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Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication Cover

Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication

By:   
Open Access
|Mar 2022

Figures & Tables

Table 1

Cardiovascular risk factors for increased light chain amyloidosis mortality.36,37,38,39,40,41,42,43,44,45,46,47,48 OS: overall survival; MRI: magnetic resonance imaging; EF: ejection fraction

FINDINGRISK FACTORNOTES
ArrhythmiasAtrial fibrillationInferior short-term OS but no impact on peritransplant mortality36,37
Nonsustained ventricular tachycardiaInferior short-term OS but no impact on peritransplant mortality36
Ventricular tachycardiaIncreased mortality37
Cardiac MRI FindingsLeft atrial EF < 16%Higher risk of 2-year mortality41
Long axis strain > –7%Higher risk of death and cardiac transplantation42
Myocardial contraction fraction < 52.6%Higher risk of death and cardiac transplantation42
Right ventricular dysfunctionPredicts all-cause mortality43
New York Heart Association functional class 3/4Increased mortality44
Clinical FindingsLeft heart valve thickening > 3 mmHigher all-cause mortality45
Echocardiographic FindingsInterventricular septal thickness > 15 mmInferior OS46
Relative wall thickness > 0.74Increased mortality44
Right atrial volume index > 35 and other signs of right ventricular dysfunctionHigh 1-year mortality37
Global longitudinal strain (GLS) < 17%5-year survival of 47% vs 95% if GLS > 17%38,39,40
Electrocardiographic FindingsAbnormal QRS axisPoorer survival47
Frontal QRS-T angle > 102Increased mortality48
QTc > 483 msecPoorer survival47
Table 2

Overall survival (OS) of light chain amyloidosis patients by stage at diagnosis and time from treatment initiation. Group 1: 2015 European modification of Mayo 2004 staging; Group 2: Mayo 2012 staging; NR: not reached; N/A: not assessed

GROUP 1 STAGE (1,357 PTS)MEDIAN OS AT DIAGNOSIS (YRS)MEDIAN OS AT 3 MO (YRS)MEDIAN OS AT 6 MO (YRS)
I1211.8NR
II5.410.8NR
IIIA1.84.65.4
IIIB0.41.10.9
GROUP 2 STAGE (1,339 PTS)
I11.411.8NR
II8.29.0NR
III2.45.24.6
IV0.50.80.9
DOI: https://doi.org/10.14797/mdcvj.1070 | Journal eISSN: 1947-6108
Language: English
Page range: 27 - 35
Submitted on: Dec 2, 2021
Accepted on: Feb 2, 2022
Published on: Mar 14, 2022
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services

© 2022 Kelty R. Baker, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.