Comparison of different types of cardiac amyloidosis by cardiac magnetic resonance imaging. (June 2015)

• Record Type: Journal Article
• Authors: Kristen, Arnt V.; aus dem Siepen, Fabian; Scherer, Katrin; Kammerer, Rebekka; Andre, Florian; Buss, Sebastian J.; Bauer, Ralf; Lehrke, Stephanie; Voss, Andreas; Giannitsis, Evangelos; Katus, Hugo A.; Steen, Henning
• Is Part Of: Amyloid. Volume 22:Number 2(2015:Jun.)
• Page Start: 132
• Page End: 141
• Publication Date: 2015-06
• Subjects: Amyloidosis -- Periodicals; 616.3995
• DOI: 10.3109/13506129.2015.1020153
• Languages: English
• ISSNs: 1350-6129

Abstract

<abstract> <title>Abstract</title> <p> <italic>Objectives</italic>: We sought to determine cardiac morphological and functional differences between light-chain (AL), mutant-type transthyretin (ATTRmt) and wild-type TTR (ATTRwt) amyloidosis using contrast-enhancement cardiac magnetic resonance imaging (CE-CMR). Finally, we attempted to establish the diagnostic and prognostic impact of these findings. I<italic>ntroduction</italic>: The most common forms of cardiac amyloid are AL and ATTR amyloidosis, but the clinical courses of these variants are quite heterogeneous. While CE-CMR is used to evaluate patients with cardiac amyloidosis, its ability to predict prognosis in these patients is debatable.</p> <p> <italic>Methods</italic>: About 130 patients with cardiac amyloidosis (AL, <italic>n</italic> = 62; ATTRmt, <italic>n</italic> = 30, ATTRwt, <italic>n</italic> = 33) were assessed by CE-CMR (cardiac morphology, cardiac function, late gadolinium enhancement).</p> <p> <italic>Results</italic>: Left ventricular (LV) mass, basal and mid-ventricular maximal wall thickness, and thickness of the inter-atrial septum were higher in ATTRwt when compared to AL and ATTRmt amyloidosis. Tricuspid annular excursion was lower in ATTRwt amyloidosis than in AL amyloidosis. CE was observed in 94.6% of the patients (AL 80.6%; ATTRmt 90%; ATTRwt 87.9%) with significant differences in quality and intensity between the groups. Differentiation of amyloid types was achieved by combination of age, number of organs, the presence of inferolateral CE-CMR, thickness of inter-atrial septum and troponin T. Overall 1-year-survival rates were 93.3, 93.9 and 70.5% in ATTRwt, ATTRmt and AL amyloidosis, respectively. LV mass, mitral annular excursion and NT-proBNP in AL amyloidosis, LV mass maximal apical wall thickness and troponin T in ATTRwt amyloidosis, and finally NT-proBNP and renal function in ATTRmt amyloidosis were independent predictors of outcome.</p> <p> <italic>Conclusions</italic>: This study demonstrates that CE-CMR can highlight morphological and functional differences between different types of cardiac amyloidosis. In addition, CE-CMR and cardiac biomarkers provide useful prognostic information in patients with cardiac amyloidosis.</p> </abstract>