Familial Evaluation in Idiopathic Ventricular Fibrillation

Background: Familial cascade screening is well established in patients with heritable cardiac disease and cases of sudden arrhythmic death syndrome. The clinical benefit family idiopathic ventricular fibrillation (IVF) unknown. Methods: Patients IVF were identified from national institutional registries. All underwent systematic comprehensive evaluation to exclude identifiable causes arrest a minimum requirement ECG, (echocardiogram or magnetic resonance imaging) coronary imaging, exercise sodium channel blocker provocation. Additional investigations including genetic testing performed at the physician’s discretion. First-degree relatives who assessed least 12-lead ECG included final cohort. Results additional investigations, discretion, also recorded. coded as normal, abnormal, minor findings. Results: We 201 first-degree 96 patients. In addition echocardiography was 159 (79%) ≥1 investigation 162 (80%) relatives. An inherited arrhythmia syndrome diagnosed 5 (3%) individuals 4 (4%) families. Two hosted DPP6 risk haplotype single proband, one whom received primary prevention implantable cardioverter defibrillator. 3 separate families, an asymptomatic parent proband developed type 1 Brugada pattern during managed lifestyle measures only. early repolarization (ER) present 16% probands more common those families than where did not have (25% versus 8%, P =0.04). Conclusions: yield low when comprehensively investigated. significance J wave syndromes role for provocation are, however, uncertain require further research.