Korean Journal of Radiology

The Korean Society of Radiology (대한영상의학회)
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Light-Chain Cardiac Amyloidosis: Cardiac Magnetic Resonance for Assessing Response to Chemotherapy

  • Yubo Guo (Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Xiao Li (Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Yajuan Gao (Department of Hematology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Kaini Shen (Department of Hematology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Lu Lin (Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Jian Wang (Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Jian Cao (Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Zhuoli Zhang (Department of Radiological Sciences, University of California) ;
  • Ke Wan (Department of Geriatrics and National Clinical Research Center for Geriatrics, West China Hospital, Sichuan University) ;
  • Xi Yang Zhou (Department of Diagnostic Radiology, Jinling Hospital, Medical School of Nanjing University) ;
  • Yucheng Chen (Department of Cardiology, West China Hospital, Sichuan University) ;
  • Long Jiang Zhang (Department of Diagnostic Radiology, Jinling Hospital, Medical School of Nanjing University) ;
  • Jian Li (Department of Hematology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Yining Wang (Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College)
  • Received : 2023.10.09
  • Accepted : 2024.03.18
  • Published : 2024.05.01
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Abstract

Objective: Cardiac magnetic resonance (CMR) is a diagnostic tool that provides precise and reproducible information about cardiac structure, function, and tissue characterization, aiding in the monitoring of chemotherapy response in patients with light-chain cardiac amyloidosis (AL-CA). This study aimed to evaluate the feasibility of CMR in monitoring responses to chemotherapy in patients with AL-CA. Materials and Methods: In this prospective study, we enrolled 111 patients with AL-CA (50.5% male; median age, 54 [interquartile range, 49-63] years). Patients underwent longitudinal monitoring using biomarkers and CMR imaging. At follow-up after chemotherapy, patients were categorized into superior and inferior response groups based on their hematological and cardiac laboratory responses to chemotherapy. Changes in CMR findings across therapies and differences between response groups were analyzed. Results: Following chemotherapy (before vs. after), there were significant increases in myocardial T2 (43.6 ± 3.5 ms vs. 44.6 ± 4.1 ms; P = 0.008), recovery in right ventricular (RV) longitudinal strain (median of -9.6% vs. -11.7%; P = 0.031), and decrease in RV extracellular volume fraction (ECV) (median of 53.9% vs. 51.6%; P = 0.048). These changes were more pronounced in the superior-response group. Patients with superior cardiac laboratory response showed significantly greater reductions in RV ECV (-2.9% [interquartile range, -8.7%-1.1%] vs. 1.7% [-5.5%-7.1%]; P = 0.017) and left ventricular ECV (-2.0% [-6.0%-1.3%] vs. 2.0% [-3.0%-5.0%]; P = 0.01) compared with those with inferior response. Conclusion: Cardiac amyloid deposition can regress following chemotherapy in patients with AL-CA, particularly showing more prominent regression, possibly earlier, in the RV. CMR emerges as an effective tool for monitoring associated tissue characteristics and ventricular functional recovery in patients with AL-CA undergoing chemotherapy, thereby supporting its utility in treatment response assessment.

Keywords

Acknowledgement

The authors thank each of the study subjects for their participation.

References

  1. Gertz MA. Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment. Am J Hematol 2022;97:818-829
  2. Falk RH, Alexander KM, Liao R, Dorbala S. AL (light-chain) cardiac amyloidosis: a review of diagnosis and therapy. J Am Coll Cardiol 2016;68:1323-1341
  3. Kyle RA, Linos A, Beard CM, Linke RP, Gertz MA, O'Fallon WM, et al. Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989. Blood 1992;79:1817-1822
  4. Jaccard A, Comenzo RL, Hari P, Hawkins PN, Roussel M, Morel P, et al. Efficacy of bortezomib, cyclophosphamide and dexamethasone in treatment-naive patients with high-risk cardiac AL amyloidosis (Mayo Clinic stage III). Haematologica 2014;99:1479-1485
  5. Palladini G, Campana C, Klersy C, Balduini A, Vadacca G, Perfetti V, et al. Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis. Circulation 2003;107:2440-2445
  6. Fontana M, Pica S, Reant P, Abdel-Gadir A, Treibel TA, Banypersad SM, et al. Prognostic value of late gadolinium enhancement cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 2015;132:1570-1579
  7. Banypersad SM, Fontana M, Maestrini V, Sado DM, Captur G, Petrie A, et al. T1 mapping and survival in systemic light-chain amyloidosis. Eur Heart J 2015;36:244-251
  8. Guo Y, Li X, Wang Y. State of the art: quantitative cardiac MRI in cardiac amyloidosis. J Magn Reson Imaging 2022;56:1287-1301
  9. Gertz MA, Dispenzieri A. Systemic amyloidosis recognition, prognosis, and therapy: a systematic review. JAMA 2020;324:79-89
  10. Palladini G, Dispenzieri A, Gertz MA, Kumar S, Wechalekar A, Hawkins PN, et al. New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes. J Clin Oncol 2012;30:4541-4549
  11. Messroghli DR, Moon JC, Ferreira VM, Grosse-Wortmann L, He T, Kellman P, et al. Clinical recommendations for cardiovascular magnetic resonance mapping of T1, T2, T2* and extracellular volume: a consensus statement by the Society for Cardiovascular Magnetic Resonance (SCMR) endorsed by the European Association for Cardiovascular Imaging (EACVI). J Cardiovasc Magn Reson 2017;19:75
  12. Lin L, Li X, Feng J, Shen KN, Tian Z, Sun J, et al. The prognostic value of T1 mapping and late gadolinium enhancement cardiovascular magnetic resonance imaging in patients with light chain amyloidosis. J Cardiovasc Magn Reson 2018;20:2
  13. Yim D, Riesenkampff E, Caro-Dominguez P, Yoo SJ, Seed M, Grosse-Wortmann L. Assessment of diffuse ventricular myocardial fibrosis using native T1 in children with repaired tetralogy of fallot. Circ Cardiovasc Imaging 2017;10:e005695
  14. Li X, Huang S, Han P, Zhou Z, Azab L, Lu M, et al. Nonenhanced chemical exchange saturation transfer cardiac magnetic resonance imaging in patients with amyloid light-chain amyloidosis. J Magn Reson Imaging 2022;55:567-576
  15. Chamling B, Bietenbeck M, Drakos S, Korthals D, Vehof V, Stalling P, et al. A compartment-based myocardial density approach helps to solve the native T1 vs. ECV paradox in cardiac amyloidosis. Sci Rep 2022;12:21755
  16. Leone O, Longhi S, Quarta CC, Ragazzini T, De Giorgi LB, Pasquale F, et al. New pathological insights into cardiac amyloidosis: implications for non-invasive diagnosis. Amyloid 2012;19:99-105
  17. Martinez-Naharro A, Patel R, Kotecha T, Karia N, Ioannou A, Petrie A, et al. Cardiovascular magnetic resonance in light-chain amyloidosis to guide treatment. Eur Heart J 2022;43:4722-4735
  18. Martinez-Naharro A, Abdel-Gadir A, Treibel TA, Zumbo G, Knight DS, Rosmini S, et al. CMR-verified regression of cardiac AL amyloid after chemotherapy. JACC Cardiovasc Imaging 2018;11:152-154
  19. Fontana M, Martinez-Naharro A, Chacko L, Rowczenio D, Gilbertson JA, Whelan CJ, et al. Reduction in CMR derived extracellular volume with patisiran indicates cardiac amyloid regression. JACC Cardiovasc Imaging 2021;14:189-199
  20. Bodez D, Ternacle J, Guellich A, Galat A, Lim P, Radu C, et al. Prognostic value of right ventricular systolic function in cardiac amyloidosis. Amyloid 2016;23:158-167
  21. Sengupta PP, Tajik AJ, Chandrasekaran K, Khandheria BK. Twist mechanics of the left ventricle: principles and application. JACC Cardiovasc Imaging 2008;1:366-376
  22. Cohen OC, Ismael A, Pawarova B, Manwani R, Ravichandran S, Law S, et al. Longitudinal strain is an independent predictor of survival and response to therapy in patients with systemic AL amyloidosis. Eur Heart J 2022;43:333-341
  23. Cuddy SAM, Jerosch-Herold M, Falk RH, Kijewski MF, Singh V, Ruberg FL, et al. Myocardial composition in light-chain cardiac amyloidosis more than 1 year after successful therapy. JACC Cardiovasc Imaging 2022;15:594-603
  24. Zheng Y, Huang S, Xie B, Zhang N, Liu Z, Tse G, et al. Cardiovascular toxicity of proteasome inhibitors in multiple myeloma therapy. Curr Probl Cardiol 2023;48:101536
  25. Kim J, Hong YJ, Han K, Kim JY, Lee HJ, Hur J, et al. Chemotherapy-related cardiac dysfunction: quantitative cardiac magnetic resonance image parameters and their prognostic implications. Korean J Radiol 2023;24:838-848
  26. Edwards CV, Rao N, Bhutani D, Mapara M, Radhakrishnan J, Shames S, et al. Phase 1a/b study of monoclonal antibody CAEL-101 (11-1F4) in patients with AL amyloidosis. Blood 2021;138:2632-2641
  27. Cuddy SAM, Bravo PE, Falk RH, El-Sady S, Kijewski MF, Park MA, et al. Improved quantification of cardiac amyloid burden in systemic light chain amyloidosis: redefining early disease? JACC Cardiovasc Imaging 2020;13:1325-1336