Amyloidosis and cardiac involvement

Background. - Amyloidosis is a rare disease characterized by the extracellu lar accumulation of a protein polysaccharid complex: amyloid. Cardiac invol vement may occur with or without clinical manifestations, and 15 considered as a major prognostic factor. Aim of the study. - Firstly, to analyze the clinical, electrocardiographic, radiological and echocardiographic features in a group of patients with ex tracardiac biopsy-proven amyloid infiltration and evidence of echocardiogra phic amyloid heart disease. Secondly, to compare the survival of amyloidosi s patients, with or without cardiac involvement. Patients and methods. -We retrospectively analyzed the main echocardiograph ic features of 47 patients with biopsy proven amyloidosis. No clinical, ele ctrocardiographic, radiological or scintigraphic criterium were selective f or cardiac Involvement. Thirty patients with echographic features of amyloi d heart disease were identified and compared to 17 patients without echogra phic features of amyloid heart disease. Results. -Amyloid disease with heart involvement was AL in 25/30 (83%) pati ents and occurred more commonly in middle age men (mean age: 53+/-11 years) , The main clinical presentation was congestive heart failure (59%), but 37 % of patients had no clinical cardiac features. The electrocardiogram was a bnormal in 86% and the cardiac silhouette was enlarged on chest roentgenogr am in 27% of patients. The main echocardiographic findings were: diffuse ve ntricular wall thickening in 21 patients (70%) and Isolated septal wall thi ckening in 9 patients (30%); restrictive pattern of left ventricular (LV) d iastolic function in 17 patients (57%); pericardial effusion in 12 patients (40%); impaired LV systolic function in 8 patients (27%); atrial enlargeme nt in 8 patients (27%); characteristic granular sparkling of LV myocardium in 8 patients (27%); mitral and/or aortic valve thickening in 4 patients (1 3%). Cardiac symptoms developed in 72% of the non symptomatic patients havi ng echocardiographic evidence of cardiac involvement. Twenty-five patients died during the study period and the death was due to cardiac disease in 76 %. Median survival time was 36 months from time of amyloidosis diagnosis, a nd it was 23 months from time of amyloid myocardiopathy diagnosis. It short ened to 6 months: when congestive heart failure appeared. Conclusion. Patients with a histologically proven amyloidosis should be exa mined by echocardiography, because cardiac involvment is frequently found i n patients with no clinical symptoms, and non symptomatic patients having e chocardiographic evidence of cardiac involvement will almost always develop cardiac symptoms. Survival actuarial study confirms the significant advers e influence of cardiac involvement in amyloidosis.