The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement

We reviewed clinical presentation, investigations, therapy, prognosis andoutcome of 232 patients with primary (AL) cardiac amyloidosis. There were142 men and 90 women. Median age at presentation was 59 years (range29-85). 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 thecommonest presenting symptoms. Hallmark features of periorbital ecchymosesand macroglossia were present in 12.5% and 27.2%, respectively. AL cardiacamyloidosis was unusual in isolation (3.9%), and most frequently patientshad features of multiorgan dysfunction; heavy proteinuria and features ofmalabsorption predominating in this respect. Heart involvement representsthe worst prognostic indicator, with a median survival from diagnosis of1.08 years, falling to 0.75 years with the onset of heart failure. Currenttherapeutic procedures appear to prolong survival, with left ventricularwall thickness, mass and ejection fraction on echocardiography and latepotentials on signal averaged electrocardiography of use in prognosticstratification. Cardiac involvement from AL amyloidosis is rapidly fatal.It should be suspected in all patients with heart failure who have wallthickening on echo, normal chamber sizes, low EKG voltages and evidencesuggesting a multisystem disease.