Holter monitoring in AL amyloidosis: Prognostic implications

The heart is involved in more than one third of patients with primary (AL) amyloidosis at diagnosis and it is by far the most common cause of death. R hythm and conduction abnormalities generally represent the terminal event. The aims of this study were to determine the spectrum of Holter abnormaliti es found in AL amyloidosis and to assess their prognostic significance, par ticularly in relation to sudden death. Fifty-one patients with AL amyloidos is were included, and all of them had a complete history, physical examinat ion, two-dimensional echocardiography, and 24-hour Holter monitoring. Fifty -five percent of these patients had echographic signs of heart involvement and 23% had heart failure. Complex ventricular arrhythmias were found in 57 % of patients, couplets in 29%, and nonsustained ventricular tachycardia in 18%. Overall median survival was 23.4 months. Congestive heart failure, ec hocardiographic abnormalities, and Holter abnormalities adversely affected survival. The multivariate analysis demonstrated that interventricular sept um thickness and couplets were independent predictors of survival. The pres ence of couplets correlated with sudden death. Holter monitoring may contri bute to assessing the prognosis of patients with AL amyloidosis.