title: Comparison of different types of cardiac amyloidosis by cardiac magnetic resonance imaging.
abstract: OBJECTIVE
  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.
  
  
  BACKGROUND
  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.
  
  
  METHODS
  About 130 patients with cardiac amyloidosis (AL, n = 62; ATTRmt, n = 30, ATTRwt, n = 33) were assessed by CE-CMR (cardiac morphology, cardiac function, late gadolinium enhancement).
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Cardiology and.
authors.forename: Arnt V
authors.initials: AV
authors.lastname: Kristen
authors.forename: Fabian
authors.initials: F
authors.lastname: aus dem Siepen
authors.forename: Katrin
authors.initials: K
authors.lastname: Scherer
authors.forename: Rebekka
authors.initials: R
authors.lastname: Kammerer
authors.forename: Florian
authors.initials: F
authors.lastname: Andre
authors.forename: Sebastian J
authors.initials: SJ
authors.lastname: Buss
authors.forename: Ralf
authors.initials: R
authors.lastname: Bauer
authors.forename: Stephanie
authors.initials: S
authors.lastname: Lehrke
authors.forename: Andreas
authors.initials: A
authors.lastname: Voss
authors.forename: Evangelos
authors.initials: E
authors.lastname: Giannitsis
authors.forename: Hugo A
authors.initials: HA
authors.lastname: Katus
authors.forename: Henning
authors.initials: H
authors.lastname: Steen
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000401:mortality / Q000473:pathology*; D006332:Cardiomegaly / Q000401:mortality / Q000473:pathology; D009202:Cardiomyopathies / Q000401:mortality / Q000473:pathology; D005260:Female; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged
keywords: Amyloidosis; cardiac magnetic resonance imaging; contrast enhancement; hypertrophy; risk stratification