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Resumen Introduccion y objetivos La amiloidosis hereditaria por transtirretina (ATTRv) es una enfermedad causada por mutaciones en el gen de la transtirretina que frecuentemente presenta afeccion cardiaca debido al deposito de amiloide en el miocardio. Nuestro objetivo es describir esta afeccion en una cohorte espanola. Metodos Estudio retrospectivo multicentrico de pacientes con ATTRv y afeccion cardiaca provenientes de centros espanoles. Se recogieron datos demograficos, clinicos y geneticos. Resultados En 26 centros se incluyo a 181 pacientes, el 65,2% varones, con una mediana de edad al diagnostico de 62 anos. Las mutaciones mas frecuentes fueron Val50Met (67,7%) y Val142Ile (12,4%). El principal motivo de consulta fue extracardiaco (69%), principalmente neurologico. La media de la fraccion aminoterminal del propeptido natriuretico cerebral (NT-proBNP) fue 2.145 ± 3.586 pg/ml. Lo mas caracteristico del electrocardiograma fueron el patron de seudoinfarto (25,9%) y el bloqueo auriculoventricular (25,3%). El grosor ventricular medio fue 15,4 ± 4,1 mm. El strain longitudinal estaba reducido en segmentos basales en el 29,4%. Se observo realce tardio subendocardico difuso en el 58,8%. En la gammagrafia habia captacion de grados 2-3 en un 75%. En el seguimiento, el 24,9% ingreso por insuficiencia cardiaca, el 34,3% preciso marcapasos y el 31,6%, trasplante hepatico. El 32,5% fallecio, principalmente por insuficiencia cardiaca (28,8%). Las mutaciones diferentes de Val50Met se asociaron en general con un peor pronostico. Conclusiones La ATTRv cardiaca en Espana tiene un espectro genetico y de afeccion heterogeneo. El pronostico es malo principalmente por las complicaciones cardiacas, por lo que son esenciales un diagnostico y un tratamiento precoces.

Introduction and objectives Hereditary transthyretin amyloidosis (hATTR) is a disease caused by mutations in the transthyretin gene that frequently shows cardiac involvement due to amyloid deposition in the myocardium. Our objective was to identify cardiac involvement in a Spanish cohort. Methods Retrospective multicenter study of patients diagnosed with hATTR with cardiac involvement from Spanish centers. We collected demographic, clinical, and genetic data. Results A total of 181 patients from 26 centers were included (65.2% men, with a median age at diagnosis of 62 years). The most frequent mutations were Val50Met (67.7%) and Val142Ile (12.4%). The main reason for consultation was extracardiac symptoms (69%), mainly neurological. The mean N-terminal pro-B-type natriuretic peptide level was 2145±3586 pg/mL. The most characteristic electrocardiogram findings were a pseudoinfarct pattern (25.9%) and atrioventricular block (25.3%). Mean ventricular thickness was 15.4±4.1mm. Longitudinal strain was reduced in basal segments by 29.4%. Late diffuse subendocardial enhancement was observed in 58.8%. Perugini grade 2 or 3 uptake was observed in 75% of scintigraphy scans. During follow-up, 24.9% of the patients were admitted for heart failure, 34.3% required a pacemaker, and 31.6% required a liver transplant. One third (32.5%) died during follow-up, mainly due to heart failure (28.8%). The presence of non-Val50Met mutations was associated with a worse prognosis. Conclusions HATTR cardiac amyloidosis in Spain shows heterogeneous genetic and clinical involvement. The prognosis is poor, mainly due to cardiac complications. Consequently early diagnosis and treatment are vital.

Aim: To analyze the frequency and variables related to inappropriate rivaroxaban dosage in clinical practice and its impact on outcomes after 2 years. Materials & methods: Postauthorization, observational, multicenter study, in which atrial fibrillation patients, treated with rivaroxaban ≥6 months were included. Results: A total of 1421 patients (74.2 ± 9.7 years, CHA 2 DS 2 -VASc 3.5 ± 1.6) were included. Overall, 22.9% received rivaroxaban 15 mg. The proper dose of rivaroxaban was taken by 83.3% (9.7% underdosed, 7.0% overdosed). Older age and renal insufficiency were associated with inadequate rivaroxaban dosage. There was a trend toward higher all-cause mortality among underdosed patients (adjusted hazard ratio 1.39; 95% CI 0.75–2.58), and more bleedings in overdosed patients (2.29 vs 0.80 events/100 patient-years; p = 0.14). Conclusion: In clinical practice, rivaroxaban is properly dosed in most patients.

Altres ajuts: AstraZeneca Farmacéutica, Spain, S.A. (ESR-17-13244). Hyperkalaemia is a growing concern in the treatment of patients with heart failure and reduced ejection fraction (HFrEF) as it limits the use of some prognostic-modifying drugs and has a negative impact on prognosis. The objective of the present study was to estimate the prevalence of hyperkalaemia in outpatients with HFrEF and its impact on achieving optimal medical treatment. For this purpose, a multicentre, prospective, and observational study was carried out on consecutive HFrEF patients who were monitored as outpatients in heart failure (HF) units and who, in the opinion of their doctor, received optimal medical treatment. A total of 565 HFrEF patients were included from 16 specialised HF units. The mean age was 66 ± 12 years, 78% were male, 45% had an ischemic cause, 39% had atrial fibrillation, 43% were diabetic, 42% had a glomerular filtration rate < 60 mL/min/1.7 m, and the mean left ventricular ejection fraction was 31 ± 7%. Treatment at the study entry included: 76% on diuretics, 13% on ivabradine, 7% on digoxin, 18.9% on angiotensin-conversing enzyme inhibitors (ACEi), 11.3% on angiotensin receptors blockers (ARBs), 63.8% on angiotensin-neprilysin inhibitors (ARNi), 78.5% on mineralocorticoid receptor antagonists (MRAs), and 92.9% on beta-blockers. Potassium levels in the baseline analysis were: ≤5 mEq/L = 80.5%, 5.1-5.4 mEq/L = 13.8%, 5.5-5.9 mEq/L = 4.6%, and ≥6 mEq/L = 1.06%. Hyperkalaemia was the reason for not prescribing or reaching the target dose of an MRAs in 34.8% and 12.5% of patients, respectively. The impact of hyperkalaemia on not prescribing or dropping below the target dose in relation to ACEi, ARBs, and ARNi was significantly less. In conclusion, hyperkalaemia is a frequent problem in the management of patients with HFrEF and a limiting factor in the optimisation of medical treatment.

INTRODUCCION El balance entre el riesgo embolico y hemorragico supone un reto en pacientes oncologicos que requieren anticoagulacion. No hay recomendaciones especificas con respecto al mismo en pacientes con cancer y fibrilacion auricular (FA). Objetivo: comparar el tratamiento antitrombotico utilizado en una poblacion similar con y sin cancer, asi como la incidencia de eventos tromboticos y hemorragicos. METODOS Se trata de un estudio ambispectivo observacional y multicentrico que analiza pacientes con FA no valvular tratados en consultas externas de Oncologia y Cardiologia en el periodo de 2011-2018. Se incluyeron un total de 1237 mujeres con FA: 638 con cancer de mama y 599 sin cancer. El analisis estadistico se realizo con SPSS V.22.0. RESULTADOS El seguimiento medio fue de 3.1 anos. Ambos grupos fueron similares en edad, CHA2DS2-VASc y HASB-LED. Al inicio, recibian tratamiento anticoagulante un 85% de las pacientes con cancer y un 88% de las pacientes sin cancer. En su mayoria, el tratamiento pautado fueron antagonistas de vitamina K (AVK) (65% vs 66%, respectivamente). Los anticoagulantes de accion directa (ACOD) eran la segunda opcion mas frecuente. La presencia de cancer no se asocio con una mayor incidencia de eventos embolicos ni hemorragicos. La utilizacion de ACOD fue menor en el grupo oncologico (15 Vs 19%). CONCLUSIONES Los pacientes con cancer de mama y FA mostraron una incidencia de ictus y embolismo sistemico superior muestra similar con FA y sin cancer, con un riesgo de eventos hemorragicos mayores similar. El uso de ACOD fue menor en el grupo con cancer, y funcionaron sin diferencias en ambos grupos.

Bleeding in ACS patients is an independent marker of adverse outcomes. Its prognostic impact is even worse in elderly population. Current bleeding risk scores include chronological age but do not consider biologic vulnerability. No studies have assessed the effect of frailty on major bleeding. The aim of this study is to determine whether frailty status increases bleeding risk in patients with ACS.This prospective and observational study included patients aged ≥75years admitted due to type 1 myocardial infarction. Exclusion criteria were severe cognitive impairment, impossibility to measure handgrip strength, cardiogenic shock and limited life expectancy due to oncologic diseases. The primary endpoint was 30-day major bleeding defined as a decrease of ≥3g/dl of haemoglobin or need of transfusion.A total of 190 patients were included. Frail patients (72, 37.9%) were older, with higher comorbidity features and with a higher CRUSADE score at admission. On univariate analysis, frailty predicted major bleeding during 30-day follow-up despite less frequent use of a P2Y12 inhibitor (66.2% vs 83.6%, p=0.007) and decreased catheterisation rate (69.4% vs 94.1%, p0.001). Major bleeding was associated with increased all-cause mortality at day 30 (18.2% vs 2.5%, p0.001). On multivariate analysis, frailty was an independent predictor for major bleeding.Frailty phenotype, as a marker of biological vulnerability, is an independent predictor of major bleeding in elderly patients with ACS. Frailty can play an important role in bleeding risk stratification and objective indices should be integrated into routine initial evaluation of these patients.

Cardiac surgery induces geometrical and functional changes, which are not clearly explained. Objective: to investigate the physiopathology of the heart after cardiac surgery using advanced techniques of echocardiography. Thirty patients undergoing cardiac surgery had echocardiographic study prior and after surgery. Left and right ventricular (RV) longitudinal displacement and strain were studied with speckle-tracking. Using longitudinal displacement, we defined a static longitudinal reference-point (sLRP) to which the other segments moved during systole. Transversal displacement and global function were determined by conventional-echo. Left and RV segments showed systolic longitudinal displacement towards the apex, which was the sLRP before surgery; and towards the medium segment of lateral RV-wall one week after surgery. The displacement of basal RV segment towards this sLRP was smaller, causing decreased TAPSE. Apical segments showed an inverse displacement towards the new sLRP, and septum displacement was decreased or inverted towards the lateral RV-wall, causing paradoxus septal motion. RV-wall longitudinal strain was reduced (− 23.1 ± 8.6 vs. − 14.6 ± 5.3;p

Background Swirling flow, organized in vortices, contributes to adequate left ventricular function. In this study, we apply a novel echocardiographic flow-mapping technique, vector flow mapping (VFM), to evaluate the main characteristics of left ventricular vortices and its relation to filling parameters. Methods Forty-eight subjects underwent conventional transthoracic echocardiographic examination with additional intracardiac flow assessment with VFM using a Aloka Alpha-10 system and experimental VFM analysis software. To analyze vortex behavior, its rotation direction, duration, location inside the left ventricle, size, and intensity were assessed in apical long-axis view. Its relation to conventional left ventricular filling parameters was then analyzed. Results Two vortex components were consistently identified following each transmitral filling wave. The anterior component of these visualized vortices was analyzed, due to its higher significance in the cardiac cycle, following early filling (V1) and atrial contraction (V2). Differences were observed in several aspects of vortex behavior between V1 and V2, particularly in patients with normal left ventricular filling parameters. These differences may be related to varying roles of vortices in different periods of the cardiac cycle. Conclusions Vector flow mapping allowed visualization and measurement of several parameters defining vortex behavior inside the cardiac cycle. The differences observed in these parameters between vortices in different phases of the cardiac cycle may be related to their role in optimizing cardiac function.