title: Pericardial Effusion and Multiple Organ Involvement Are Independent Predictors of Mortality in Patients with Systemic Light Chain Amyloidosis.
abstract: OBJECTIVE
  Left ventricular (LV) functions assessed by echocardiography and cardiac biomarkers are strong predictors of mortality in patients with systemic light chain (AL) amyloidosis. However, most previous studies have been conducted in Western countries, and the predictors of mortality in Asian patients with AL amyloidosis have not been characterized. To address this issue, we aimed to determine the predictors of mortality in Asian patients with biopsy-confirmed AL amyloidosis.
  
  
  METHODS
  We retrospectively enrolled 31 patients (59±11 years, 55% men) in whom AL amyloidosis was confirmed by biopsies from cardiac or non-cardiac tissues. Of these patients, 15 (48%) met the international echocardiographic criteria for cardiac amyloidosis (mean LV wall thickness >12 mm without other causes of LV hypertrophy).
  
  
  RESULTS
  During a mean follow-up period of 21±20 months, 15 patients died. Non-survivors had a higher number of involved organs, lower e', and higher rates of E/e' >15, pericardial effusion (PE), low voltage on an electrocardiogram and a New York Heart Association (NYHA) functional class ≥ III, compared with survivors. In multivariate analysis, a NYHA functional class ≥ III (p=0.024) and cardiac involvement (p=0.032) were independent predictors of PE in patients with AL amyloidosis. Multivariate Cox proportional hazard analysis indicated that PE (hazard ratio: 21.9, p=0.025) and the number of involved organs (hazard ratio: 2.8, p=0.015), but not LV diastolic parameters of tissue Doppler echocardiography, independently predict mortality in patients with AL amyloidosis.
  
  
  CONCLUSIONS
  PE and multiple organ involvement, compared with e' and E/e', are stronger predictors of mortality in patients with AL amyloidosis. The advanced disease stage of AL amyloidosis might underlie the strong association between PE and a poor outcome.
authors.affiliation: Department of Clinical Laboratory Medicine, Sapporo Medical University School of Medicine, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yuda
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Hayashi
authors.forename: Kenji
authors.initials: K
authors.lastname: Yasui
authors.forename: Atsuko
authors.initials: A
authors.lastname: Muranaka
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Ohnishi
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Hashimoto
authors.forename: Tadao
authors.initials: T
authors.lastname: Ishida
authors.forename: Kazufumi
authors.initials: K
authors.lastname: Tsuchihashi
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Shinomura
authors.forename: Naoki
authors.initials: N
authors.lastname: Watanabe
authors.forename: Tetsuji
authors.initials: T
authors.lastname: Miura
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000000981:diagnostic imaging / Q000401:mortality*; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000000981:diagnostic imaging / Q000401:mortality* / Q000503:physiopathology; D007147:Immunoglobulin Light Chains; D000075363:Immunoglobulin Light-chain Amyloidosis; D008297:Male; D008875:Middle Aged; D010490:Pericardial Effusion / Q000000981:diagnostic imaging / Q000401:mortality* / Q000503:physiopathology; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D016277:Ventricular Function, Left
chemical_list: D007147:Immunoglobulin Light Chains