title: Prognosis of Light Chain Amyloidosis With Preserved LVEF: Added Value of 2D Speckle-Tracking Echocardiography to the Current Prognostic Staging System.
abstract: This study evaluated whether 2-dimensional speckle-tracking echocardiography (2D-STE) has incremental value for prognosis over traditional clinical, echocardiographic, and serological markers-with main focus on the current prognostic staging system-in light-chain (AL) amyloidosis patients with preserved left ventricular ejection fraction.
  
  
  
  Cardiac amyloidosis (CA) is the major determinant of outcome in AL amyloidosis. The current prognostic staging system is based primarily on serum levels of cardiac troponin T (cTnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and free light chain differential (FLC-diff).
  
  
  
  Consecutive patients with biopsy-proven AL amyloidosis and left ventricular ejection fraction ≥55% were divided into group 1 with CA (n = 63) and group 2 without CA (n = 87). Global longitudinal strain (GLS) by 2D-STE was performed with Vivid E9 (GE Healthcare Co., Milwaukee, Wisconsin) and syngo Velocity Vector Imaging (VVI) software (Siemens Medical Solutions USA, Inc., Malvern, Pennsylvania) (GLSGEand GLSVVI, respectively).
  
  
  
  Thirty-two deaths (51%) occurred in group 1 and 13 (15%) in group 2 (p ≤ 0.001). Group 1 had thicker walls, lower early diastolic tissue Doppler velocity at septal mitral annulus, and greater left ventricular mass, left atrial volume, glomerular filtration rate, FLC-diff, cTnT, and NT-proBNP (p < 0.001). For the entire cohort, GLSGE≥ -14.81, GLSVVI≥-15.02, cTnT, NT-proBNP, FLC-diff, age, left ventricular wall thickness, early diastolic tissue Doppler velocity at septal mitral annulus, diastolic dysfunction grade, glomerular filtration rate, deceleration time, and left atrial volume were univariate predictors of death. In a multivariate Cox model, GLSGE≥-14.81 (hazard ratio [HR]: 2.68; 95% confidence interval [CI]: 1.07 to 7.13; p = 0.03), FLC-diff, NT-proBNP, and age were independent predictors of survival. There was also a strong trend for GLSVVI≥-15.02 (HR: 2.44; 95% CI: 0.98 to 6.33; p = 0.055). Using a nested logistic regression model, GLSGE(p = 0.03) and GLSVVI(p = 0.05) provided incremental prognostic value over cTnT, NT-proBNP, and FLC-diff. For survival analysis limited to group 2 (non-CA), GLSGEand GLSVVIboth predicted all-cause mortality (GLSGEHR: 1.23; 95% CI: 1.03 to 1.47 [p = 0.02]; GLSVVIHR: 1.22; 95% CI: 1.01 to 1.49 [p = 0.04], respectively).
  
  
  
  2D-STE predicted outcome and provided incremental prognostic information over the current prognostic staging system, especially in the group without CA.
authors.affiliation: Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Sergio
authors.initials: S
authors.lastname: Barros-Gomes
authors.affiliation: Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Brittney
authors.initials: B
authors.lastname: Williams
authors.affiliation: Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Lara F
authors.initials: LF
authors.lastname: Nhola
authors.affiliation: Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Martha
authors.initials: M
authors.lastname: Grogan
authors.affiliation: Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Joseph F
authors.initials: JF
authors.lastname: Maalouf
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.affiliation: Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Patricia A
authors.initials: PA
authors.lastname: Pellikka
authors.affiliation: Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address: villarraga.hector@mayo.edu.
authors.forename: Hector R
authors.initials: HR
authors.lastname: Villarraga
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000097:blood / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D015415:Biomarkers / Q000097:blood; D001696:Biomechanical Phenomena; D001706:Biopsy; D009202:Cardiomyopathies / Q000097:blood / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D016022:Case-Control Studies; D016009:Chi-Square Distribution; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D007090:Image Interpretation, Computer-Assisted; D007147:Immunoglobulin Light Chains / Q000097:blood*; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009200:Myocardial Contraction; D009206:Myocardium / Q000378:metabolism / Q000473:pathology*; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012307:Risk Factors; D012720:Severity of Illness Index; D013318:Stroke Volume; D020107:Troponin T / Q000097:blood; D016277:Ventricular Function, Left
chemical_list: D015415:Biomarkers; D007147:Immunoglobulin Light Chains; D010446:Peptide Fragments; D020107:Troponin T; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
keywords: amyloid; cardiac amyloidosis; cardiomyopathy; echocardiography; mortality