Jérémy Florence, Univ. Paris Cité: Refining risk stratification using CMR in patients with hypertrophic cardiomyopathy

Abstract: Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, characterized by ventricular hypertrophy, myocyte disarray, and interstitial fibrosis, often resulting from sarcomere gene variants. Patients experience adverse events such as heart failure (HF), stroke, arrythmias and sudden cardiac death (SCD) leading to an increased risk of mortality. Late gadolinium enhancement (LGE) detected by cardiovascular magnetic resonance (CMR) imaging has emerged as a valuable method for the quantification of myocardial scarring. The presence and extent of LGE are both associated with a heightened risk of all-cause mortality in HCM patients. However, despite broad acceptance and recent inclusion in the ACC/AHA and ESC guidelines, optimal cutoff values for LGE quantification remain debated. Furthermore, several studies on non-ischemic cardiomyopathies have demonstrated that specific features of LGE, such as its location and pattern, may play a crucial role in risk stratification, which is pivotal for providing tailored care and optimized decision-making regarding medications and preventive implantable cardioverter-defibrillator (ICD). However, there is a lack of data on the prognostic impact of these specific LGE features in HCM and very few studies have explored simultaneously the role of LGE extent, location and pattern in predicting outcomes for this population. Given the importance of improving risk stratification in HCM patients to guide personalized treatment decisions, particularly regarding medications and ICD, our aim is to assess the incremental prognostic value of the “LGE granularity” concept —encompassing LGE extent, location, and pattern — in predicting all-cause mortality in a patients with HCM.