Author + information
- Katherine Lee Chuy, MDa,
- Esther Drill, DrPHb,
- Ji Can Yang, DOc,
- Heather Landau, MDd,e,
- Hani Hassoun, MDe,f,
- Omar Nahhas, MDg,
- Carol L. Chen, MDe,g,
- Anthony F. Yu, MDe,g,
- Richard M. Steingart, MDe,g and
- Jennifer E. Liu, MDe,g,∗ (, )@JLiu_MSKCardOnc
- aDepartment of Cardiology, Cook County Health, Chicago, Illinois
- bDepartment of Epidemiology and Biostatistics, Memorial Sloan Kettering Cancer Center, New York, New York
- cNew York Presbyterian–Brooklyn Methodist Hospital, New York, New York
- dAdult Bone Marrow Transplant Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York
- eDepartment of Medicine, Weill Cornell Medical College, New York, New York
- fMyeloma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York
- gCardiology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York
- ↵∗Address for correspondence:
Dr. Jennifer E. Liu, Department of Medicine, Weill Cornell Medical College, 1275 York Avenue, New York, New York 10065.
Background Advanced light-chain (AL) amyloidosis is associated with poor prognosis, with a 5-year survival rate of <25%. Prognostication is based on the revised Mayo (rMayo) staging according to serum cardiac biomarkers.
Objectives This study sought to determine whether global longitudinal strain (GLS) can provide incremental prognostic value in patients with advanced disease.
Methods Baseline (pre-treatment) clinical, 2-dimensional echocardiogram with GLS and laboratory data were collected prospectively in 94 patients with newly diagnosed AL amyloidosis with rMayo stage III or IV disease. Overall survival (OS) was defined as time from baseline echocardiography to death.
Results Of 94 patients, 60% (n = 56) had rMayo stage III and 40% (n = 38) had stage IV disease. Ninety of the 94 patients underwent plasma cell-directed therapy. The median left ventricular ejection fraction (LVEF) was 60%, and the median GLS was 13.2%. Of 94 patients, 64 died during follow-up. The median OS was 11.2 months, with an estimated 5-year OS of 21%. In univariable analysis, brain natriuretic peptides, GLS, LVEF, E/e′ ratio, and rMayo stage were significantly associated with OS. In Cox regression, GLS provided incremental value over brain natriuretic peptide, troponin, and LVEF for predicting OS. Patients with GLS < –14.2% had a corresponding median OS and 5-year OS rate of 33.2 months and 39%, respectively, versus 7.7 months and 6% for those with GLS ≥ –14.2%. This difference was maintained despite further stratification by rMayo stage.
Conclusions Baseline GLS is an independent predictor of OS beyond the circulating biomarkers and can identify groups with different survival outcomes beyond the Mayo Staging.
Dr. Landau has received research funding from Amgen, Spectrum Pharmaceuticals, and Takeda Pharmaceuticals; has provided consultancy for Karyopharm; and has served on the advisory board for Janssen, Celgene, Caelum Pharmaceuticals, Takeda Pharmaceuticals, and Spectrum Pharmaceuticals. Dr. Hassoun has received research funding and served on the advisory board for Takeda and Celgene; and has provided consultancy for Novartis. Dr. Yu has provided consultancy for Eidos Therapeutics, Genentech, and Glenmark. Dr. Liu has served on the advisory board for Pfizer; and provided consultancy for Bay Labs. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: CardioOncology author instructions page.
- Received February 26, 2020.
- Revision received April 3, 2020.
- Accepted April 5, 2020.
- 2020 The Authors