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• A validated risk calculator (HCM Risk-Kids score) is useful to assess the risk for SCD in HCM patients younger than 16 years. • Myotonic dystrophy patients with palpitations suspected of arrhythmia, syncope or aborted sudden death need to be evaluated by invasive electrophysiological study. • In patients with repaired tetralogy of Fallot and monomorphic VT, catheter ablation is the preferred treatment.

9.3 Primary electrical disease

• Nadolol or propranolol are the preferred beta-blockers in LQTS and CPVT patients. • In asymptomatic LQTS patients the arrhythmic risk (1-2-3 Risk calculator) may be useful to calculate. • A type 1 Brugada ECG pattern provoked by sodium channel blocker test in the absence of other findings does not diagnose the BrS. • SCD risk stratification in asymptomatic BrS patients with a spontaneous type 1 pattern remains controversial. • Routine catheter ablation is not recommended in asymptomatic BrS patients. • The diagnosis of idiopathic VF requires exclusion of an underlying structural, channelopathic, or metabolic aetiology. • ERP can be a benign finding and is distinct from ERS. • Left cardiac denervation plays an important role in the management of CPVT and LQTS patients.

10 Gaps in evidence

10.1 General aspects

• Accurate screening tests to detect heart conditions associated with SCD in asymptomatic individuals in the general population are required. • In patients with SHD, the optimal time interval between repeated non-invasive and invasive prognostic tests, in case of a negative test, is unknown. • Improved assessment of genetic variants of uncertain significance and likely pathogenic variants is needed. • The utility of polygenic risk scores in patients at risk of SCD requires investigation.

10.2 Structural heart disease—general

• The long-term safety and efficacy of S-ICDs is unknown. • The role of primary preventive ICD therapy in patients with SHD and mildly reduced or preserved ejection fraction has not been studied systematically. • The optimal techniques to undertake VT substrate mapping and ablation in SHD remain to be determined. • The role of ICD implantation in end-stage heart failure patients supported by current-generation, continuous-flow LVADs is unclear. 10.3 Idiopathic premature ventricular

complexes/ventricular tachycardia

• The beneficial role of catheter ablation or anti-arrhythmic drug treatment in patients with asymptomatic, frequent PVCs and preserved cardiac function needs to be determined.

10.4 Coronary artery disease

• It is unknown which patients with chronic CAD and severely impaired LVEF are at low risk for SCD. • The role of LGE-CMR for risk stratification for SCD in chronic CAD is unclear. • RCTs are needed to determine the role of ICDs after successful VT ablation in chronic CAD with mildly reduced or preserved LVEF.

10.5 Cardiomyopathies

• It is unknown if PVC-induced cardiomyopathy is a diagnosis on its own or if an underlying predisposition is needed. • The predictive value of LGE-CMR findings (e.g. pattern and amount of LGE) for individual risk stratification for SCD in patients with cardiac sarcoidosis, HCM, and DCM/HNDC is unclear. • Studies are needed to determine the role of PES in patients with cardiac sarcoidosis and DCM/HNDC who have a mildly reduced or preserved cardiac function and LGE on CMR. • Prospective data on the association between intensity and duration of exercise and manifestation and severity of the phenotype in healthy ARVC mutation carriers are lacking. • The beneficial role of ICDs after successful ablation in ARVC patients who present with haemodynamically tolerated VT needs to be studied. • Data on clinical outcome, predictors for arrhythmic events, and indication for treatment, including ICD therapy, in patients with biventricular and left-dominant arrhythmogenic cardiomyopathy are required.

10.6 Valvular heart disease

• There is a lack of knowledge to identify patients with MVP at risk for VA and SCD.

10.7 Congenital heart disease

• There is a lack of knowledge regarding the absolute risk for VA and SCD in CHD that have undergone repair with contemporary surgical approaches.

10.8 Primary electrical disease

• Robust evidence is required to support the prophylactic use of the ICD on top of medical therapy with beta-blockers and genespecific therapy in LQTS patients. • More data are required to determine the role of LCSD and ICD in high-risk LQTS patients who do not tolerate medical therapy. Downloaded from academic.oup.com/eurheartj/article/43/40/3997/6675633 by guest on 07 October 2026