Prognosis assessment of cardiac involvement in systemic AL amyloidosis by magnetic resonance imaging.
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.
Mekinian
Arsene
A
Hôpital Claude Huriez, Centre Hospitalier Régional Universitaire, Lille, France.
Lions
Christophe
C
Leleu
Xavier
X
Duhamel
Alain
A
Lamblin
Nicolas
N
Coiteux
Valerie
V
De Groote
Pascal
P
Hatron
Pierre-Yves
PY
Facon
Thierry
T
Beregi
Jean-Paul
JP
Hachulla
Eric
E
Launay
David
D
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
D015415:Biomarkers; D020097:Natriuretic Peptide, Brain; D005682:Gadolinium