QJM, Vol 91, Issue 2 141-157, Copyright © 1998 by Oxford University Press
SW Dubrey, K Cha, J Anderson, B Chamarthi, J Reisinger, M Skinner and RH Falk
We reviewed clinical presentation, investigations, therapy, prognosis and
outcome of 232 patients with primary (AL) cardiac amyloidosis. There were
142 men and 90 women. Median age at presentation was 59 years (range
29-85). AL heart disease was unusual both in patients under the age of 40
(3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the
commonest presenting symptoms. Hallmark features of periorbital ecchymoses
and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac
amyloidosis was unusual in isolation (3.9%), and most frequently patients
had features of multiorgan dysfunction; heavy proteinuria and features of
malabsorption predominating in this respect. Heart involvement represents
the worst prognostic indicator, with a median survival from diagnosis of
1.08 years, falling to 0.75 years with the onset of heart failure. Current
therapeutic procedures appear to prolong survival, with left ventricular
wall thickness, mass and ejection fraction on echocardiography and late
potentials on signal averaged electrocardiography of use in prognostic
stratification. Cardiac involvement from AL amyloidosis is rapidly fatal.
It should be suspected in all patients with heart failure who have wall
thickening on echo, normal chamber sizes, low EKG voltages and evidence
suggesting a multisystem disease.
ORIGINAL PAPERS
The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement
Section of Cardiology, Boston University School of Medicine, Massachusetts, USA.
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