title: Prognosis assessment of cardiac involvement in systemic AL amyloidosis by magnetic resonance imaging.
abstract: BACKGROUND
  Cardiac involvement is one of the most important prognostic factors in systemic AL amyloidosis. The aim of our study was to assess the role of cardiovascular magnetic resonance (CMR) imaging in prognosis evaluation in AL amyloidosis.
  
  
  METHODS
  We retrospectively analyzed 29 consecutive patients with AL amyloidosis who had undergone CMR. Clinical, laboratory, echocardiographic, and CMR characteristics were compared between CMR-positive (ie, with CMR signs of cardiac localization of AL amyloidosis) and CMR-negative patients. Univariate and multivariate analyses were performed to assess the prognostic value of positive CMR in comparison with other prognostic factors.
  
  
  RESULTS
  CMR was positive in 11 patients (38%). The overall survival rates for CMR-positive patients were 28%, 14%, and 14% versus 84%, 77%, and 45% at 1, 2, and 5 years, respectively, for CMR-negative patients (P=.002). Late gadolinium enhancement patterns, biventricular hypertrophy, and pericardial effusion on CMR were more frequent in nonsurvivors. Congestive heart failure, abnormal echocardiography, Eastern Cooperative Oncology Group grade >1, brain natriuretic peptide, and left ventricular ejection fraction <55% also were associated with a decreased survival. The presence of congestive heart failure was the only significant variable associated with survival on multivariate analysis.
  
  
  CONCLUSIONS
  We found that the presence of a positive CMR in AL amyloidosis was associated with a significantly increased risk of death, in particular of cardiac origin, but was not independent of clinical congestive heart failure.
authors.affiliation: Hôpital Claude Huriez, Centre Hospitalier Régional Universitaire, Lille, France.
authors.forename: Arsene
authors.initials: A
authors.lastname: Mekinian
authors.forename: Christophe
authors.initials: C
authors.lastname: Lions
authors.forename: Xavier
authors.initials: X
authors.lastname: Leleu
authors.forename: Alain
authors.initials: A
authors.lastname: Duhamel
authors.forename: Nicolas
authors.initials: N
authors.lastname: Lamblin
authors.forename: Valerie
authors.initials: V
authors.lastname: Coiteux
authors.forename: Pascal
authors.initials: P
authors.lastname: De Groote
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Hatron
authors.forename: Thierry
authors.initials: T
authors.lastname: Facon
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Beregi
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: David
authors.initials: D
authors.lastname: Launay
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000175:diagnosis* / Q000401:mortality; D000704:Analysis of Variance; D015415:Biomarkers / Q000097:blood; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000401:mortality; D004452:Echocardiography; D005260:Female; D005602:France / Q000453:epidemiology; D005682:Gadolinium; D006333:Heart Failure / Q000175:diagnosis* / Q000401:mortality; D006801:Humans; D053208:Kaplan-Meier Estimate; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D013318:Stroke Volume
chemical_list: D015415:Biomarkers; D020097:Natriuretic Peptide, Brain; D005682:Gadolinium