Abstract
One hundred thirty-three patients with biopsy-proven AL amyloidosis were studied with echocardiography, Holter recording, 12-lead electrocardiography, and signal-averaged electrocardiograms. Features from these tests were analyzed in relation to their effect on mortality. Late potentials were more frequent in patients with echocardiographic evidence of cardiac amyloidosis (31%) compared with patients with normal echocardiograms (9%, p < 0.003). One hundred six of the 133 patients died during follow-up, of which 34 were nonsudden cardiac deaths and 33 were sudden deaths. Abnormal echocardiograms and signal-averaged electrocardiograms were each predictive of all-cause cardiac death ( p < 0.0001) and sudden cardiac death ( p < 0.0001). Abnormal signal-averaged electrocardiograms were also independently predictive of sudden death in the subgroup of patients with an abnormal echocardiogram ( p < 0.05). Thus late potentials are predictive of sudden death in patients with AL amyloidosis and provide independent prognostic information in patients with echocardiographic evidence of amyloid involvement. (Am Heart J 1997;134:994-1001.)
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