Abstract
Methods 30 patients (21 male, 9 female; median age 69.5 years) with diagnosis of TTR amyloidosis (wild-type n=13, hereditary n=17) were evaluated by cardiac MRI (CMR; Achieva Intera Philips Medical Systems, Best, The Netherlands), and Technetium-DPD scintigraphy. EDV, ESV, EF and myocardial mass were analyzed on a standard workstation (Philips Viewforum). Longitudinal function was assessed by mitral (MAPSE) and tricuspid (TAPSE) annular plane systolic excursion. Atrial septum thickness was measured on SSFP-4 chamber views. Gadolinium contrast-enhanced CMR (CE-CMR) was assessed semi-quantitatively (absent=0, weak=1, moderate=2, severe=3) in an AHA modified 16 segment model of the left ventricle as well as for the right/left atrium and right ventricle. Nuclear DPDretention was assessed semi-quantitatively using a region of interest technique by comparison of counts in the heart at 3 hours after injection with whole body counts at 5 min after injection.
Highlights
Transthyretin (TTR) is the major constituent of severe systemic amyloid diseases with a broad spectrum of genotypes and heterogeneous phenotypes
Longitudinal function was assessed by mitral (MAPSE) and tricuspid (TAPSE) annular plane systolic excursion
Atrial septum thickness was measured on SSFP-4 chamber views
Summary
Transthyretin (TTR) is the major constituent of severe systemic amyloid diseases with a broad spectrum of genotypes and heterogeneous phenotypes. We evaluated the impact of different imaging tools for risk assessment in TTR patients
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