Abstract

This study aimed to assess the utility of cardiac magnetic resonance imaging (MRI) T1 and T2 mapping as quantitative imaging biomarkers in transthyretin amyloid cardiomyopathy (ATTR-CM). This study retrospectively evaluated 74 patients with confirmed wild-type ATTR-CM who underwent cardiac MRI, 99mTc-labeled pyrophosphate (99mTc-PYP) scintigraphy, and echocardiography. We assessed the quantitative disease parameters, for example, left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS) by echocardiography, native T1, extracellular volume fraction (ECV), and native T2 value by cardiac MRI, heart-to-contralateral ratio (H/CL) by 99mTc-PYP, and high-sensitive cardiac troponin T. Myocardial native T2 of ≥50ms was defined as myocardial edema. Correlations between the disease's quantitative parameters were evaluated, and the ECV was compared to other parameters in ATTR-CM with/without myocardial edema. ECV in all patients with ATTR-CM revealed a strong correlation with native T1 (r=0.62), a moderate correlation with hs-TnT (r=0.59), LVEF (r=-0.48), GLS (r=0.58), and H/CL (r=0.48). Correlations between ECV and other quantitative parameters decreased in ATTR-CM with myocardial edema except for H/CL. Meanwhile, the correlations increased in ATTR-CM without myocardial edema. The presence of myocardial edema affected the interpretation of ECV assessment, although ECV can be a comprehensive imaging biomarker for ATTR-CM. ECV showed a significant correlation with various quantitative disease parameters and can be a reliable disease monitoring marker in patients with ATTR-CM when myocardial edema was excluded.

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