Variant-Sequence Transthyretin (Isoleucine 122) in Late-Onset Cardiac Amyloidosis in Black Americans

Isolated cardiac amyloidosis appearing late in life (senile cardiac amyloidosis) was first described in the 19th and early 20th centuries. 1 , 2 Pathological studies established its greater prevalence with increasing age, but the distinction between atrial and ventricular deposits was usually ignored. 3 – 6 The amyloid was thought to be a coincidental finding of limited importance. Later studies documented the clinical significance of the ventricular deposits in producing congestive heart failure, atrial fibrillation, and death from cardiac causes. 7 , 8 The subsequent detection of small vascular deposits in other tissues prompted the suggestion that this disorder be renamed senile systemic amyloidosis. The deposited . . .