Impact of Monoclonal Light Chain Suppression Therapy on Global Longitudinal Strain in Patients with Advanced Light Chain Amyloidosis

The major determinant of survival in patients with advanced light chain amyloidosis (AL) is cardiac dysfunction. The mainstay of AL is monoclonal light chain suppression therapy; however, it is unclear whether this treatment can improve cardiac function. Echocardiogram with global longitudinal strain (GLS) has been recommended as a means of follow-up in patients at risk of cancer chemotherapy-related left ventricular systolic dysfunction.1

A total of 94 patients with newly diagnosed AL who had been diagnosed with Mayo stage III or IV disease from 2007 to 2018 were included in a study conducted by Omar Nahhas, MD, and colleagues at Memorial Sloan Kettering Cancer Center in New York, NY.2

Prior to treatment and 1 year after treatment, researchers collected echocardiogram with GLS, clinical, and laboratory data.

Of the patients included in the study, 40% (N = 38) had Mayo stage IV disease and 60% (N = 56) had Mayo stage III disease. Thirty-three of the 90 patients (95%) who underwent plasma cell–directed therapy were alive with available hematologic markers and echocardiogram at 1 year posttreatment. Those who achieved complete remission at 1 year demonstrated significant improvement in GLS and troponin from baseline compared with patients who achieved noncomplete remission (14.8% vs 18.9%, P = .03 and 0.11-0.04 μg/mL, P = .01, respectively) with no change in left ventricular ejection fraction or interventricular septum thickness. Patients with noncomplete remission showed no change in GLS or cardiac biomarkers and a drop in left ventricular ejection fraction (61% vs 56%; P = .02).

When stratified according to noncomplete remission compared with complete remission, those patients who achieved complete remission at 1 year showed marked improvement in overall survival as defined by hematologic response.

While it is unclear whether there is prognostic value in GLS improvement, future studies may find additional evidence. This study is the first to demonstrate that complete remission is associated with improvement in GLS in patients with advanced AL.


  1. Yang H, Wright L, Negishi T, et al. Assessment of left ventricular global longitudinal strain for surveillance of cancer chemotherapeutic-related cardiac dysfunction. JACC Cardiovasc Imaging. 2018;11:1196-1201.
  2. Nahhas O, Chuy KL, Yang JC, et al. Does light chain suppression therapy improve cardiac structure and function in patients with advanced AL amyloidosis? Presented at: American College of Cardiology 2020/World Congress of Cardiology Virtual Meeting. Abstract 1415-133.

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