Unique type of isolated cardiac valvular amyloidosis.
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.


METHODS
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.


CONCLUSIONS
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
Shehzad
S
Cardiothoracic Surgery, Heart Hospital, University College of London, London, UK. drshehzadiqbal@yahoo.com
Reehana
Salma
S
Lawrence
David
D
D000368:Aged; D000686:Amyloidosis / Q000473:pathology / Q000601:surgery; D001021:Aortic Valve; D006349:Heart Valve Diseases / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male