Incidence, Diagnosis and Prognosis of Cardiac Amyloidosis

Abstract

Introduction: Cardiac involvement is frequent in systemic amyloidosis and is the most important determinant of clinical outcome. The aims of this study were to assess the incidence and prognosis of cardiac amyloidosis and discuss diagnostic issues related to cardiac amyloidosis. Methods: We retrospectively studied all patients diagnosed with systemic amyloidosis who presented to our institution from January 1999 to December 2011. Results: Of the 129 patients with systemic amyloidosis,cardiac amyloidosis was diagnosed in 62 patients. At a mean of 3 years follow-up of the patients with systemic amyloidosis, there was statisticallysignificant difference in mortality between patients with cardiac amyloidosis and the rest of the patients (38.3% vs. 14.2%, log rank p = 0.014). And decreased LV function was theonly factor independently associated withsurvivalin cardiac amyloidosis patients in both univariate and multivariate analysis(HR3.936, 95% CI1.247-12.425, p = 0.020). In the diagnosis of monoclonal gammopathy, serum or urine PEPis not sensitive enough to be used clinically compared to serum FLC assay (35.8%vs. 96.4%). Conclusions: In systemic amyloidosis, cardiac involvement is the most important determinant ofprognosis and decreased LV function was independently associated withsurvivalin cardiac amyloidosis patients.