Amiloidosis cardiacacaracterización clínica, manejo terapéutico y pronóstico

Abstract

INTRODUCTION AND OBJECTIVES The two most common subtypes of cardiac amyloidosis (CA) are light-chain (AL-CA) and transthyretin (ATTR-CA). The aims of this study are to characterize both entities and analyze their prognoses. METHODS A single-centre retrospective review of CA patients diagnosed between 1998 and 2018 in Complexo Hospitalario Universitario A Coruña, with clinical follow-up until june 2019. Baseline clinical characteristics and test results were collected; survival and adverse clinical outcomes were analyzed. RESULTS There were 105 CA patients: 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years, 24.8% were women. Heart failure (HF) was the most frequent clinical presentation (55.2%) in both groups. The most prevalent electrocardiographic findings were pseudoinfarction pattern (68.5%) and Sokolow-Lyon index <1.5 mV (67.7%), with no differences between CA subtypes. After a median follow-up of 13.7 months (interquartile range 5.8-28.6 months), one, three and five-year survival were 43.3%, 40.4% and 35.4% for AL-CA patients, and 85.1%, 57.3% and 31.4% in ATTR-CA patients (p=0.004). Independent predictors of mortality were AL-CA subtype (Hazard ratio (HR) 3.41, 95% confidence interval (CI95%) 1.45-8.06, p=0.005), previous admission for HF (HR 4.25, CI95% 1.63-11.09, p=0.003) and New York Heart Association (NYHA) class III-IV (HR 2.76, CI 95%, 1.09-7.03, p=0.033). Beta-blocker therapy was associated with longer survival (HR 0.23, CI 95% 0.09-0.59, p=0.002). CONCLUSIONS Differences exist between the clinical presentation of AL-CA and ATTR-CA patients; both diseases, particularly AL-CA, are associated with poor life prognosis.