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Diffuse Fibrosis in Hypertrophic Cardiomyopathy: Incremental Prognostic Value of Extracellular Volume.
BACKGROUND 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). OBJECTIVES The authors sought to evaluate the prognostic value of ECV beyond established clinical and imaging variables in a large contemporary HCM cohort. METHODS 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. RESULTS 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). CONCLUSIONS Myocardial ECV was independently associated with long-term adverse outcomes. Multiparametric CMR assessment may improve risk stratification in HCM.
Reappraisal of Surgical Threshold in Patients With Ascending Aortic Dilation
Background Indexing ascending aortic dimensions to height, rather than absolute diameters, may better identify at-risk ascending aortopathy (AA) patients who could benefit from earlier surgery. Objectives This study evaluated whether height-indexed aortic dimensions improve long-term risk stratification in AA patients who do not meet current surgical guidelines. Methods A total of 11,083 patients (68 ± 13 years, 79% men, 28% with bicuspid aortic valve, 2,196 with aortic surgery and 8,887 unoperated) with AA (4-5 cm) diagnosed on echocardiography (2010-2023) were included. Patients with syndromic aortopathy and acute aortic dissection/rupture at presentation were excluded. The maximum AA diameter, AA height index (AHI) (cm/m), and AA cross-sectional area-to-height (CSAH) (cm2/m) index were calculated. The primary outcome was mortality. Results The mean AA diameter, AHI, and CSAH were 4.4 ± 0.2 cm, 2.53 ± 0.3 cm/m, and 8.8 ± 1 cm2/m, respectively. At 6 ± 4 years, there were 84 (8.3%) deaths in the operated group and 1,058 (11.9%) in the unoperated group. There were 128 aortic dissections (1.4%) and 4 aortic ruptures (0.04%) in the unoperated group. Dividing the unoperated sample into a derivation cohort (n = 3,887), spline analysis demonstrated an increase in hazard for death in women with AA diameter ≥4.5 cm and in all patients with AHI ≥2.5 cm/m or CSAH ≥9 cm2/m. Validation in a separate cohort (n = 5,000) confirmed that AHI ≥2.5 cm/m and CSAH ≥9 cm2/m were associated with higher long-term mortality (both P < 0.05). Conclusions Indexing AA dimensions to height better identifies high-risk AA patients, particularly women, who fall below surgical criteria. Further studies are needed to determine if this cohort may benefit from earlier intervention at lower size thresholds.
Optimal Reclassification of Aortic Aneurysms: Cross-Sectional CT vs 2D Transthoracic Echocardiography.
Indexed parameters, particularly CSAH, improve risk stratification beyond absolute diameters and highlight size-based differences in AA and provide an individualized precision imaging-based approach to surgical decision-making.