Genetics of idiopathic ventricular fibrillation

Abstract Funding Acknowledgements Type of funding sources: None. Background Idiopathic ventricular fibrillation (IVF) accounts for up to 44% sudden cardiac deaths (SCD); the diagnostic yield genetic testing in probands is reported as low 3%. Purpose The aim this study describe results IVF from a high-volume center. Methods All consecutive patients with and were retrospectively analyzed. was diagnosed, following HRS/EHRA guidelines, by means excluding other causes through clinical, imaging data. Genetic analysis performed all SeqCap v3 Brugada syndrome next generation sequencing HiSeq1500 inherited primary arrhythmia syndromes. variants classified ACMG pathogenic (P)/likely (LP) or unknown significance (VUS). Results Fourteen included (Table); an ICD implanted patients. A variant found 9 (64.3%), 5 P/LP (35.7%) 4 (28.6%) VUS variant. Genes mutated included: RYR2 3 TTN 2 MYPN one patient, SCN4A DES patient CACNA1C patient. At mean follow-up 76 months experienced shock [2 (40%) vs (33%) VUS/no variants, p=NS). Conclusion can be useful IVF; prognosis modest high rate at long-term follow up.