title: Late gadolinium enhancement in cardiac amyloidosis: attributable both to interstitial amyloid deposition and subendocardial fibrosis caused by ischemia.
abstract: Gadolinium contrast agents used for late gadolinium enhancement (LGE) distribute in the extracellular space. Global diffuse myocardial LGE pronounced in the subendocardial layers is common in cardiac amyloidosis. However, the pathophysiological basis of these findings has not been sufficiently explained. A 64-year-old man was admitted to our hospital with leg edema and nocturnal dyspnea. Bence Jones protein was positive in the urine, and an endomyocardial and skin biopsy showed light-chain (AL) amyloidosis. He died of ventricular fibrillation 3 months later. 9 days before death, the patient was examined by cardiac magnetic resonance (CMR) imaging on a 3-T system. We acquired LGE data at 2, 5, 10, and 20 min after the injection of gadolinium contrast agents, with a fixed inversion time of 350 ms. Myocardial LGE developed sequentially. The myocardium was diffusely enhanced at 2 min, except for the subendocardium, but LGE had extended to almost the entire left ventricle at 5 min and predominantly localized to the subendocardial region at 10 and 20 min. An autopsy revealed massive and diffused amyloid deposits in perimyocytes throughout the myocardium. Old and recent ischemic findings, such as replacement fibrosis and coagulative myocyte necrosis, were evident in the subendocardium. In the intramural coronary arteries, mild amyloid deposits were present within the subepicardial to the mid layer of the left ventricle, but no stenotic lesions were evident. However, capillaries were obstructed by amyloid deposits in the subendocardium. In conclusion, the late phase of dynamic LGE (at 10 and 20 min) visualized in the subendocardium corresponded to the interstitial amyloid deposition and subendocardial fibrosis caused by ischemia in our patient.
authors: Department of Pathology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka, Japan.|Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Japan.
authors: Hiromi
authors: H
authors: Hashimura
authors: Department of Pathology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka, Japan. hueda@hsp.ncvc.go.jp.
authors: Hatsue
authors: H
authors: Ishibashi-Ueda
authors: Department of Pathology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka, Japan.
authors: Yumiko
authors: Y
authors: Yonemoto
authors: Department of Pathology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka, Japan.
authors: Keiko
authors: K
authors: Ohta-Ogo
authors: Department of Pathology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka, Japan.
authors: Taka-Aki
authors: TA
authors: Matsuyama
authors: Department of Pathology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka, Japan.
authors: Yoshihiko
authors: Y
authors: Ikeda
authors: Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Japan.
authors: Yoshiaki
authors: Y
authors: Morita
authors: Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Japan.
authors: Naoaki
authors: N
authors: Yamada
authors: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan.
authors: Hiroki
authors: H
authors: Yasui
authors: Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Japan.
authors: Hiroaki
authors: H
authors: Naito
mesh_terms: D000682:Amyloid / Q000032:analysis*; D000686:Amyloidosis / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D001344:Autopsy; D009202:Cardiomyopathies / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D003287:Contrast Media / Q000008:administration & dosage*; D003324:Coronary Artery Disease / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D003326:Coronary Circulation; D003331:Coronary Vessels / Q000737:chemistry / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D017809:Fatal Outcome; D005355:Fibrosis; D019786:Gadolinium DTPA / Q000008:administration & dosage*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D055414:Myocardial Perfusion Imaging / Q000379:methods*; D009206:Myocardium / Q000737:chemistry / Q000473:pathology; D011237:Predictive Value of Tests
chemical_list: D000682:Amyloid; D003287:Contrast Media; D019786:Gadolinium DTPA
keywords: Cardiac amyloidosis; Late gadolinium enhancement; Magnetic resonance imaging; Pathology