• Gonzalo Barge-Caballero, Raquel Vázquez-García, Eduardo Barge-Caballero, David Couto-Mallón, María J Paniagua-Martín, Roberto Barriales-Villa, Pablo Piñón-Esteban, Alberto Bouzas-Mosquera, Jorge Pombo-Otero, Guillermo Debén-Ariznavarreta, José M Vázquez-Rodríguez, and María G Crespo-Leiro.
• Unidad de Insuficiencia Cardíaca Avanzada y Trasplante Cardiaco, Servicio de Cardiología, Complexo Hospitalario Universitario A Coruña, España; Instituto de Investigación Biomédica de A Coruña (INIBIC), España; Centro de Investigación Biomédica en Red (CIBERCV), Instituto de Salud Carlos III, Madrid, España. Electronic address: gonzalo.barge.caballero@sergas.es.
• Med Clin (Barc). 2021 Apr 23; 156 (8): 369-378.

Introduction And ObjectivesLight-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases.MethodsWe conducted a single-centre, retrospective review of all patients diagnosed with CA between 1998 and 2018. Clinical characteristics, complementary tests, survival and other adverse clinical events were studied.ResultsWe identified 105 patients with CA, 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years; 24.8% were women. In both groups, heart failure was the most frequent clinical presentation (55.2%). The most prevalent electrocardiographic findings were the pseudoinfarct pattern (68.5%) and a Sokolow-Lyon index < 1.5 mV (67.7%), with no differences between the two subtypes of CA. One-year, 3-year, and 5-year survival was 43.3%, 40.4% and 35.4%, respectively, in AC-AL patients, and 85.1%, 57.3% and 31.4% in AC-ATTR patients (p = 0.004). AL-CA subtype (HR 3.41; 95% CI 1.45-8.06; p = 0.005), previous admission for heart failure (HR 4.25; 95% CI 1.63-11.09; p = 0.003) and a NYHA class III-IV (HR 2.76; 95% CI; 1.09-7.03; p = 0.033) were independent predictors of mortality, while beta-blocker therapy was associated with longer survival (HR 0.23; 95% CI 0.09-0.59; p = 0.002).ConclusionsDifferences exist between the clinical presentation of AL-CA and ATTR-CA patients. Both diseases, particularly AL-CA, are associated with poor life prognosis.Copyright © 2020 Elsevier España, S.L.U. All rights reserved.

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