Afección cardíaca en pacientes con amiloidosis

Abstract

Introduction: Cardiac involvement is the leading cause of morbidity and mortality of amyloidosis, especially due to light-chain (AL ) and transthyretin in both wild and hereditary types . Objectives: To estimate the prevalence and mortality of cardiac involvement due to amyloidosis in patients diagnosed with amyloidosis. Methods: A clinic ambispective cohort, which included all consecutive patients who had evidence of amyloidosis in the Amyloidosis Institutional Registry in the Italian Hospital of Buenos Aires , in the period 01/ 2007 - 03 /2013 . The diagnostic criteria were prespecified to define cardiac amyloidosis . Mortality from cardiac amyloidosis was assessed using a combination of active and passive assessment. Survival time was evaluated using the Kaplan -Meier estimator. SPSS software version 19 was used. Results: The registry included 126 patients and the prevalence of cardiac amyloidosis was 40 % (31-49 % CI). Among the 40 % (50 /126) patients with confirmed and suspected cardiac amyloidosis, the median age was 72 years (IQR 79-81) and 26% were women (13/ 50). The overall mortality of patients with cardiac involvement was 20% (11/ 50), the median survival was 1782 days of follow up (95% CI 1365-2199). 47% of patients with cardiac involvement by amyloidosis was confirmed with biopsy. The main reasons for diagnosis were: clinical suspicion of 82.4 % (42 /50), complementary studies 67 % (34 /50), derived with diagnosis 6% (3/ 50). Conclusions: The prevalence of cardiac disease and mortality in patients with amyloidosis was high. Advances in the diagnosis of the cause of amyloidosis in the future may allow detecting patients at high risk of death from cardiac causes in our country