Diffuse Fibrosis in Hypertrophic Cardiomyopathy: Incremental Prognostic Value of Extracellular Volume.

Authors

Susan K Keen, Hesham Sheashaa, Stuti Shah, Mohammed Abusafia, Shada Jadam, Simrat Kaur, Andrew Gaballa, Susan Ospina, Katy Rutkowski, Jay Ramchand, Zoran B Popovic, Maran Thamilarasan, Nicholas G Smedira, Paul Schoenhagen, Michael Bolen, Bo Xu, Milind Y Desai

Published in

JACC. Advances. Pages 103224. Aug 28, 2026. Epub Aug 28, 2026.

Abstract

Left ventricular (LV) myocardial extracellular volume (ECV), from cardiac magnetic resonance (CMR) T1 mapping, primarily reflects diffuse fibrosis and is a marker of adverse myocardial remodeling in hypertrophic cardiomyopathy (HCM).
The authors sought to evaluate the prognostic value of ECV beyond established clinical and imaging variables in a large contemporary HCM cohort.
We evaluated 1,050 consecutive patients with HCM and preserved LV ejection fraction (≥50%) who underwent CMR, including quantification of ECV (%) and late gadolinium enhancement (LGE) burden (% LV mass, >6 SDs above remote myocardium), between 2012 and 2021. The primary endpoint was a composite of mortality, appropriate implantable cardioverter-defibrillator (ICD) therapy, or heart transplantation. Statistical methods included multivariable Cox proportional hazards regression, restricted cubic spline analysis, assessment of incremental discrimination using changes in the C-statistic, and net reclassification improvement analysis.
Median age was 60 years (IQR: 49-68 years) with 44% women. Median follow-up was 5.6 years (IQR: 2.5-7.8 years). The primary endpoint occurred in 124 patients (11.8%), including 111 deaths (10.6%), 12 appropriate ICD discharges (1.1%), and 1 heart transplantation (0.1%). In multivariable Cox regression, older age (HR: 1.05, 95% CI: 1.03-1.06; P < 0.001), NYHA functional class ≥II (HR: 1.96, 95% CI: 1.23-3.11; P = 0.004), diabetes mellitus (HR: 1.89, 95% CI: 1.27-2.81; P = 0.002), and higher ECV (HR: 1.05 per 1% increase, 95% CI: 1.02-1.09; P = 0.001) were independently associated with the primary outcome. Patients with ECV ≥32% had lower event-free survival than those with ECV <32% (adjusted HR: 1.81, 95% CI: 1.24-2.64; P < 0.001).
Myocardial ECV was independently associated with long-term adverse outcomes. Multiparametric CMR assessment may improve risk stratification in HCM.

PMID:
42663371
Bibliographic data and abstract were imported from PubMed on 28 Aug 2026.

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