title: Noninvasive detection of cardiac involvement in patients with hereditary transthyretin associated amyloidosis using cardiac magnetic resonance imaging: a prospective study.
abstract: BACKGROUND
  Most of the studies that described cardiac amyloidosis using cardiac magnetic resonance (CMR) imaging refer to patients with primary light chain (AL) amyloidosis. The goal of this study was to evaluate cardiac involvement in patients with hereditary transthyretin associated (ATTR) amyloidosis and asymptomatic carriers and its relationships with clinical symptoms and genotype, using CMR imaging.
  
  
  RESULTS
  Fifty-three patients with hereditary ATTR amyloidosis and 14 asymptomatic carriers were included in this study. Morphological, functional and late gadolinium enhancement (LGE) findings were noted on CMR images. A positive LGE suggesting cardiac amyloidosis was detected in 60% of patients. The pattern of LGE was diffuse, focal and circumferential in 32, 26 and 2% of patients, respectively. The inferior basal segment was the most frequently involved (93%) in case of focal involvement. Diffuse pattern was exclusively encountered in patients with cardiac symptoms. Nineteen percent of patients with isolated neurological symptoms and 20% of subjects without left ventricular wall thickening exhibited cardiac abnormalities on CMR.
  
  
  CONCLUSIONS
  Cardiac involvement can be detected in patients with hereditary ATTR amyloidosis with isolated neurological symptoms and without left ventricular wall thickening, suggesting that CMR could be useful in detecting preclinical cardiac amyloidosis.
authors.affiliation: Radiology Department, Henri Mondor Hospital, University Paris Est Créteil, Assistance Publique-Hôpitaux de Paris , Créteil , France .
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Deux
authors.forename: Thibaud
authors.initials: T
authors.lastname: Damy
authors.forename: Alain
authors.initials: A
authors.lastname: Rahmouni
authors.forename: Julie
authors.initials: J
authors.lastname: Mayer
authors.forename: Violaine
authors.initials: V
authors.lastname: Planté-Bordeneuve
mesh_terms: D000328:Adult; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D006321:Heart / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies
keywords: Amyloid; cardiac; magnetic resonance imaging; transthyretin