CONGRESS ABSTRACT

Abstract Background: Non-dilated left ventricular cardiomyopathy (NDLVC) is a recently defined entity, often characterized by arrhythmic risk disproportionate to ventricular function. According to the 2023 ESC guidelines, cardiac magnetic resonance (CMR) and genetics are essential for risk stratification. Recent multicenter studies have highlighted the prognostic role of ring-like late gadolinium enhancement (LGE) and pathogenic variants in high-risk genes, such as LMNA. Case Presentation: A 46-year-old woman, heavy smoker, with a brother affected by LMNA-related dilated cardiomyopathy and implanted with an implantable cardioverter-defibrillator (ICD), was admitted for chest pain and palpitations. Continuous ECG monitoring showed frequent non-sustained ventricular tachycardia. Echocardiography documented preserved left ventricular ejection fraction (60%), while coronary CT angiography excluded obstructive disease. CMR revealed a ring-like pattern of LGE, consistent with diffuse fibrotic involvement and an arrhythmogenic substrate. Given the combination of arrhythmic burden, family history, and CMR features, an extravascular ICD was implanted for primary prevention. Discussion: This case illustrates how the integration of advanced imaging and genetic background can unmask high-risk NDLVC phenotypes even in the absence of overt systolic dysfunction. The ring-like LGE pattern, previously associated with major arrhythmic events and adverse remodeling, coupled with a family history of LMNA mutation-related cardiomyopathy, provided converging evidence for early device therapy. Conclusion: In NDLVC, integrating CMR tissue characterization with family history and genetic context can refine arrhythmic risk assessment and support timely preventive strategies for sudden cardiac death, particularly when echocardiography appears normal.