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[Abstract] Sudden cardiac death due to ventricular fibrillation (VF) during ST-elevation acute myocardial infarction (STEAMI) significantly contributes to cardiovascular-related deaths. Although VF has been linked to genetic factors, variations in copy number variation (CNV), a significant source of genetic variation, have remained largely unexplored in this context. To address this knowledge gap, this study performed whole exome sequencing analysis on a cohort of 39 patients with STEAMI who experienced VF, aiming to elucidate the role of CNVs in this pathology. The analysis revealed CNVs in the form of duplications in the PARP2 and TTC5 genes as well as CNVs in the form of deletions in the MUC15 and PPP6R1 genes, which could potentially serve as risk indicators for VF during STEAMI. The analysis also underscores notable CNVs with an average gene copy number equal to or greater than four in DEFB134, FCGR2C, GREM1, PARM1, SCG5, and UNC79 genes. These findings provide further insight into the role of CNVs in VF in the context of STEAMI.

[Resumo] Esta tese doutoral é un estudo observacional realizado en doentes con infarto agudo de miocardio con elevación do segmento ST (IAMCEST), tratados mediante intervencionismo coronario percutáneo, co obxectivo de analizar a presenza e tipo de bacterias, mediante a detección de Ácido Desoxirribonucleico (ADN) nos trombos aspirados das arterias coronarias. Non se estudou a presenza, prevalencia e variedade de organismos bacterianos en pacientes galegos. Incluíronse 109 doentes consecutivos nos que se obtivo material trombótico e mostra de sangue periférica; analizouse a presenza do ADN bacteriano coa Reacción en Cadena de la Polimerasa (PCR) en tempo real de: Aggregatibacter actinomycetemcomitans, Chlamydia pneumoniae, viridans group streptococci, Porphyromonas gingivalis, Fusobacterium nucleatum, Tannarella forsythia, Treponema denticola, Helycobacter pylori, Mycoplasma pneumoniae, Staphylococus aureus, Prevotella intermedia, e Streptococcus mutans. Detectouse ADN bacteriano de 4 especies no 9,2% dos doentes: viridans group streptococci, (6 doentes, 5,5%), Staphylococus aureus (2 doentes, 1,8%); Porphyromonas gingivalis e Prevotella intermedia (0,9% respectivamente). Non se atopou ADN bacteriano na sangue periférica nestes doentes. En conclusión, hai ADN de 4 especies bacterianas nos trombos dunha pequena proporción de pacientes con IAMCEST. Estas bacterias poderían ter un rol na inestabilidade da placa e na formación do trombo que conduce ó IAMCEST nestes pacientes., [Resumen] Esta tesis doctoral es un estudio observacional en pacientes con infarto agudo de miocardio con elevación del segmento ST (IAMCEST), con el objetivo de analizar la presencia de bacterias mediante la detección de Ácido Desoxirribonucleico (ADN) bacteriano en trombos de pacientes con IAMCEST. La presencia, prevalencia y variedad de bacterias en pacientes gallegos no ha sido estudiada. Incluímos 109 pacientes consecutivos, obteniendo trombo coronario por tromboaspiración, y una muestra de sangre periférica. Se analizó mediante la Reacción en Cadena de la Polimerasa en tiempo real (q-PCR) la presencia de ADN bacteriano de: Aggregatibacter actinomycetemcomitans, Chlamydia pneumoniae, viridans group streptococci, Porphyromonas gingivalis, Fusobacterium nucleatum, Tannarella forsythia Treponema denticola, Helycobacter pylori, Mycoplasma pneumoniae, Staphylococus aureus, Prevotella intermedia, y Streptococcus mutans. Se detectó presencia de 4 especies en el 9,2% de los pacientes: viridans group streptococci, (5,5%), seguido de Staphylococus aureus (1,8%); Porphyromonas gingivalis y Prevotella intermedia (0,9% respectivamente). No se detectó ADN bacteriano en sangre periférica. En conclusión, hay ADN de 4 especies bacterianas en los trombos de una pequeña proporción de pacientes con IAMCEST. Estas bacterias podrían tener un rol en la inestabilidad de la placa y en la formación del trombo que conduce a un IAMCEST en estos pacientes., [Abstract] This doctoral thesis is an observational study in patients with ST-segment elevation myocardial infarction (STEMI) in our geographical area treated with coronary intervention. The presence, prevalence, and variety of bacteria in thrombi have never been studied in Galician patients. The objective was to detect bacterial deoxyribonucleic acid (DNA) in thrombotic material from patients with STEMI. We included 109 consecutive patients in whom coronary thrombus was obtained by thromboaspiration, and one peripheral blood sample. The presence of bacterial DNA was analysed by probe-based real-time Polymerase Chain Reaction (PCR). The presence of: Aggregatibacter actinomycetemcomitans, Chlamydia pneumoniae, viridans group streptococci, Porphyromonas gingivalis, Fusobacterium nucleatum, Tannarella forsythia, Treponema denticola, Helycobacter pylori, Mycoplasma pneumoniae, Staphylococus aureus, Prevotella intermedia, and Streptococcus mutans was studied. Bacterial DNA from 4 species was detected in 9,2% of the patients studied: viridans group streptococci, (5.5%), followed by Staphylococcus aureus (1.8%); Porphyromonas gingivalis and Prevotella intermedia (0.9% respectively). No bacterial DNA was detected in peripheral blood. In conclusion, DNA from 4 bacterial species is present in thrombi from a small proportion of STEMI patients. Bacteria could be latently present in plaques and might play a role in plaque instability and thrombus formation leading to STEMI in these patients.

Introduction and objectives: Transcatheter aortic valve implantation (TAVI) was first introduced in 2007 as an alternative to open heart surgery to treat patients with severe symptomatic aortic stenosis (sSAS) with various indication expansions since that date. Recently, the PARTNER 3 study (Placement of aortic transcatheter valve) demonstrated clinical benefits with TAVI with the SAPIEN 3 valve vs surgical aortic valve replacement (SAVR) in selected low surgical mortality risk patients. We reviewed data from the PARTNER 3 and economic data from Spain to assess the cost-effectiveness ratio of TAVI vs SAVR in patients with sSAS and low surgical mortality risk. Methods: A 2-stage model was used to estimate direct healthcare costs and health-related quality of life data regarding TAVI with the SAPIEN 3 valve and SAVR. Early adverse events associated with TAVI from the PARTNER 3 were fed into a Markov model that captured longer-term outcomes after TAVI or SAVR. Results: TAVI with SAPIEN 3 improved quality-adjusted life years per patient (+ 1.00) with an increase in costs vs SAVR (€6971 per patient). This meant an incremental cost-effectiveness ratio/quality-adjusted life year of €6952 per patient. The results were robust with TAVI with the SAPIEN 3 valve remaining cost-effective across several sensitivity analyses. Conclusions: TAVI with the SAPIEN 3 valve is cost effective compared to SAVR in patients with sSAS and low surgical mortality risk. These findings can inform policymakers to facilitate policy development in Spain on intervention selection in this patient population., Introducción y objetivos: El implante percutáneo de válvula aórtica (TAVI) se introdujo en 2007 como una alternativa a la cirugía a corazón abierto para tratar a pacientes con estenosis aórtica grave sintomática, y desde entonces han aumentado las indicaciones autorizadas. Recientemente, el Placement of Aortic Transcatheter Valve Study (PARTNER) 3 ha demostrado beneficios clínicos con el TAVI con la válvula SAPIEN 3 frente al reemplazo quirúrgico de válvula aórtica (RVAo) en pacientes seleccionados con bajo riesgo de mortalidad quirúrgica. Utilizando los datos del PARTNER 3 junto con datos económicos de España, se evaluó la relación coste-efectividad del TAVI en comparación con el RVAo en pacientes con estenosis aórtica grave sintomática con bajo riesgo de mortalidad quirúrgica. Métodos: Se utilizó un modelo en dos etapas para estimar los costes directos sanitarios y los datos de calidad de vida relacionadas con la salud para TAVI con la válvula SAPIEN 3 y RVAo. Los eventos adversos tempranos relacionados con TAVI del PARTNER 3 se incluyeron en un modelo de Markov, que capturó los resultados a más largo plazo tras TAVI o RVAo. Resultados: El TAVI con SAPIEN 3 mejoró los años de vida ajustados por calidad por paciente (+1,00), con un aumento en el coste frente al RVAo de 6.971 € por paciente. Esto representó una ratio coste-efectividad incremental por año de vida ganado ajustado por calidad de 6.952 € por paciente. Los resultados fueron robustos en los diversos análisis de sensibilidad realizados, en los que el TAVI con SAPIEN 3 se mantiene como una opción coste-efectiva. Conclusiones: El TAVI con SAPIEN 3 es coste-efectivo en comparación con el RVAo en pacientes con estenosis aórtica grave sintomática con bajo riesgo de mortalidad quirúrgica. Estos resultados pueden informar a los decisores políticos en España para facilitar el desarrollo de políticas sobre la selección de opciones terapéuticas en esta población de pacientes.

[Resumen] Introducción y objetivos: Existe poca evidencia acerca del impacto que las redes de atención al Síndrome Coronario Agudo Con Elevación del segmento ST (SCACEST), tienen sobre la población. El objetivo de este estudio fue averiguar si el Programa Gallego de Atención al Infarto Agudo de Miocardio (PROGALIAM) en la zona norte de Galicia, mejoró el pronóstico y consiguió la equidad no solo en términos de acceso a las mejores estrategias de reperfusión sino también en términos de supervivencia. Métodos: Se recogieron todos los eventos codificados en el Conjunto Mínimo Básico de Datos (CMBD), como SCACEST entre 2001 y 2013 en los hospitales del área norte del PROGALIAM. Se identificaron 6.783 pacientes, que fueron divididos en dos grupos en función del periodo en el que sufrieron el evento: pre-PROGALIAM (2001-2005); 2.878 pacientes y PROGALIAM (2006-2013); 3.905 pacientes. Resultados: En la etapa Pre-PROGALIAM, la mortalidad ajustada a 30 días, 12 meses y 5 años fue superior en la población global HR: 1,52 - 95% IC (1,31 - 1,77), p < 0,001; HR: 1,48 - 95% IC (1,31 - 1,69), p < 0,001; HR: 1,22 - 95% IC (1,14 - 1,29), p < 0,001, respectivamente. También se observó un incremento significativo en la mortalidad ajustada a 30 días, 12 meses y 5 años en cada una de las áreas antes de la implantación de la red. Las cifras para el área de A Coruña fueron HR: 1,27 - 95% IC (1,01 - 1,60), p = 0,0045; HR: 1,30 - 95% IC (1,07 - 1,58), p = 0,009; HR: 1,12 - 95% IC (1,02 - 1,23), p = 0,02, respectivamente. Para el área de Lugo: HR: 1,94 - 95% IC (1,49 - 2,51), p < 0,001; 1,66 - 95% IC (1,34 - 2,07), p < 0,001; 1,34 - 95% IC (1,2 - 1,49), p < 0,001, respectivamente. Y para el área de Ferrol: HR: 1,58 - 95% IC (1,1 - 2,24), p = 0,001); HR: 1,64 - 95% IC (1,23 - 2,19), p = 0,001); HR: 1,23 - 95% IC (1,10 - 1,40), p = 0,001), respectivamente. Antes de la implantación del PROGALIAM, las mortalidades a 30 días, 12 meses y 5 años era superiores en las áreas de Lugo: HR: 1,57 - 95% I, [Resumo] Introdución e obxectivos: Existe moi pouca evidencia sobre o impacto que teñen na poboación as redes asistenciais de Síndrome Coronaria Aguda con elevación do segmento ST (SCACEST). O obxectivo deste estudo foi coñecer se o Programa Galego de Atención ao Infarto Agudo de Miocardio (PROGALIAM) na zona norte de Galicia mellorou o prognóstico e conseguiu equidade non só no acceso ás mellores estratexias de reperfusión senón tamén en materia de supervivencia. Métodos: Recolléronse todos os eventos codificados no Conxunto Mínimo de Datos Básicos (CMBD), como SCACEST entre 2001 e 2013 nos hospitais da zona norte de PROGALIAM. Un total de 6.783 pacientes foron identificados e divididos en dous grupos en función do período no que sufriron o evento: pre-PROGALIAM (2001-2005); 2.878 pacientes e PROGALIAM (2006-2013); 3.905 pacientes. Resultados: Na etapa Pre-PROGALIAM, a mortalidade axustada aos 30 días, 12 meses e 5 anos foi maior na poboación global HR: 1,52 - IC 95% (1,31 - 1,77), p < 0,001; HR: 1,48 - IC 95 % (1,31 - 1,69), p < 0,001; HR: 1,22 - IC 95 % (1,14 - 1,29), p < 0,001 respectivamente. Tamén se observou un importante incremento da mortalidade axustada a 30 días, 12 meses e 5 anos en cada unha das zonas antes da implantación da rede. As cifras da área de ACoruña foron HR: 1,27 - IC 95% (1,01 - 1,60), p = 0,0045; HR: 1,30 - IC 95 % (1,07 - 1,58), p = 0,009; HR: 1,12 - 95% IC (1,02 - 1,23), p = 0,02, respectivamente. Para a área de Lugo: HR: 1,94 - IC 95% (1,49 - 2,51), p < 0,001; 1,66 - IC 95 % (1,34 - 2,07), p < 0,001; IC 1,34 - 95 % (1,2 - 1,49), p < 0,001, respectivamente. E para a área de Ferrol: HR: 1,58 - IC 95% (1,1 - 2,24), p = 0,001); HR: 1,64 - IC 95% (1,23 - 2,19), p = 0,001); HR: 1,23 - IC 95% (1,10 - 1,40), p = 0,001), respectivamente. Antes da implantación do PROGALIAM, a mortalidade aos 30 días, 12 meses e 5 anos era maior nas áreas de Lugo: HR: 1,57 - IC 95% (1,26 - 1,96), p = 0,001; HR: 1,39 - IC 95 % (1,40 - 1,68), p = 0,001; HR: 1,25 - I, [Abstract] Introduction and objectives: Very little is known about the impact that ST-segment Elevation Acute Myocardial Infarction (STEMI) care networks have on the population. The objective of this study was to find out if the Galician Acute Myocardial Infarction Care Program (PROGALIAM) in the northern area of Galicia, improved prognosis and achieved equity not only in terms of access to the best reperfusion strategies but also in terms of survival. Methods: All the events coded in the Minimum Basic Data Set (MBDS) as STEMI between 2001 and 2013 in the hospitals of the northern area of PROGALIAM were collected. A total of 6,783 patients were identified and divided into two groups based on the period in which they suffered the event: pre-PROGALIAM (2001-2005); 2,878 patients and PROGALIAM (2006-2013); 3,905 patients. Results: In the Pre-PROGALIAM stage, adjusted mortality at 30 days, 12 months and 5 years was higher in the global population HR: 1.52 - 95% CI (1.31 - 1.77), p < 0.001; HR: 1.48 - 95% CI (1.31 - 1.69), p < 0.001; HR: 1.22 - 95% CI (1.14 - 1.29), p < 0.001, respectively. A significant increase in mortality adjusted to 30 days, 12 months and 5 years was also observed in each of the areas before the implementation of the network. The figures for the A Coruña area were HR: 1.27 - 95% CI (1.01 - 1.60), p = 0.0045; HR: 1.30 - 95% CI (1.07 - 1.58), p = 0.009; HR: 1.12 - 95% CI (1.02 - 1.23), p = 0.02, respectively. For the Lugo area: HR: 1.94 - 95% CI (1.49 - 2.51), p < 0.001; 1.66 - 95% CI (1.34 - 2.07), p < 0.001; 1.34 - 95% CI (1.2 - 1.49), p < 0.001, respectively. And for the Ferrol area: HR: 1.58 - 95% CI (1.1 - 2.24), p = 0.001); HR: 1.64 - 95% CI (1.23 - 2.19), p = 0.001); HR: 1.23 - 95% CI (1.10 - 1.40), p = 0.001), respectively. Before the implementation of PROGALIAM, mortality at 30 days, 12 months and 5 years was higher in the Lugo areas: HR: 1.57 - 95% CI (1.26 - 1.96), p = 0.001; HR: 1.39 - 95% CI (1.40 - 1.68), p = 0.001; HR: 1.25 - 95% CI (1.

Introduction. A frequent cause of hospitalization in patients with heart failure is congestion refractory to oral diuretics and is treated with intravenous furosemide. Subcutaneous furosemide administration may be an alternative. Objective: To analyse our initial experience with the use of subcutaneous furosemide administration by elastomeric infuser for 7 days. Material and methods. Retrospective single-centre study. Inclusion criteria: 1) Refractory congestion without low output; and 2) informed consent. The indication is made by the cardiologist. Fixed dose subcutaneous furosemide (120 mg/day) was administered; the infuser was prepared by hospital pharmacy and the implantation, follow-up and specific education was carried out by the nurse. The patient controls daily diuresis, as well as routine self-control of HF. Every 48 hours, analytical and telematic control was performed. The quantitative variables were analysed using the Wilcoxon test. Results. Since April 2020, 20 treatments were performed in 15 patients. Three patients required >1 treatment. Median treatment time was: 7 days (IQR 5-13). There was a significant weight reduction (median weight loss 2.9 kg, IQR 0.45–4.45, p=0.002), without renal deterioration (basal median Cr 1.46 vs. final Cr 1.58 mg/dL; p=0.233) or hypokalaemia (basal median K+ 4.3 vs. final K+ 4 mEq/L; p=0.640). After a median follow-up of 171 days, 73% of patients remained free of admission, three patients died, one was transplanted, and two had mild adverse effects. Conclusion. In this preliminary experience, subcutaneous furosemide administration by elastomeric infuser for 7 days achieved clinical and weight improvement in advanced heart failure patients with refractory congestion, safely avoiding hospitalizations in >70% of episodes., Introducción. Una causa frecuente de hospitalización en pacientes con insuficiencia cardiaca es congestión refractaria a diuréticos orales y se trata con furosemida intravenosa. La administración de furosemida subcutánea puede ser una alternativa. Objetivo: analizar nuestra experiencia inicial con administración de furosemida subcutánea mediante infusor elastomérico de 7 días. Material y métodos. Estudio retrospectivo unicéntrico. Criterios inclusión: 1) congestión refractaria sin bajo gasto, 2) consentimiento informado. La indicación la realiza el cardiólogo. Dosis fija furosemida subcutánea: 120 mg/día, el infusor lo prepara el servicio de Farmacia y el implante, seguimiento y educación específica lo realiza la enfermera. El paciente controla diariamente diuresis y autocontrol habitual de insuficiencia cardiaca. Cada 48h, control analítico y telemático. Las variables cuantitativas fueron analizadas mediante test de Wilcoxon. Resultados. Desde abril de 2020 se realizaron 20 tratamientos en 15 pacientes. Tres pacientes, requirieron > 1 tratamiento. Tiempo mediano de tratamiento: 7 días (RIC 5-13). Hubo descenso significativo del peso (descenso mediano 2,9 Kg, RIC 0,45-4,45, p=0,002), sin deterioro renal (creatinina mediana inicial 1,46 vs creatinina final 1,58 mg/dL, p=0,233) ni hipopotasemia (potasio inicial mediano 4,3 vs potasio final 4 mEq/L, p=0,640). Tras seguimiento mediano de 171 días, el 73% se mantuvo libre de ingreso, tres pacientes fallecieron, uno fue trasplantado y dos tuvieron efectos adversos leves. Conclusión. En esta experiencia preliminar, furosemida subcutánea mediante infusor elastomérico de 7 días consigue mejoría clínica y de peso en situaciones de insuficiencia cardiaca avanzada con congestión refractaria, evitando hospitalizaciones en > del 70% de los episodios de un modo seguro.

Background: The aim of this study was to analyze the impact of the presence of heart failure (HF) on the clinical profile and outcomes in patients with atrial fibrillation (AF) anticoagulated with rivaroxaban. Methods: Observational and non-interventional study that included AF adults recruited from 79 Spanish centers, anticoagulated with rivaroxaban ≥ 6 months before inclusion. Data were analyzed according to baseline HF status. Results: Out of 1,433 patients, 326 (22.7%) had HF at baseline. Compared to patients without HF, HF patients were older (75.3 ± 9.9 vs. 73.8 ± 9.6 years; p = 0.01), had more diabetes (36.5% vs. 24.3%; p < 0.01), coronary artery disease (28.2% vs. 12.9%; p < 0.01), renal insufficiency (31.7% vs. 22.6%; p = 0.01), higher CHA2DS2-VASc (4.5 ± 1.6 vs. 3.2 ± 1.4; p < 0.01) and HAS-BLED (1.8 ± 1.1 vs. 1.5 ± 1.0; p < 0.01). After a median follow-up of 2.5 years, among HF patients, annual rates of stroke/ /systemic embolism/transient ischemic attack, major adverse cardiovascular events (MACE) (non-fatal myocardial infarction, revascularization and cardiovascular death), cardiovascular death, and major bleeding were 1.2%, 3.0%, 2.0%, and 1.4%, respectively. Compared to those patients without HF, HF patients had greater annual rates of MACE (3.0% vs. 0.5%; p < 0.01) and cardiovascular death (2.0% vs. 0.2%; p < 0.01), without significant differences regarding other outcomes, including thromboembolic or bleeding events. Previous HF was an independent predictor of MACE (odds ratio 3.4; 95% confidence interval 1.6–7.3; p = 0.002) but not for thromboembolic events or major bleeding. Conclusions: Among AF patients anticoagulated with rivaroxaban, HF patients had a worse clinical profile and a higher MACE risk and cardiovascular mortality. HF was independently associated with the development of MACE, but not with thromboembolic events or major bleeding. (Cardiol J 2022; 29, 6: 936–947).

[Abstract] Acute myocardial infarction (AMI) is a pandemic in which conventional risk factors are inadequate to detect who is at risk early in the asymptomatic stage. Although gene variants in genes related to cholesterol, which may increase the risk of AMI, have been identified, no studies have systematically screened the genes involved in this pathway. In this study, we included 105 patients diagnosed with AMI with an elevation of the ST segment (STEMI) and treated with primary percutaneous coronary intervention (PPCI). Using next-generation sequencing, we examined the presence of rare variants in 40 genes proposed to be involved in lipid metabolism and we found that 60% of AMI patients had a rare variant in the genes involved in the cholesterol pathway. Our data show the importance of considering the wide scope of the cholesterol pathway in order to assess the genetic risk related to AMI.

[Abstract] Aims. In the absence of previous direct comparative studies, we aimed to evaluate the effectiveness of spironolactone and eplerenone in patients with heart failure and reduced ejection fraction (HFrEF) in a real-world clinical setting. Methods. Using Fine-Gray´s competing risk regression, we compared the clinical outcomes of 293 patients with chronic HF and left ventricular ejection fraction <40% treated with eplerenone and 293 propensity-score matched individuals treated with spironolactone. Study subjects were selected from a prospective cohort of 1404 ambulatory patients with HFrEF seen since 2010 to 2019 in a single specialized HF clinic, among which 992 received a mineralocorticoid receptor antagonist at baseline. Median follow-up was 3.95 years. Results. No statistically significant differences between patients treated with eplerenone versus spironolactone were observed with regard to the risk of the primary composite end-point cardiovascular death or HF hospitalization (HR 0.95; 95% CI 0.73–1.23; p= 0.677). However, eplerenone use was associated to lower cardiovascular mortality (HR 0.55; 95% CI 0.35–0.85; p= 0.008) and lower all-cause mortality (HR 0.67; 95% CI 0.47–0.95; p= 0.027). The incidence of drug suspension due to side effects (HR 0.58, 95% CI 0.40–0.85; p= 0.005) and drug suspension due to any reason (HR 0.70, 95% CI 0.51–0.97; p= 0.033) were lower among patients treated with eplerenone. Conclusions. In this observational, real-world, propensity-score matched study of patients with HFrEF, eplerenone was associated to lower cardiovascular mortality and lower all-cause mortality than spironolactone.

[Abstract] Introduction and objectives. Light-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. Methods. We 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. Results. We 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 = .004). AL-CA subtype (HR 3.41, CI95% 1.45−8.06, p = .005), previous admission for heart failure (HR 4.25, 95% CI 1.63−11.09, p = .003) and a NYHA class III-IV (HR 2.76, 95% CI 1.09−7.03, p = .033) were independent predictors of mortality, while beta-blocker therapy was associated with longer survival (HR .23, 95% CI .09–.59, p = .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., [Resumen] Antecedentes y objetivos. La amiloidosis cardiaca (AC) por cadenas ligeras (AC-AL) y por transtirretina (AC-ATTR) son los dos subtipos más frecuentes de la enfermedad. Nos propusimos caracterizar clínicamente estas entidades y analizar su pronóstico. Material y métodos. Realizamos una revisión retrospectiva de todos los pacientes diagnosticados de AC entre 1998 y 2018 en un centro español. Además de recoger las características clínicas y los resultados de las pruebas complementarias al diagnóstico, analizamos la supervivencia y la incidencia de desenlaces clínicos adversos. Resultados. Identificamos 105 pacientes con AC, 65 AC-ATTR y 40 AC-AL. La edad media era de 74,4 años; el 24,8% eran mujeres. En ambos grupos la insuficiencia cardiaca (IC) fue la forma de presentación clínica más frecuente (55,2%). Los hallazgos electrocardiográficos más prevalentes fueron el patrón de pseudoinfarto (68,5%) y un índice de Sokolow-Lyon <1,5 mV (67,7%), sin diferencias entre los dos subtipos. La supervivencia a 1, 3 y 5 años fue del 43,3%, 40,4% y 35,4%, respectivamente, en pacientes con AC-AL y de 85,1%, 57,3% y 31,4% en pacientes con AC-ATTR (p = 0,004). El subtipo AC-AL (HR 3,41, IC95% 1,45-8,06, p = 0,005), el ingreso previo por IC (HR 4,25, IC95% 1,63-11,09, p = 0,003) y una clase NYHA III-IV (HR 2,76, IC95% 1,09-7,03, p = 0,033) fueron predictores independientes de mortalidad, mientras que el tratamiento betabloqueante se asoció con una mayor supervivencia (HR 0,23, IC95% 0,09-0,59, p = 0,002). Conclusiones. Existen ciertas diferencias en la presentación clínica de los pacientes con AC-AL y AC-ATTR. Ambas entidades, y muy especialmente la AC-AL, presentan un pobre pronóstico vital.