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A 65-year-old woman was admitted to our hospital with heart failure secondary to aortic regurgitation and paroxysmal atrial fibrillation. During surgery for valve-sparing aortic root replacement, a biopsy was obtained from the right atrium because of paroxysmal AF and mild LV hypokinesis. A histopathological examination revealed atrial natriuretic peptide amyloidosis, and the patient was clinically diagnosed with isolated atrial amyloidosis (IAA). Although IAA is rarely diagnosed clinically, it should be considered in patients with AF and heart failure.