P310 CARDIAC AMYLOIDOSIS: INITIAL MONOCENTRIC EXPERIENCE

Abstract

Abstract Cardiac amyloidosis is a group of diseases characterized by the accumulation in the interstitial space of amorphous material consisting of amyloid fibrils and, until recently, considered rare, with poor therapeutic prospects and burdened by a poor prognosis. However, both for amyloidosis from immunoglobulin light chains (AL) and for transthyretin amyloidosis in its wild type and mutated forms, there has been a significant increase in diagnostic capabilities. The aim of this work is to show the characteristics found in our population affected by cardiac amyloidosis. Materials and Methods We enrolled 18 patients observed over 12 months. The mean age was 63 ± 5, 14 patients were male. 8 patients in NYHA Class I – II, 10 patients in NYHA Class II – III. On the ECG in 88% of cases low QRS voltages in the peripheral leads. Among the conduction disturbances: 1st degree AV block in 45%, EAS in 33%, BBSn in 17%, occurrence of isolated BEV and BESV in 90%; 1 patient presented with AF. Defibrillator was implanted in 3 patients for detection of TVS and TNnS. In the whole population, the echocardiogram showed concentric parietal hypertrophy with a granular sparkling appearance; the mean FE was 55 ± 3%, the diastolic filling pattern was altered in type I in 42%, type II in 33% and type III in 25% of patients, respectively. In 92% of cases the left atrium was dilated. In 87% of cases there was an involvement of the valves in particular in 64% of cases we found calcifications of the aortic valve and in 66% mitral insufficiency. In 42% of patients there was an increase in the thickness of the atrial septum as well as the free wall of the right ventricle. Finally, signs of non–buffering pericardial effusion were found in 33% of patients. Results In our study population, 15 patients had multiple myeloma and presented with cardiac AL–type amyloidosis; among these 3 also presented renal involvement. In 3 patients, on the other hand, after positive bone scan and genetic screening, we diagnosed cardiac amyloidosis due to transthyretin mutation for which therapy with Tafamidis was undertaken. 3 patients died. Conclusions Our population presented echographic and echocardiographic characteristics comparable to those reported in the literature.