Primary systemic amyloidosis presenting as angina pectoris due to intramyocardial coronary artery involvement: a case report

• Published in: Heart and vessels Vol. 16; no. 4; pp. 157 - 160
• Main Authors: Yamano, S., Motomiya, Kotomi, Akai, Yasuhiro, Shiiki, Hideo, Toyama, Takayuki, Hashimoto, Toshio, Fujimoto, Shinichi, Nakamura, Shinobu, Fukuoka, Yoshiaki
• Format: Journal Article
• Language: English
• Published: Japan Springer Nature B.V 01.05.2002
• Subjects: Aged; Amyloidosis - complications; Amyloidosis - diagnosis; Angina pectoris; Angina Pectoris - diagnosis; Angina Pectoris - etiology; Arteries - pathology; Autopsy; Cardiomyopathies - complications; Cardiomyopathies - diagnosis; Case studies; Coronary Artery Disease - complications; Coronary Artery Disease - diagnosis; Coronary vessels; Coronary Vessels - pathology; Diagnosis, Differential; Electrocardiography; Female; Humans; Myocardium - pathology
• ISSN: 0910-8327; 1615-2573
• DOI: 10.1007/s003800200013

We describe a 76-year-old Japanese woman with primary systemic amyloidosis who presented with angina pectoris associated with ST-segment and T-wave abnormalities resulting from intramyocardial coronary artery amyloidosis. The patient was admitted to our hospital because of dyspnea and pretibial edema 7 years after the diagnosis of variant angina. A diagnosis of primary systemic amyloidosis (AL amyloid protein) was made after examination of gastric and endomyocardial biopsy specimens. The patient died of progressive, uncontrolled heart failure 3 months later. An autopsy study demonstrated only mild-to-moderate atherosclerosis in the epicardial coronary arteries. However, histological examination of the heart revealed diffuse stenoses and obstructions in the intramural coronary arteries by amyloid deposits. This patient had small-vessel coronary disease with ST-segment changes and angina caused by cardiac amyloidosis. A correct diagnosis of ischemic heart disease due to primary amyloidosis is important for estimation of the prognosis and for appropriate management.