title: Cardiovascular magnetic resonance in cardiac amyloidosis.
abstract: BACKGROUND
  Cardiac amyloidosis can be diagnostically challenging. Cardiovascular magnetic resonance (CMR) can assess abnormal myocardial interstitium.
  
  
  RESULTS
  Late gadolinium enhancement CMR was performed in 30 patients with cardiac amyloidosis. In 22 of these, myocardial gadolinium kinetics with T1 mapping was compared with that in 16 hypertensive controls. One patient had CMR and autopsy only. Subendocardial T1 in amyloid patients was shorter than in controls (at 4 minutes: 427+/-73 versus 579+/-75 ms; P<0.01), was shorter than subepicardium T1 for the first 8 minutes (P< or =0.01), and was correlated with markers of increased myocardial amyloid load, as follows: left ventricular (LV) mass (r=-0.51, P=0.013); wall thickness (r=-0.54 to -0.63, P<0.04); interatrial septal thickness (r=-0.52, P=0.001); and diastolic function (r=-0.42, P=0.025). Global subendocardial late gadolinium enhancement was found in 20 amyloid patients (69%); these patients had greater LV mass (126+/-30 versus 93+/-25 g/m2; P=0.009) than unenhanced patients. Histological quantification showed substantial interstitial expansion with amyloid (30.5%) but only minor fibrosis (1.3%). Amyloid was dominantly subendocardial (42%) compared with midwall (29%) and subepicardium (18%). There was 97% concordance in diagnosis of cardiac amyloid by combining the presence of late gadolinium enhancement and an optimized T1 threshold (191 ms at 4 minutes) between myocardium and blood.
  
  
  CONCLUSIONS
  In cardiac amyloidosis, CMR shows a characteristic pattern of global subendocardial late enhancement coupled with abnormal myocardial and blood-pool gadolinium kinetics. The findings agree with the transmural histological distribution of amyloid protein and the cardiac amyloid load and may prove to have value in diagnosis and treatment follow-up.
authors.affiliation: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors.forename: Alicia Maria
authors.initials: AM
authors.lastname: Maceira
authors.forename: Jayshree
authors.initials: J
authors.lastname: Joshi
authors.forename: Sanjay Kumar
authors.initials: SK
authors.lastname: Prasad
authors.forename: James Charles
authors.initials: JC
authors.lastname: Moon
authors.forename: Enrica
authors.initials: E
authors.lastname: Perugini
authors.forename: Idris
authors.initials: I
authors.lastname: Harding
authors.forename: Mary Noelle
authors.initials: MN
authors.lastname: Sheppard
authors.forename: Philip Alexander
authors.initials: PA
authors.lastname: Poole-Wilson
authors.forename: Philip Nigel
authors.initials: PN
authors.lastname: Hawkins
authors.forename: Dudley John
authors.initials: DJ
authors.lastname: Pennell
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology*; D001706:Biopsy; D009202:Cardiomyopathies / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology*; D003287:Contrast Media / Q000493:pharmacokinetics; D005260:Female; D019786:Gadolinium DTPA / Q000097:blood / Q000493:pharmacokinetics; D006801:Humans; D006973:Hypertension / Q000473:pathology; D017379:Hypertrophy, Left Ventricular / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D007457:Iodine Radioisotopes / Q000493:pharmacokinetics; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009929:Organ Size; D011446:Prospective Studies; D011877:Radionuclide Imaging; D000683:Serum Amyloid P-Component / Q000493:pharmacokinetics; D013318:Stroke Volume; D014463:Ultrasonography; D018487:Ventricular Dysfunction, Left / Q000209:etiology / Q000503:physiopathology
chemical_list: D003287:Contrast Media; D007457:Iodine Radioisotopes; D000683:Serum Amyloid P-Component; D019786:Gadolinium DTPA