Localized dystrophic amyloidosis of heart valves

A survey of 152 surgically resected heart valves (90 aortic, 60 mitral, two pulmonic) from a consecutive series of 142 adult patients revealed congophilic, green birefringent, amyloid deposits in 81 valves (59 aortic, 22 mitral) from 77 patients from 41 to 79 years of age. Amyloid was found in 50 (82 per cent) of 61 valves showing degenerative calcific sclerosis and in 31 (53 per cent) of 58 valves showing post-rheumatic sclerosis but in none of 33 valves showing more acute disease processes (myxoid transformation, "stretch lesions," healed bacterial endocarditis). All the amyloidotic valves showed some calcification, and amyloid deposits frequently occurred around the calcified foci. Histochemically, the valvular amyloid was distinguishable from other major forms of amyloid by its largely negative alcian blue and periodic acid-Schiff staining and by its pepsin-resistant iodine staining. The localized, dystrophic nature and unusual histochemical properties of this valvular amyloidosis clearly distinguish it from other forms of cardiac amyloidosis.