Abstract

Cardiac amyloidosis (CA) is an infiltrative disease. In the present study, we compared the diagnostic accuracy of cardiovascular magnetic resonance (CMR)-based T1-mapping and subsequent extracellular volume fraction (ECV) measurement and longitudinal strain analysis in the same patients with (a) biopsy-proven cardiac amyloidosis (CA) and (b) hypertrophic cardiomyopathy (HCM). N = 30 patients with CA, N = 20 patients with HCM and N = 15 healthy control patients without relevant cardiac disease underwent dedicated CMR studies. The CMR protocol included standard sequences for cine-imaging, native and post-contrast T1-mapping and late-gadolinium-enhancement. ECV measurements were based on pre- and post-contrast T1-mapping images. Feature-tracking analysis was used to calculate 3D left ventricular longitudinal strain (LV-LS) in basal, mid and apical short-axis cine-images and to assess the presence of relative apical sparing. Receiver-operating-characteristic analysis revealed an area-under-the-curve regarding the differentiation of CA from HCM of 0.984 for native T1-mapping (p < 0.001), of 0.985 for ECV (p < 0.001) and only 0.740 for the “apical-to-(basal + midventricular)”-ratio of LV-LS (p = 0.012). A multivariable logistical regression analysis showed that ECV was the only statistically significant predictor of CA when compared to the parameter LV-LS or to the parameter “apical-to-(basal + midventricular)” LV-RLS-ratio. Native T1-mapping and ECV measurement are both superior to longitudinal strain measurement (with assessment of relative apical sparing) regarding the appropriate diagnosis of CA.

Highlights

  • Cardiac amyloidosis (CA) is an infiltrative disease

  • Amyloidoses are classified based on the misfolded protein precursor—with light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis being the most commonly found forms in case of cardiac amyloidosis (CA)[2]

  • Males and females showed a similar distribution in the CA and hypertrophic cardiomyopathy (HCM) group (77% in the CA group vs. 80% in the HCM group; p = 0.13)

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Summary

Introduction

We compared the diagnostic accuracy of cardiovascular magnetic resonance (CMR)-based T1-mapping and subsequent extracellular volume fraction (ECV) measurement and longitudinal strain analysis in the same patients with (a) biopsy-proven cardiac amyloidosis (CA) and (b) hypertrophic cardiomyopathy (HCM). Feature-tracking analysis was used to calculate 3D left ventricular longitudinal strain (LV-LS) in basal, mid and apical short-axis cine-images and to assess the presence of relative apical sparing. Abbreviations AL Immunoglobulin light chain amyloidosis ATTR Transthyretin amyloidosis CA Cardiac amyloidosis CAD Coronary artery disease CMR Cardiovascular magnetic resonance DCM Dilated cardiomyopathy ECV Extracellular volume EMB Endomyocardial biopsy FT Feature tracking GLS Global longitudinal strain HCM Hypertrophic cardiomyopathy HF Heart failure LGE Late-gadolinium-enhancement LV Left ventricle LV-EDV Left ventricular end-diastolic volume ukmuenster.de. Deposition of amyloid fibrils in the atrial and ventricular wall may cause conduction abnormalities and contributes to the high prevalence of ventricular arrhythmias and atrial fibrillation, increasing the risk of sudden cardiac death (SCD) and t­hromboembolism[7]

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