Cardiovascular magnetic resonance-derived extracellular volume fraction was a significant independent predictor of HCM-related events (adjusted HR 1.08 per 1% increase; 95% CI 1.02-1.15).
Cohort (n=990)
Does CMR-derived extracellular volume fraction (ECV) improve risk stratification for HCM-related events beyond late gadolinium enhancement (LGE) in patients with hypertrophic cardiomyopathy?
CMR-derived extracellular volume fraction (ECV) provides incremental prognostic value beyond late gadolinium enhancement (LGE) for predicting adverse outcomes in patients with hypertrophic cardiomyopathy.
Hazard Ratio: 1.08 (95% CI 1.02–1.15)
AIMS: This study investigated the incremental prognostic value of cardiovascular magnetic resonance (CMR)-derived extracellular volume fraction (ECV), a marker of diffuse interstitial fibrosis, beyond late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: We analysed 990 consecutive HCM patients (median age 58 years, male 68.3%) who underwent CMR between 2012 and 2024. LGE and global ECV were quantified, and their associations with the primary endpoint of HCM-related events-a composite of sudden cardiac death (SCD) events, heart failure (HF) events, and HCM-related death-were assessed. During a median follow-up of 3.2 years, 64 (6.5%) patients experienced the primary endpoint. While LGE (median 7.1%, IQR 2.3-16.9%) and ECV (median 29.0%, IQR 26.6-32.0%) were moderately correlated (R = 0.604, P < 0.001), both were significantly associated with increased risk of the primary endpoint and individual outcomes of SCD and HF events, and optimal cutoffs were determined as LGE ≥ 27% and ECV ≥ 35%. Patients with ECV ≥ 35% had more symptoms, a more severe phenotype with greater systolic and diastolic dysfunction, and more pathogenic gene variants. Notably, ECV remained a significant predictor of the primary endpoint (adjusted HR 1.08, 95% CI 1.02-1.15, per 1%) after adjustment for key disease variables, including left ventricular ejection fraction and LGE. Elevated ECV effectively identified high-risk individuals even among lower-risk subgroups, including those with low LGE burden. CONCLUSION: Increased ECV is an independent predictor of HCM-related outcomes. ECV may serve as a novel imaging biomarker to refine risk stratification in HCM patients who do not meet traditional LGE-based high-risk criteria.
Lee et al. (Mon,) conducted a cohort in hypertrophic cardiomyopathy (n=990). Extracellular volume fraction (ECV) was evaluated on HCM-related events (composite of sudden cardiac death, heart failure events, and HCM-related death) (HR 1.08, 95% CI 1.02-1.15). Cardiovascular magnetic resonance-derived extracellular volume fraction was a significant independent predictor of HCM-related events (adjusted HR 1.08 per 1% increase; 95% CI 1.02-1.15).