Predictors of cardiac involvement and survival in patients with primary systemic light-chain amyloidosis: roles of the clinical, chemical, and 3-D speckle tracking echocardiography parameters

Changhui Lei,Lei Zuo,David H Hsi,Yan Wang,Jing Wang,Lei Xu,Liwen Liu,Xiaoli Zhu,Xueli Zhao,Shengjun Ta,Shiren Sun,Qianli Yang

doi:10.1186/s12872-021-01856-3

Changhui Lei, Lei Zuo + Show 10 more

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https://doi.org/10.1186/s12872-021-01856-3

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Abstract

BackgroundLight-chain (AL) amyloidosis is the most common type of systemic amyloidosis with poor prognosis. Currently, the predictors of cardiac involvement and prognostic staging systems are primarily based on conventional echocardiography and serological biomarkers. We used three-dimensional speckle tracking echocardiography (STE-3D) measurements of strain, hypothesizing that it could detect cardiac involvement and aid in prediction of mortality.MethodsWe retrospectively analysed 74 consecutive patients with biopsy-proven AL amyloidosis. Among them, 42 showed possible cardiac involvement and 32 without cardiac involvement. LV global longitudinal strain (GLS), global radial strain, global circumferential strain and global area strain (GAS) measurements were obtained.ResultsThe GLS and GAS were considered significant predictors of cardiac involvement. The cut-off values discriminating cardiac involvement were 16.10% for GLS, 32.95% for GAS. During the median follow-up of 12.5 months (interquartile range 4–25 months), 20 (27%) patients died. For the Cox proportional model survival analysis, heart rate, cardiac troponin T, NT-proBNP levels, E/e’, GLS, and GAS were univariate predictors of death. Multivariate Cox model showed that GLS ≤ 14.78% and cardiac troponin T ≥ 0.049 mg/l levels were independent predictors of survival.ConclusionsSTE-3D measurements of LV myocardial mechanics could detect cardiac involvement in patients with AL amyloidosis; GLS and cardiac biomarkers can provided prognostic information for mortality prediction.

Highlights

Amyloidosis is a disease where the deposition of an amorphous protein-derived substance in the extracellular compartment results in damage to numerous organs.Light-chain (AL) amyloidosis is the most common subtype [1] and is effectively treated with chemotherapy if captured early [2]
Mayo cardiac stage was significantly associated with cardiac involvement (P < 0.001), For the entire study population, all 32 patients without cardiac involvement were of Mayo stage I and II
Among 42 patients with cardiac involvement, 11 (26.2%) patients were with Mayo stage I and II, while 31(73.8%) patients were with Mayo stage III and IV

Summary

Introduction

Light-chain (AL) amyloidosis is the most common subtype [1] and is effectively treated with chemotherapy if captured early [2]. Cardiac amyloidosis (CA) involves deposition of amyloid fibrils in the myocardial interstitium [4] and portends a poor prognosis. More than half of the patients with AL amyloidosis (51–63%) demonstrate cardiac involvement on diagnosis [5, 6]. Light-chain (AL) amyloidosis is the most common type of systemic amyloidosis with poor prognosis. The predictors of cardiac involvement and prognostic staging systems are primarily based on conventional echocardiography and serological biomarkers. We used three-dimensional speckle tracking echocardiography (STE3D) measurements of strain, hypothesizing that it could detect cardiac involvement and aid in prediction of mortality

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