Late gadolinium enhancement in cardiac amyloidosis: attributable both to interstitial amyloid deposition and subendocardial fibrosis caused by ischemia

• Published in: Heart and vessels Vol. 31; no. 6; pp. 990 - 995
• Main Authors: Hashimura, Hiromi, Ishibashi-Ueda, Hatsue, Yonemoto, Yumiko, Ohta-Ogo, Keiko, Matsuyama, Taka-aki, Ikeda, Yoshihiko, Morita, Yoshiaki, Yamada, Naoaki, Yasui, Hiroki, Naito, Hiroaki
• Format: Journal Article
• Language: English
• Published: Tokyo Springer Japan 01.06.2016; Springer Nature B.V
• Subjects: Amyloid - analysis; Amyloidosis - diagnostic imaging; Amyloidosis - metabolism; Amyloidosis - pathology; Amyloidosis - physiopathology; Autopsy; Biomedical Engineering and Bioengineering; Cardiac Surgery; Cardiology; Cardiomyopathies - diagnostic imaging; Cardiomyopathies - metabolism; Cardiomyopathies - pathology; Cardiomyopathies - physiopathology; Cardiovascular disease; Case Report; Contrast Media - administration & dosage; Coronary Artery Disease - diagnostic imaging; Coronary Artery Disease - metabolism; Coronary Artery Disease - pathology; Coronary Artery Disease - physiopathology; Coronary Circulation; Coronary Vessels - chemistry; Coronary Vessels - diagnostic imaging; Coronary Vessels - pathology; Coronary Vessels - physiopathology; Fatal Outcome; Fibrosis; Gadolinium DTPA - administration & dosage; Humans; Ischemia; Magnetic Resonance Imaging; Male; Medicine; Medicine & Public Health; Middle Aged; Myocardial Perfusion Imaging - methods; Myocardium - chemistry; Myocardium - pathology; NMR; Nuclear magnetic resonance; Pathology; Predictive Value of Tests; Vascular Surgery; Pathology; Cardiac amyloidosis; Magnetic resonance imaging; Late gadolinium enhancement
• ISSN: 0910-8327; 1615-2573
• DOI: 10.1007/s00380-015-0658-0

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.