Abstract

BackgroundMyocardial disarray is an important histological feature of hypertrophic cardiomyopathy (HCM) which has been studied post-mortem, but its in-vivo prevalence and extent is unknown. Cardiac Diffusion Tensor Imaging (cDTI) provides information on mean intravoxel myocyte orientation and potentially myocardial disarray. Recent technical advances have improved in-vivo cDTI, and the aim of this study was to assess the interstudy reproducibility of quantitative in-vivo cDTI in patients with HCM.Methods and resultsA stimulated-echo single-shot-EPI sequence with zonal excitation and parallel imaging was implemented. Ten patients with HCM were each scanned on 2 different days. For each scan 3 short axis mid-ventricular slices were acquired with cDTI at end systole. Fractional anisotropy (FA), mean diffusivity (MD), and helix angle (HA) maps were created using a cDTI post-processing platform developed in-house. The mean ± SD global FA was 0.613 ± 0.044, MD was 0.750 ± 0.154 × 10-3 mm2/s and HA was epicardium −34.3 ± 7.6°, mesocardium 3.5 ± 6.9° and endocardium 38.9 ± 8.1°. Comparison of initial and repeat studies showed global interstudy reproducibility for FA (SD = ± 0.045, Coefficient of Variation (CoV) = 7.2%), MD (SD = ± 0.135 × 10-3 mm2/s, CoV = 18.6%) and HA (epicardium SD = ± 4.8°; mesocardium SD = ± 3.4°; endocardium SD = ± 2.9°). Reproducibility of FA was superior to MD (p = 0.003). Global MD was significantly higher in the septum than the reference lateral wall (0.784 ± 0.188 vs 0.750 ± 0.154 x10-3 mm2/s, p < 0.001). Septal HA was significantly lower than the reference lateral wall in all 3 transmural layers (from −8.3° to −10.4°, all p < 0.001).ConclusionsTo the best of our knowledge, this is the first study to assess the interstudy reproducibility of DTI in the human HCM heart in-vivo and the largest cDTI study in HCM to date. Our results show good reproducibility of FA, MD and HA which indicates that current technology yields robust in-vivo measurements that have potential clinical value. The interpretation of regional differences in the septum requires further investigation.

Highlights

  • Myocardial disarray is an important histological feature of hypertrophic cardiomyopathy (HCM) which has been studied post-mortem, but its in-vivo prevalence and extent is unknown

  • To the best of our knowledge, this is the first study to assess the interstudy reproducibility of Diffusion Tensor Imaging (DTI) in the human HCM heart in-vivo and the largest Cardiac Diffusion Tensor Imaging (cDTI) study in HCM to date

  • Our results show good reproducibility of Fractional Anisotropy (FA), Mean Diffusivity (MD) and Helix Angle (HA) which indicates that current technology yields robust in-vivo measurements that have potential clinical value

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Summary

Introduction

Myocardial disarray is an important histological feature of hypertrophic cardiomyopathy (HCM) which has been studied post-mortem, but its in-vivo prevalence and extent is unknown. Disarray may play a key role the in the genesis of the substrate responsible for malignant ventricular arrhythmias and myocardial contractile dysfunction, [4] but to date, insights into the extent and spatial distribution of myocyte disarray have been derived largely from small post-mortem series examining hearts obtained after sudden cardiac death [5,6,7,8] These studies have considerable selection bias and as a result, the in-vivo prevalence and functional consequences of disarray in HCM remain unknown. The diagnostic utility of disarray in HCM is unclear as the phenomenon has been described in a variety of congenital and acquired myocardial diseases [9,10] These include other conditions associated with left ventricular hypertrophy, such as aortic stenosis and hypertensive heart disease, [11] the latter frequently posing a challenge in the differential diagnosis of HCM [12]

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