Fig. 1

Images of Case 1. A Transthoracic echocardiogram showed left ventricular dysfunction (ejection fraction 26%), severe aortic stenosis, and moderate functional mitral regurgitation. B Pathology of the left atrial appendage showed diffuse interstitial amyloid deposits by Hematoxylin-Eosin and direct fast scarlet staining, positive for atrial natriuretic peptide immunohistochemical staining, as well as nodular amyloid deposits positive for transthyretin (asterisks). C Pathology of the aortic valve revealed nodular amyloid deposits by direct fast scarlet staining with apple-green birefringence under polarized light microscopy, positive for transthyretin immunohistochemical staining. D ^99mTechnetium-pyrophophate scintigraphy showed no significant myocardial uptake, and cardiac magnetic resonance showed no late gadolinium enhancement and normal extracellular volume fraction (29.9%)