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. 2025 Sep 18;5(5):oeaf104.
doi: 10.1093/ehjopen/oeaf104. eCollection 2025 Sep.

Improvement in global longitudinal strain following plasma cell-directed therapy is associated with long-term survival among patients with AL amyloidosis

Kristine H Jang  1 , Anthony F Yu  2   3 , Heather Landau  3   4 , Xiaoyue Ma  5 , Richard K Cheng  6 , Mathew S Mauer  7 , Katherine Lee Chuy  2 , Daniel Lenihan  8   9 , Ji Can Yang  10 , Carol L Chen  2   3 , Jennifer E Liu  2   3
Affiliations

Improvement in global longitudinal strain following plasma cell-directed therapy is associated with long-term survival among patients with AL amyloidosis

Kristine H Jang et al. Eur Heart J Open. .

Abstract

Aims: Cardiac impairment in AL amyloidosis is the major determinant of survival. Treatment goals include reducing circulating light chains to improve organ function. Global longitudinal strain (GLS) is an independent predictor of survival and useful for assessing cardiac function before and after therapy. This study aimed to describe GLS change from baseline to one year post-treatment, identify factors associated with GLS improvement (GLS+), and evaluate its prognostic significance.

Methods and results: Ninety-seven patients with AL amyloidosis and cardiac stage II/III disease who underwent echocardiogram and haematologic evaluation at baseline and one year were included. GLS+ was defined as a 2.0%-point increase. A cardiac or B-type natriuretic peptide (BNP+) response was defined as a 30% reduction from baseline. Overall survival was measured from baseline echocardiogram to death. Of 97 patients, 62% had Stage II, 29% Stage IIIa, and 9% Stage IIIb disease. Baseline median left ventricular ejection fraction, GLS, and septal thickness were 65%, -14.9%, and 1.3 cm, respectively. GLS+ was observed in 36% of patients and BNP+ in 51%. Median overall survival was 113.4 months. The hazard ratio for survival was 0.42 in the GLS+ group and 0.46 in the BNP+ group, after adjusting for haematologic response.

Conclusion: GLS improvement post-treatment confers a significant survival benefit. This study supports GLS as an important marker for risk stratification and cardiac response.

Keywords: AL amyloidosis; Cardiomyopathy; Echocardiography; Global longitudinal strain; Prognosis.

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Conflict of interest statement

Conflict of interest: A.F.Y.: consultancy for Genentech and Avacta; grant support from Merck. H.L.: consultancy for Pfizer, Prothena, Legend Biotech USA, Takeda Pharmaceuticals, Janssen Pharmaceuticals; research funding from Alexion Pharmaceuticals, Takeda Pharmaceuticals. M.S.M.: researcher for NIH R01HL139671 and R01AG081582-01, Alnylam Pharmaceuticals, BridgeBio (formerly Eidos Therapeutics), Ionis Pharmaceuticals, Pfizer, and Prothena Biosciences; and as a consultant or advisor for Alnylam Pharmaceuticals, AstraZeneca, Attralus, BridgeBio (formerly Eidos Therapeutics), Intellia Therapeutics, Ionis Pharmaceuticals, Novo Nordisk, and Pfizer. D.L.: consultant for Myocardial Solutions, AstraZeneca, Clementia, and Intellia; speaker for BridgeBio. J.E.L.: DMSB for Caelum Biosciences; research support from Johnson and Johnson; speaking fees from GE Healthcare and Philips Medical. The remaining authors have nothing to disclose.

Figures

Graphical Abstract
Graphical Abstract
Figure 1
Figure 1
Kaplan–Meier survival curves based on haematologic response at 1 year following treatment. dFLC, difference between involved and uninvolved light chains. Patients who achieved deep response showed improved survival than patients who did not (median overall survival 120.2 vs. 90.2 months, respectively, P = 0.02). Product-limit survival estimates with number of subjects at risk and 95% confidence limits.
Figure 2
Figure 2
Kaplan–Meier survival curves based on BNP response at one year following treatment. Patients with a positive BNP response showed significantly better survival than patients with a negative BNP response (median overall survival 90.2 months for BNP(−) and not reached for BNP(+), P = 0.006), Product-limit survival estimates with number of subjects at risk and 95% confidence limits. BNP, B-type natriuretic peptide.
See this image and copyright information in PMC

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