Table 2: Recommendations following Idiopathic Ventricular Fibrillation and Sudden Adult Death Syndrome.
| Recommendation | Class |
|---|---|
| Idiopathic Ventricular Fibrillation (IVF) | |
| Evaluation | |
| Genetic testing in IVF when there is suspicion of a specific genetic disease following clinical evaluation of the patient and/or family members | IIa |
| Genetic screening of a large panel of genes in patients in whom there is no suspicion of an inherited arrhythmogenic disease after clinical evaluation | III |
| Therapeutic Interventions | |
| ICD implantation in patients with the diagnosis of IVF | I |
| Antiarrhythmic therapy with quinidine, guided or empirical programmed electrical stimulation in patients with a diagnosis of IVF in conjunction with ICD implantation or when ICD implantation is contraindicated or refused | IIb |
| Ablation of Purkinje potentials in patients with a diagnosis of IVF presenting with uniform morphology premature ventricular contractions in conjunction with ICD implantation or when ICD implantation is contraindicated or refused. | IIb |
| If a first-degree relative of an IVF victim presents with unexplained syncope and no identifiable phenotype following thorough investigation, then after careful counselling an ICD implant may be considered | IIb |
| Evaluation of Family Members | |
| Evaluation of the first-degree relatives of all IVF victims with resting ECG, exercise stress testing and echocardiography Assessment of first-degree relatives with a history of palpitations, arrhythmias or syncope should be prioritised | I |
| Follow up clinical assessment in young family members of IVF victims who may manifest symptoms and /or signs of the disease at an older age and in all family members whenever additional SUDS or SUDI events occur | I |
| Evaluation of first-degree relatives of IVF victims with Holter and signal averaged ECGs, cardiac MRI and provocative testing with Class Ic antiarrhythmic drugs | IIa |
| Evaluation of first-degree relatives of IVF victims with epinephrine infusion | IIb |
| Sudden Adult Death Syndrome (SUDS) | |
| Evaluation | |
| For all SUDS victims: | |
| Collect personal/family history and circumstances of the sudden death | I |
| Expert cardiac pathology to rule out the presence of microscopic indicators of structural heart disease | I |
| Collect blood and/or suitable tissue for molecular autopsy/post-mortem genetic testing | I |
| Carry out an arrhythmia syndrome-focused molecular autopsy/post-mortem genetic testing | IIa |
| Therapeutic interventions | |
| Genetic screening of first-degree relatives of a SUDS victim whenever a pathogenic mutation in a gene associated with an increased risk of sudden death is identified by molecular autopsy in the SUDS victim | I |
| Evaluation of first-degree blood relatives of all SUDS victims with resting ECG with high right ventricular leads, exercise stress testing and echocardiography. Assessment of obligate carriers and relatives with a history of palpitations, arrhythmias, or syncope should be prioritised | I |
| Follow-up clinical assessment in young family members of SUDS victims who may manifest symptoms and/or signs of the disease at an older age and in all family members whenever additional SUDS or SUDI events occur | I |
| Evaluation of first-degree relatives of SUDS victims with ambulatory and signal-averaged ECGs, cardiac MRI, and provocative testing with Class Ic antiarrhythmic drugs | IIa |
| Evaluation of first-degree relatives of SUDS victims with epinephrine infusion | IIb |
ICD = implantable cardioverter-defibrillator; IVF = idiopathic ventricular fibrillation; SUDI = sudden unexplained death of an infant; SUDS = sudden unexplained death syndrome. Adapted from Priori et al., 2013[1]