Sarah A M Cuddy1, Michael Jerosch-Herold2, Rodney H Falk3, Marie Foley Kijewski4, Vasvi Singh5, Frederick L Ruberg6, Vaishali Sanchorawala6, Heather Landau7, Matthew S Maurer8, Andrew J Yee9, Giada Bianchi10, Marcelo F Di Carli5, Ronglih Liao11, Raymond Y Kwong5, Sharmila Dorbala12. 1. Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA; Cardiovascular Imaging Program, Cardiovascular Division and Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA. 2. Cardiovascular Imaging Program, Cardiovascular Division and Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA. 3. Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA. 4. Division of Nuclear Medicine, Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA. 5. Cardiovascular Imaging Program, Cardiovascular Division and Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA; Division of Nuclear Medicine, Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA. 6. Section of Cardiovascular Medicine, Department of Medicine, Boston Medical Center, Boston University School of Medicine, Boston, Massachusetts, USA. 7. Division of Medical Oncology, Memorial Sloan Kettering Medical Center, New York, New York, USA. 8. Division of Cardiology, Columbia University Irving Medical Center, New York, New York, USA. 9. Division of Hematology and Oncology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA. 10. Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA. 11. Amyloidosis Program, Stanford University, Stanford, California, USA. 12. Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA; Cardiovascular Imaging Program, Cardiovascular Division and Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA; Division of Nuclear Medicine, Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA. Electronic address: sdorbala@bwh.harvard.edu.
Abstract
OBJECTIVES: The goals of this study were to characterize myocardial composition during the active and remission phases of light-chain (AL) cardiac amyloidosis. BACKGROUND: Cardiac dysfunction in AL amyloidosis is characterized by dual insults to the myocardium from infiltration and toxicity from light chains during the active phase and by infiltration alone in the remission phase. METHODS: Prospectively enrolled subjects with cardiac AL amyloidosis (21 remission AL amyloidosis; age: 63.4 ± 7.3 years; 47.6% male; and 48 active AL amyloidosis; age: 62.5 ± 7.4 years; 60.4% male) underwent contrast-enhanced cardiac magnetic resonance with T1 and T2 mapping and measurement of extracellular volume (ECV). By definition, serum free light-chain levels were normal for at least 1 year following successful AL therapy in the remission group and abnormal in the active group. RESULTS: Myocardial ECV was similarly expanded in the remission and active AL amyloidosis groups (0.488 ± 0.082 vs 0.519 ± 0.083, respectively; P = 0.15). However, myocardial T2 relaxation times (47.7 ± 3.2 ms vs 45.5 ± 3.0 ms; P = 0.008) as well as native T1 times (1,368 ms [IQR: 1,290-1,422 ms] vs 1,264 ms [IQR: 1,203-1,380 ms]; P = 0.024) were significantly higher in the remission compared to the active AL amyloidosis group. CONCLUSIONS: Myocardial ECV is substantially expanded in the active AL and remission AL cardiac amyloidosis groups, but native T1 values were higher, suggesting a different myocardial composition. There is no evidence of myocardial edema in active AL cardiac amyloidosis. Future phenotyping studies of AL cardiac amyloidosis need to consider complementary myocardial markers that define the interstitial milieu in addition to changes in extracellular volume. (Molecular Imaging of Primary Amyloid Cardiomyopathy; NCT02641145).
OBJECTIVES: The goals of this study were to characterize myocardial composition during the active and remission phases of light-chain (AL) cardiac amyloidosis. BACKGROUND: Cardiac dysfunction in AL amyloidosis is characterized by dual insults to the myocardium from infiltration and toxicity from light chains during the active phase and by infiltration alone in the remission phase. METHODS: Prospectively enrolled subjects with cardiac AL amyloidosis (21 remission AL amyloidosis; age: 63.4 ± 7.3 years; 47.6% male; and 48 active AL amyloidosis; age: 62.5 ± 7.4 years; 60.4% male) underwent contrast-enhanced cardiac magnetic resonance with T1 and T2 mapping and measurement of extracellular volume (ECV). By definition, serum free light-chain levels were normal for at least 1 year following successful AL therapy in the remission group and abnormal in the active group. RESULTS: Myocardial ECV was similarly expanded in the remission and active AL amyloidosis groups (0.488 ± 0.082 vs 0.519 ± 0.083, respectively; P = 0.15). However, myocardial T2 relaxation times (47.7 ± 3.2 ms vs 45.5 ± 3.0 ms; P = 0.008) as well as native T1 times (1,368 ms [IQR: 1,290-1,422 ms] vs 1,264 ms [IQR: 1,203-1,380 ms]; P = 0.024) were significantly higher in the remission compared to the active AL amyloidosis group. CONCLUSIONS: Myocardial ECV is substantially expanded in the active AL and remission AL cardiac amyloidosis groups, but native T1 values were higher, suggesting a different myocardial composition. There is no evidence of myocardial edema in active AL cardiac amyloidosis. Future phenotyping studies of AL cardiac amyloidosis need to consider complementary myocardial markers that define the interstitial milieu in addition to changes in extracellular volume. (Molecular Imaging of Primary Amyloid Cardiomyopathy; NCT02641145).
Authors: Andrea Baggiano; Michele Boldrini; Ana Martinez-Naharro; Tushar Kotecha; Aviva Petrie; Tamer Rezk; Maurizio Gritti; Cristina Quarta; Daniel S Knight; Ashutosh D Wechalekar; Helen J Lachmann; Stefano Perlini; Gianluca Pontone; James C Moon; Peter Kellman; Julian D Gillmore; Philip N Hawkins; Marianna Fontana Journal: JACC Cardiovasc Imaging Date: 2019-06-12
Authors: Sarah A M Cuddy; Paco E Bravo; Rodney H Falk; Samir El-Sady; Marie Foley Kijewski; Mi-Ae Park; Frederick L Ruberg; Vaishali Sanchorawala; Heather Landau; Andrew J Yee; Giada Bianchi; Marcelo F Di Carli; Su-Chun Cheng; Michael Jerosch-Herold; Raymond Y Kwong; Ronglih Liao; Sharmila Dorbala Journal: JACC Cardiovasc Imaging Date: 2020-05-13
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