A 72-year-old man presented with a unique case of isolated aortic valve amyloidosis that lacked association with scar tissue or calcific deposits, unlike previously described dystrophic types.
Case Report (n=1)
No
This report describes a unique case of isolated AL valvular amyloidosis on a bicuspid aortic valve without associated calcification or scar tissue, suggesting a potentially new variant of the disease.
BACKGROUND: Amyloid deposition in heart is a common occurrence in systemic amyloidosis. But localised valvular amyloid deposits are very uncommon. It was only in 1922 that the cases of valvular amyloidosis were reported. Then in 1980, Goffin et al reported another type of valvular amyloidosis, which he called the dystrophic valvular amyloidosis. We report a case of aortic valve amyloidosis which is different from the yet described valvular amyloidosis. CASE PRESENTATION: A 72 years old gentleman underwent urgent aortic valve replacement. Intraoperatively, a lesion was found attached to the inferior surface of his bicuspid aortic valve.Histopathology examination of the valve revealed that the lesion contained amyloid deposits, identified as AL amyloidosis. The serum amyloid A protein (SAP) scan was normal and showed no evidence of systemic amyloidosis. The ECG and echocardiogram were not consistent with cardiac amyloidosis. CONCLUSION: Two major types of cardiac amyloidosis have been described in literature: primary-myelomatous type (occurs with systemic amyolidosis), and senile type(s). Recently, a localised cardiac dystrophic valvular amyloidosis has been described. In all previously reported cases, there was a strong association of localised valvular amyloidosis with calcific deposits.Ours is a unique case which differs from the previously reported cases of localised valvular amyloidosis. In this case, the lesion was not associated with any scar tissue. Also there was no calcific deposit found. This may well be a yet unknown type of isolated valvular amyloidosis.
Iqbal et al. (Wed,) conducted a case report in Isolated cardiac valvular amyloidosis (n=1). Aortic valve replacement was evaluated. A 72-year-old man presented with a unique case of isolated aortic valve amyloidosis that lacked association with scar tissue or calcific deposits, unlike previously described dystrophic types.