthmus.	C
Class III	PVS is not recommended to stratify the risk in patients with CHD in the absence of other risk factors or symptoms	C

AV atrioventricular, CHD congenital heart disease, HF heart failure, ICD implantable cardioverter-defibrillator, LV left ventricle, LVEF left ventricular ejection fraction, NYHA New York Heart Association, PVS programmed ventricular stimulation, RV right ventricle, VF ventricular fibrillation, VT ventricular tachycardia