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Pulmonary vascular remodeling is common among patients with advanced heart failure. Right heart catheterization is the gold standard to assess pulmonary hypertension, but is limited by indirect measurement assumptions, a steady-flow view, load-dependency, and interpretation variability. We aimed to assess pulmonary vascular remodeling with intravascular optical coherence tomography (OCT) and to study its correlation with hemodynamic data.This observational, prospective, multicenter study recruited 100 patients with advanced heart failure referred for heart transplant evaluation. All patients underwent right heart catheterization together with OCT evaluation of a subsegmentary pulmonary artery.OCT could be performed and properly analyzed in 90 patients. Median age was 57.50 [interquartile range, 48.75-63.25] years and 71 (78.88%) were men. The most frequent underlying heart condition was nonischemic dilated cardiomyopathy (33 patients [36.66%]). Vascular wall thickness significantly correlated with mean pulmonary artery pressure, pulmonary vascular resistance, and transpulmonary gradient (R coefficient=0.42, 0.27 and 0.32 respectively). Noninvasive estimation of pulmonary artery systolic pressure, acceleration time, and right ventricle-pulmonary artery coupling also correlated with wall thickness (R coefficient of 0.42, 0.27 and 0.49, respectively). Patients with a wall thickness over 0.25mm had significantly higher mean pulmonary pressures (37.00 vs 25.00mmHg; P=.004) and pulmonary vascular resistance (3.44 vs 2.08 WU; P=.017).Direct morphological assessment of pulmonary vascular remodeling with OCT is feasible and is significantly associated with classic hemodynamic parameters. This weak association suggests that structural remodeling does not fully explain pulmonary hypertension.

[Abstract] Introduction and objectives: This report updates the annual data of the Spanish Heart Transplant Registry with the procedures performed in 2021. Methods: We describe the clinical profile, therapeutic characteristics and outcomes in terms of survival of the procedures performed in 2021. Their temporal trends are updated for the 2012 to 2020 period. Results: In 2021, 302 heart transplants were performed (8.6% increase versus 2020). The tendency in 2021 confirmed that of prior years, with fewer urgent transplants and a preference for the use of ventricular assist devices. The remaining characteristics and survival showed a clear trend toward stability in the last decade. Compared with 2019, the SARS-CoV-2 pandemic (2020 and 2021) did not affect short- or long-term survival. Conclusions: In 2021, transplant activity returned to prepandemic levels. The SARS-CoV-2 pandemic did not significantly affect transplant outcomes. The main transplant features and outcomes have clearly stabilized in the last decade. [Resumen] Introducción y objetivos. Se actualizan los datos anuales de Registro Español de Trasplante Cardiaco con los hallazgos de los procedimientos realizados en 2021. Métodos. Se describen las principales características clínicas, del tratamiento recibido y de los resultados en términos de supervivencia del año 2021 y las tendencias en el periodo 2012-2020. Resultados. En 2021 se han realizado 302 trasplantes cardiacos (un 8,6% más que el año anterior). En 2021 se ha confirmado la tendencia observada en años anteriores a una disminución de los trasplantes urgentes y a la realización de estos mayoritamente con dispositivos de asistencia ventricular. Las demás características y los resultados en términos de supervivencia muestran una clara tendencia a la estabilización en la última década. Respecto a 2019, en los años de la pandemia por SARS-CoV-2 (2020 y 2021) no se detecta un impacto relevante en los resultados en la fase aguda tras el trasplante y en la serie histórica. Conclusiones. En 2021 se ha recuperado la actividad de trasplante hasta cifras previas a la pandemia por SARS-CoV-2, que no ha tenido un impacto global significativo en los resultados. Las características del procedimiento y los resultados muestran una clara tendencia a la estabilización en la última década.

Monoclonal gammopathies (MGs) are a wide range of diseases that may evolve or progress over time. Comorbidity plays a critical role in this setting. The co-occurrence of two MGs is not a rare event. The evidence on the association of systemic light chain (AL) amyloidosis and multiple myeloma (MM) is scarce and controversial. Herein we aim to address this topic in a large series of patients of a referral center. All consecutive AL amyloidosis patients treated at our center from January 2005 to April 2023 were prospectively enrolled in a clinical and epidemiological registry. 141 patients diagnosed with AL amyloidosis were included, of which 7 (5%) had localized whereas 134 presented with systemic disease. The heart was the most frequently affected organ (90.3%). 25 patients (18.7%) fulfilled the IMWG diagnostic criteria of MM (AL/MM). Time-dependent association between AL and MM showed that the synchronous pattern is more frequent than the appearance of a second primary malignancy. The diagnostic delay was six months (m). Patients with AL/MM had a poorer median overall survival (OS) than AL-only patients (35.5 m, CI 95% 0–88.9, vs. 52.6 m, CI 95% 16.7–88.5), but this difference was not statistically significant. The prognosis in AL is dominated by the heart involvement, which is massive in this series. In our Cox regression model, only three prognostic variables remain as independent prognostic factors: age, N-terminal pro-brain natriuretic peptide (≥8500 ng/L), and undergoing an autologous stem cell transplant, whereas left ventricular ejection fraction shows a marginal effect. More and large studies focusing on the AL/MM association are needed to uncover the characteristics and prognostic impact of this association.

In this observational and multicenter study, that included all patients who underwent a heart transplantation (HT) in Spain from 1984 to 2018, we analyzed the incidence, management, and prognosis of colorectal cancer (CRC) after HT. Of 6,244 patients with a HT and a median follow-up of 8.8 years since the procedure, 116 CRC cases (11.5% of noncutaneous solid cancers other than lymphoma registered) were diagnosed, mainly adenocarcinomas, after a mean of 9.3 years post-HT. The incidence of CRC increased with age at HT from 56.6 per 100,000 person-years among under 45 year olds to 436.4 per 100,000 person-years among over 64 year olds. The incidence rates for age-at-diagnosis groups were significantly greater than those estimated for the general Spanish population. Curative surgery, performed for 62 of 74 operable tumors, increased the probability of patient survival since a diagnosis of CRC, from 31.6% to 75.7% at 2 years, and from 15.8% to 48.6% at 5 years, compared to patients with inoperable tumors. Our results suggest that the incidence of CRC among HT patients is greater than in the general population, increasing with age at HT.

BACKGROUND: Heart transplantation is an effective treatment offering the best recovery in both quality and quantity of life in those affected by refractory, severe heart failure. However, transplantation is limited by donor organ availability. The reintroduction of heart donation after the circulatory determination of death (DCD) in 2014 offered an uplift in transplant activity by 30%. Thoraco-abdominal normothermic regional perfusion (taNRP) enables in-situ reperfusion of the DCD heart. The objective of this paper is to assess the clinical outcomes of DCD donor hearts recovered and transplanted from donors undergoing taNRP. METHOD: This was a multicentre retrospective observational study. Outcomes included functional warm ischaemic time, use of mechanical support immediately following transplantation, perioperative and long-term actuarial survival and incidence of acute rejection requiring treatment. 157 taNRP DCD heart transplants, performed between February 2, 2015, and July 29, 2022, have been included from 15 major transplant centres worldwide including the UK, Spain, the USA and Belgium. 673 donations after the neurological determination of death (DBD) heart transplantations from the same centres were used as a comparison group for survival. FINDINGS: taNRP resulted in a 23% increase in heart transplantation activity. Survival was similar in the taNRP group when compared to DBD. 30-day survival was 96.8% ([92.5%-98.6%] 95% CI, n = 156), 1-year survival was 93.2% ([87.7%-96.3%] 95% CI, n = 72) and 5-year survival was 84.3% ([69.6%-92.2%] 95% CI, n = 13). INTERPRETATION: Our study suggests that taNRP provides a significant boost to heart transplantation activity. The survival rates of taNRP are comparable to those obtained for DBD transplantation in this study. The similar survival may in part be related to a short warm ischaemic time or through a possible selection bias of younger donors, this being an uncontrolled observational study. Therefore, our study suggests that taNRP offers an effective method of organ preservation and procurement. This early success of the technique warrants further investigation and use. FUNDING: None of the authors have a financial relationship with a commercial entity that has an interest in the subject. ispartof: ECLINICALMEDICINE vol:58 ispartof: location:England status: published

The outcomes of heart-lung transplant (HLT) are worse than those of heart transplant (HT) and lung transplant alone; this and the availability of mechanical assistance have meant that the indications for HLT have been changing. This study aims to analyze the evolution of indications for HLT in a country of 47 million inhabitants.We performed a retrospective observational study of all HLTs performed in Spain (performed in 2 centers) from 1990 to 2020. The total number of patients included was 1751 (HT 1673 and HLT 78). After clinical adjustment, overall survival was compared between the 2 groups. Seven etiological subgroups were considered within the HLT group: (1) cardiomyopathy with pulmonary hypertension (CM + PH);, (2) Eisenmenger syndrome, (3) congenital heart disease without Eisenmenger syndrome, (4) idiopathic pulmonary arterial hypertension (IPAH), (5) cystic fibrosis, (6) chronic obstructive pulmonary disease (COPD) and/or emphysema), and (7) diffuse interstitial lung disease.There were a large number of differences between patients with HLT vs HT. HLT had a 2.69-fold increased probability of death in the first year compared with HT. The indications for HLT have changed over the years. In the recent period the indications are mainly congenital heart disease and Eisenmenger syndrome, with some cases of CM + PH. Other indications for HLT have virtually disappeared, mainly lung diseases (IPAH, COPD, cystic fibrosis). Median survival was low in CM + PH (18 days), diffuse interstitial lung disease (29 days), and ischemic heart disease (114 days); intermediate in Eisenmenger syndrome (600 days); and longer in IPAH, COPD and/or emphysema, and cystic fibrosis.HLT is a procedure with high mortality. This and mechanical assists mean that the indications have changed over the years. Etiological analysis is of utmost interest to take advantage of organs and improve survival.

Resumen El shock cardiogenico (SC) es una entidad que comprende multiples etiologias y asocia elevada mortalidad. Algunas escalas de gravedad han demostrado ser buenos predictores de mortalidad hospitalaria en pacientes ingresados en Unidades de Cuidados Intensivos (UCI). El objetivo principal de este estudio es analizar su utilidad y validez en una cohorte de pacientes en SC. Metodos Estudio observacional unicentrico. Se calcularon las escalas SOFA, SAPS II y APACHE II en las primeras 24 horas de ingreso en UCI. Resultados Se incluyeron 130 pacientes con SC. Las escalas SOFA, SAPS II y APACHE II mostraron buena discriminacion para la mortalidad hospitalaria, obteniendo valores de area bajo la curva (AUC) ROC similares (AUC: 0,711, 0,752 y 0,742, respectivamente; p = 0,6). La calibracion, estimada por el test de Hosmer-Lemeshow, fue adecuada en todos los casos, SOFA (p = 0,787), SAPS II (p = 0,078) y APACHE II (p = 0,522). Resultaron predictores independientes de mortalidad intrahospitalaria: el sindrome coronario agudo (SCA), los valores de lactato serico, el SAPS II y el indice de vasoactivos inotropicos (VIS) en las primeras 24 horas de ingreso en UCI. Con estas variables se desarrollo un indicador pronostico especifico para el SC (SAPS-2-LIVE) que mejora la capacidad predictiva de mortalidad en nuestra serie (AUC) ROC, 0,825 (IC 95% 0,752-0,89). Conclusion En esta cohorte contemporanea de SC, las escalas SOFA, SAPS II y APACHE II han demostrado una buena capacidad de prediccion de mortalidad hospitalaria. Estos hallazgos podrian contribuir a una mejor estratificacion del riesgo en el SC.

Resumen Introduccion y objetivos El retrasplante cardiaco (ReTC) representa un tema controvertido actualmente. Nuestro objetivo es describir y analizar los resultados del ReTC en Espana. Metodos Analisis retrospectivo del Registro Espanol de Trasplante Cardiaco de 1984 a 2018. Se recogieron datos sobre donante, receptor, cirugia, inmunosupresion y supervivencia. La mortalidad por todas las causas o la necesidad de ReTC postrasplante fueron el objetivo principal. Se estudiaron diferencias en supervivencia segun indicacion, tiempo entre trasplantes y epoca del ReTC. Resultados Se estudiaron en total 7.592 trasplantes cardiacos (TxC) y 173 (2,3%) ReTC (mediana de edad, 52,0 y 55,0 anos respectivamente). La enfermedad vascular del injerto fue la indicacion de ReTC mas frecuente (42,2%) y 59 pacientes (80,8%) recibieron el ReTC mas de 5 anos despues del trasplante inicial. El rechazo agudo y el fallo primario del injerto disminuyeron como indicaciones durante el periodo estudiado. La insuficiencia renal, la hipertension, la necesidad de ventilacion mecanica o balon intraaortico y la mayor duracion de la isquemia fria fueron mas frecuentes en el ReTC. La mediana de seguimiento del ReTC fue 5,8 anos. El ReTC tuvo peor supervivencia que el TxC (HR ponderado = 1,43; IC95%, 1,17-1,44; p Conclusiones El ReTC se asocio con mayor mortalidad que el TxC, especialmente por rechazo agudo. El pronostico del ReTC realizado mas de 5 anos despues es similar al del TxC primario.

[Abstract] Background. Primary graft dysfunction (PGD) still affects 2-28% of heart transplants (HT). Severe PGD requires mechanical circulatory support (MCS) and is the main cause of death early after HT. Earlier initiation has been suggested to improve prognosis but the best cannulation strategy is unknown. Methods. Analysis of all HT in Spain between 2010 and 2020. Early (

Resumen Introduccion y objetivos Se presentan las caracteristicas clinicas y los resultados de los trasplantes cardiacos realizados en Espana con la actualizacion correspondiente a 2019. Metodos Se describen las caracteristicas clinicas y los resultados de los trasplantes cardiacos realizados en 2019, asi como las tendencias de estos en el periodo 2010-2018. Resultados En 2019 se realizaron 300 trasplantes (8.794 desde 1984; 2.745 entre 2010 y 2019). Respecto a anos previos, los cambios mas llamativos son el descenso hasta el 38% de los trasplantes realizados en codigo urgente, y la consolidacion en el cambio de asistencia circulatoria pretrasplante, con la practica desaparicion del balon de contrapulsacion (0,7%), la estabilizacion del uso del oxigenador extracorporeo de membrana (9,6%) y el aumento de los dispositivos de asistencia ventricular (29%). La supervivencia en el trienio 2016-2018 es similar a la del trienio 2013-2015 (p = 0,34), y ambas mejores que la del trienio 2010-2012 (p = 0,002 y p = 0,01 respectivamente). Conclusiones Se mantienen estables tanto la actividad del trasplante cardiaco en Espana como los resultados en supervivencia en los ultimos 2 trienios. Hay una tendencia a realizar menos trasplantes urgentes, la mayoria con dispositivos de asistencia ventricular.

[Abstract] Background: We aimed to describe recent trends in the use and outcomes of temporary mechanical circulatory support (MCS) as a bridge to heart transplantation (HTx) in Spain. Methods: Retrospective case-by-case analysis of 1,036 patients listed for emergency HTx while on temporary MCS in 16 Spanish institutions from January 1st, 2010 to December 31st, 2020. Patients were classified in 3 eras according to changes in donor allocation criteria (Era 1: January 2010/May 2014; Era 2: June 2014/May 2017; Era 3: June 2017/December 2020). Results: Over time, the proportion of candidates listed with intra-aortic balloon pumps decreased (Era 1 = 55.9%, Era 2 = 32%, Era 3 = 0.9%; p < 0.001), while the proportion of candidates listed with surgical continuous-flow temporary VADs (Era 1 = 10.6%, Era 2 = 32%, Era 3 = 49.1%; p < 0.001) and percutaneous VADs (Era 1 = 0.3%, Era 2 = 6.3%; Era 3 = 17.2%; p < 0.001) increased. Rates of HTx increased from Era 1 (79.4%) to Era 2 (87.8%), and Era 3 (87%) (p = 0.004), while rates of death before HTx decreased (Era 1 = 17.7%; Era 2 = 11%, Era 3 = 12.4%; p = 0.037) Median time from listing to HTx increased in patients supported with intra-aortic balloon pumps (Era 1 = 8 days, Era 2 = 15 days; p < 0.001) but remained stable in other candidates (Era 1 = 6 days; Era 2 = 5 days; Era 3 = 6 days; p = 0.134). One-year post-transplant survival was 71.4% in Era 1, 79.3% in Era 2, and 76.5% in Era 3 (p = 0.112). Preoperative bridging with ECMO was associated with increased 1-year post-transplant mortality (adjusted HR=1.71; 95% CI 1.15-2.53; p = 0.008). Conclusions: During the period 2010 to 2020, successive changes in the Spanish organ allocation protocol were followed by a significant increase of the rate of HTx and a significant reduction of waiting list mortality in candidates supported with temporary MCS. One-year post-transplant survival rates remained acceptable.

[Abstract] Introduction and objectives: The Index for Mortality Prediction After Cardiac Transplantation (IMPACT) score was derived and validated as a predictor of mortality after heart transplantation (HT). The primary objective of this work is to externally validate the IMPACT score in a contemporary Spanish cohort. Methods: Spanish Heart Transplant Registry data were used to identify adult (>16 years) HT patients between January 2000 and December 2015. Retransplantation, multiorgan transplantation and patients in whom at least one of the variables required to calculate the IMPACT score was missing were excluded from the analysis (N = 2810). Results: Median value of the IMPACT score was five points (IQR: 3, 8). Overall, 1-year survival rate was 79.1%. Kaplan-Meier 1-year survival rates by IMPACT score categories (0-2, 3-5, 6-9, 10-14, ≥15) were 84.4%, 81.5%, 79.3%, 77.3%, and 58.5%, respectively (Log-Rank test: p < .001). Performance analysis showed a good calibration (Hosmer-Lemeshow chi-square for 1 year was 7.56; p = .47) and poor discrimination ability (AUC-ROC .59) of the IMPACT score as a predictive model. Conclusions: In a contemporary Spanish cohort, the IMPACT score failed to accurately predict the risk of death after HT.

Cardiac amyloidosis (CA) is produced by amyloid fiber deposition in the myocardium. The most frequent forms are those caused by light chains (AL) and transthyretin (ATTR). Our objective was to describe the diagnosis, treatment and outcomes of CA in a specialized Spanish center.We included all patients diagnosed with CA in Hospital Universitario Puerta de Hierro Majadahonda from May 2008 to September 2018. We analyzed their clinical characteristics, outcomes, and survival.We included 180 patients with CA, of whom 64 (36%) had AL (50% men; mean age, 65±11 years) and 116 had ATTR (72% men; mean age 79±11 years; 18 with hereditary ATTR). The most common presentation was heart failure in both groups (81% in AL and 45% in ATTR, P.01). Other forms of presentation in ATTR patients were atrial arrhythmias (16%), conduction disorders (6%), and incidental finding (6%); 70 patients (40%), had a previous alternative cardiac diagnosis. Diagnosis was noninvasive in 75% of ATTR patients. Diagnostic delay was higher in ATTR (2.8±4.3 vs 0.6±0.7 years, P.001), but mortality was greater in AL patients (48% vs 32%, P=.028). Independent predictors of mortality were AL subtype (HR, 6.16; 95%CI, 1.56-24.30; P=.01), female sex (HR, 2.35; 95%CI, 1.24-4.46; P=.01), and NYHA functional class III-IV (HR, 2.07; 95%CI, 1.11-3.89; P=.02).CA is a clinical challenge, with wide variability in its presentation depending on the subtype, leading to diagnostic delay and high mortality. Improvements are needed in the early diagnosis and treatment of these patients.

Resumen Introduccion y objetivos La mortalidad en shock cardiogenico (SC), sobre todo en casos refractarios donde se emplean dispositivos de soporte circulatorio mecanico (SCM), es muy elevada. El objetivo es analizar los resultados de un programa de atencion a pacientes en SC tras la implementacion de un equipo multidisciplinario en nuestro centro y la organizacion en red con los hospitales del area. Metodos Estudio observacional retrospectivo de los pacientes atendidos desde septiembre de 2014 a enero de 2019. Se incluyo a los pacientes en SC refractario que precisaron SCM y aquellos que por edad y ausencia de comorbilidades pueden ser candidatos a tratamientos avanzados. El objetivo principal es evaluar la supervivencia hospitalaria. Resultados En total se incluyo a 130 pacientes (69 locales y 61 trasladados). La media de edad era 52 ± 15 anos (el 72% varones). Las etiologias predominantes de SC fueron la insuficiencia cardiaca aguda descompensada (29%), el infarto agudo de miocardio (26%) y el shock tras cardiotomia (25%). En 105 pacientes (81%) se empleo SCM, mayoritariamente oxigenador extracorporeo de membrana (58%). La supervivencia al alta hospitalaria fue del 57% (74 de 130 pacientes). Los principales destinos fueron la recuperacion miocardica y el trasplante cardiaco. La puntuacion SAPS II, el lactato, el infarto como etiologia del SC y la puntuacion de inotropicos y vasoactivos resultaron predictores independientes de mortalidad hospitalaria. Conclusiones La creacion de equipos multidisciplinarios para la atencion de pacientes en SC mayoritariamente refractario y la coordinacion en red con los hospitales del area son factibles y consiguen una supervivencia hospitalaria de mas de la mitad de los pacientes atendidos.

Introduction and objectives Mortality remains high in cardiogenic shock (CS), especially in refractory CS involving the use of mechanical circulatory support (MCS) devices. The aim of this study was to analyze the results of a care program for patients in CS after the creation of a multidisciplinary team in our center and a regional network of hospitals in our area. Methods Observational and retrospective study of patients attended in this program from September 2014 to January 2019. We included patients in refractory CS who required MCS and those who, because of their age and absence of comorbidities, were candidates for advanced therapies. The primary endpoint was survival to discharge. Results A total of 130 patients were included (69 local and 61 transferred patients). The mean age was 52 ± 15 years (72% men). The most frequent causes of CS were acute decompensated heart failure (29%), acute myocardial infarction (26%), and postcardiotomy CS (25%). MCS was used in 105 patients (81%), mostly extracorporeal membrane oxygenation (58%). Survival to discharge was 57% (74 of 130 patients). The most frequent destinations were myocardial recovery and heart transplant. Independent predictors of in-hospital mortality were SAPS II score, lactate level, acute myocardial infarction etiology, and vasoactive-inotropic score. Conclusions The creation of multidisciplinary teams for patients with mainly refractory CS and a regional network is feasible and allows survival to discharge in more than a half of attended patients with CS.

Introducción y objetivos Se presentan las características clínicas y los resultados de los trasplantes cardiacos realizados en España con la actualización correspondiente a 2019. Métodos Se describen las características clínicas y los resultados de los trasplantes cardiacos realizados en 2019, así como las tendencias de estos en el periodo 2010-2018. Resultados En 2019 se realizaron 300 trasplantes (8.794 desde 1984; 2.745 entre 2010 y 2019). Respecto a años previos, los cambios más llamativos son el descenso hasta el 38% de los trasplantes realizados en código urgente, y la consolidación en el cambio de asistencia circulatoria pretrasplante, con la práctica desaparición del balón de contrapulsación (0, 7%), la estabilización del uso del oxigenador extracorpóreo de membrana (9, 6%) y el aumento de los dispositivos de asistencia ventricular (29%). La supervivencia en el trienio 2016-2018 es similar a la del trienio 2013-2015 (p = 0, 34), y ambas mejores que la del trienio 2010-2012 (p = 0, 002 y p = 0, 01 respectivamente). Conclusiones Se mantienen estables tanto la actividad del trasplante cardiaco en España como los resultados en supervivencia en los últimos 2 trienios. Hay una tendencia a realizar menos trasplantes urgentes, la mayoría con dispositivos de asistencia ventricular. Introduction and objectives: The present report describes the clinical characteristics and outcomes of heart transplants in Spain and updates the data to 2019. Methods: We describe the clinical characteristics and outcomes of heart transplants performed in Spain in 2019, as well as trends in this procedure from 2010 to 2018. Results: In 2019, 300 transplants were performed (8794 since 1984; 2745 between 2010 and 2019). Compared with previous years, the most notable findings were the decreasing rate of urgent transplants (38%), and the consolidation of the type of circulatory support prior to transplant, with an almost complete disappearance of counterpulsation balloon (0.7%), stabilization in the use of extracorporeal membrane oxygenation (9.6%), and an increase in the use of ventricular assist devices (29.0%). Survival from 2016 to 2018 was similar to that from 2013 to 2015 (P = .34). Survival in both these periods was better than that from 2010 to 2012 (P = .002 and P = .01, respectively). Conclusions: Heart transplant activity has remained stable during the last few years, as have outcomes (in terms of survival). There has been a trend to a lower rate of urgent transplants and to a higher use of ventricular assist devices prior to transplant.

Chronic kidney disease is a major complication after heart transplantation with wide inter-individual variability. Calcineurin inhibitor nephrotoxicity, mediated by transforming growth factor-beta1 (TGF-β1), is an important contributing factor. Our objective was to evaluate the association between TGF-β1 polymorphisms and renal dysfunction 1-year after heart transplantation.Single-center observational study that included patients who received a first heart transplant between 1990-2013. According to the 1-year eGFR decline, patients were classified as "Stable" (decrease in eGFR10% or eGFR60 ml/min/1.73m2) or "Progressors" (decrease in eGFR10% and eGFR60 ml/min/1.73m2). "Progressors" were then subdivided by the degree of eGFR decrease in "Mild progressors" (10-30%) or "Rapid progressors" (30%). The association between TGF-β1 +869TC polymorphism and other risk factors with the eGFR outcome was analysed.A total of 355 patients (78% male; 50.7 ± 11.8 years) were included. According to the 1-year eGFR decline, 220 patients (62%) were classified as "Stable" and 135 (38%) as "Progressors". TGF-β1+869CC genotype was more prevalent in "Stable" vs "Progressors" group (20% vs 8%, p = 0.009). In the multivariate analysis, female sex (p 0.02) and eGFR60 ml/min/1.73 m2 at first month post-heart transplant (p = 0.004) remained as risk factors of eGFR decline, and TGF-β1 + 869CC genotype (p = 0.001) and renal dysfunction pre-heart transplant (p = 0.04) as protective factors. TGF-β1 + 869CC genotype was less frequently found in "Mild progressors" compared to "Rapid progressors" [p = 0.019; OR (95%CI) = 0.19 (.05-.76)].The TGF-β1 +869CC genotype is associated with a lower risk of calcineurin inhibitor nephrotoxicity after heart transplant. This genetic susceptibility could enable a more personalized patient treatment.

This report presents the clinical characteristics, outcomes and complications of all consecutive patients implanted with a long-term mechanical circulatory support device in Spain between 2007 and 2020.Analysis of the Spanish Registry of durable ventricular assist devices (REGALAD) including data form Spanish centers with a mechanical circulatory support program.During the study period, 263 ventricular assist devices were implanted in 22 hospitals. The implanted device was an isolated continuous-flow left ventricular assist device in 182 patients (69%), a pulsatile-flow device (58 isolated left ventricular and 21 biventricular) in 79 (30%), and a total artificial heart in 2 patients (1%). The strategy of the implant was as bridge to heart transplant in 78 patients (30%), bridge to candidacy in 110 (42%), bridge to recovery in 3 (1%) and destination therapy in 72 patients (27%). Overall survival at 6, 12 and 24 months was 79%, 74% and 69%, respectively, and was better in continuous-flow left ventricular assist devices (84%, 80%, and 75%). The main adverse events related to this therapy were infections (37% of patients), bleeding (35%), neurological (29%), and device malfunction (17%).Durable ventricular assist devices have emerged in Spain in the last few years as a useful therapy for patients with advanced heart failure. As in other international registries, the current trend is to use continuous-flow intracorporeal left ventricular devices, which are associated with better results. Adverse events continue to be frequent and severe.

This report updates the annual data of the Spanish Heart Transplant Registry with the procedures performed in 2021.We describe the clinical profile, therapeutic characteristics and outcomes in terms of survival of the procedures performed in 2021. Their temporal trends are updated for the 2012 to 2020 period.In 2021, 302 heart transplants were performed (8.6% increase versus 2020). The tendency in 2021 confirmed that of prior years, with fewer urgent transplants and a preference for the use of ventricular assist devices. The remaining characteristics and survival showed a clear trend toward stability in the last decade. Compared with 2019, the SARS-CoV-2 pandemic (2020 and 2021) did not affect short- or long-term survival.In 2021, transplant activity returned to prepandemic levels. The SARS-CoV-2 pandemic did not significantly affect transplant outcomes. The main transplant features and outcomes have clearly stabilized in the last decade.

Endomyocardial biopsy (EMB) is the only technique able to establish an etiological diagnosis of myocarditis or inflammatory cardiomyopathy (ICM). The aim of this study was to analyze the clinical profile, outcomes, and prognostic factors of patients with suspected myocarditis/ICM undergoing EMB.We retrospectively analyzed the clinical characteristics, histological findings, and follow-up data of all patients with suspected myocarditis or ICM who underwent EMB between 1997 and 2019 in a Spanish tertiary hospital. The diagnostic yield was compared using the Dallas criteria vs immunohistochemical criteria (IHC).A total of 99 patients underwent EMB (67% male; mean age, 42±15 years; mean left ventricular ejection fraction [LVEF], 34%±14%). Myocarditis or ICM was confirmed in 28% with application of the Dallas criteria and in 54% with the IHC criteria (P.01). Lymphocytic myocarditis was diagnosed in 47 patients, eosinophilic myocarditis in 6, sarcoidosis in 3, and giant cell myocarditis in 1 patient. After a median follow-up of 18 months, 23 patients (23%) required heart transplant (HTx), a left ventricular assist device (LVAD), and/or died. Among the patients with IHC-confirmed myocarditis, 21% required HTx/LVAD or died vs 7% of those without inflammation (P=.056). The factors associated with a worse prognosis were baseline LVEF ≤ 30%, left ventricular end-diastolic diameter ≥ 60mm, and NYHA III-IV, especially in the presence of inflammation.EMB allows an etiological diagnosis in more than half of patients with suspected myocarditis/ICM when IHC techniques are used. IHC-confirmed inflammation adds prognostic value and helps to identify patients with a higher probability of developing complications.

Resumen: La definición más actualizada de falla cardiaca avanzada incluye síntomas refractarios al tratamiento convencional, independiente de la fracción de eyección del ventrículo izquierdo, acompañados de elevación de péptidos natriuréticos. En esta nueva definición se destacan condiciones específicas como son la necesidad usual de diurético intravenoso, hospitalizaciones o consultas frecuentes por falla cardíaca, medicación inotrópica intermitente y arritmias malignas recurrentes.Esta condición está presente en aproximadamente 4 a 6% de los pacientes con falla cardiaca y se asocia a más síntomas, más comorbilidades y mayor mortalidad (hasta 75% a un año). En este punto, las terapias convencionales han fallado o son refractarias y se requiere la toma de decisiones para instaurar tratamientos más avanzados (p. ej.: inotrópicos, asistencia ventricular, trasplante cardiaco, cuidado paliativo, entre otras).El tratamiento representa un reto para los sistemas de salud de Colombia por la necesidad de optimizar y racionalizar el uso de estos recursos; por esto se debe considerar que estos pacientes, en algún momento de su evolución, que sean remitidos para valoración en las Clínicas de Falla Cardíaca ya que estos servicios cuentan con personal altamente calificado y una estructura administrativa, logística y tecnológica que garantizan el acceso a un tratamiento integral y multidisciplinario con estándares de calidad internacionales que es lo que finalmente se requiere en estas condiciones.En el “Consenso colombiano de falla cardiaca avanzada” se presentan diferentes opciones de tratamiento a considerar y un modelo de atención integral que inicia desde el momento del diagnóstico hasta etapas refractarias y avanzadas. Abstract: The most up-to-date definition of advanced heart failure includes symptoms refractory to conventional treatment (regardless of the left ventricular ejection fraction), accompanied by an elevation in natriuretic peptides. Specific conditions are highlighted in this new definition, such as the usual need of an intravenous diuretic, hospital admissions, or frequent consultations due to heart failure, intermittent inotropic medication, and recurrent malignant arrhythmias.This condition is present in approximately 4 to 6% of patients with heart failure and is associated with more symptoms, more comorbidities, and higher mortality (up to 75% at one year). On this point, the conventional treatments have failed, or are refractory, and decisions need to be taken to introduce more advanced treatments (for example: inotropic, ventricular assistance, palliative care, among others).The treatment represents a challenge for the Health Systems of Colombia due to the need to optimise and rationalise the use of these resources. For this reason, it must be considered that these patients, at any time during its course, may be sent for assessment in Heart Failure Clinics, as these services have highly qualified staff and an administrative, logistics, and technological structure that ensures access to an integral and multidisciplinary treatment with standards of international quality, which is what is finally required in these conditions.Different treatment options to consider are presented in the “Colombian consensus on advanced heart failure”, as well as an integral care model that begins from the time of the diagnosis until the refractory and advanced stages. Palabras clave: Avanzada, Tratamiento, Clínicas, Inotrópicos, Trasplante, Paliativo, Enfermería, Key words: Advanced, Treatments, Clinics, Inotropes, Transplant, Palliative, Nursing

Sacubitril/valsartan (SV) is a new therapy in heart failure with reduced ejection fraction. Our aim was to determine the efficacy and safety of this drug daily clinical practice. We performed a multicenter registry in 10 hospitals. All patients who started SV from October 2016 to March 2017 on an outpatient basis were included. A total of 427 patients started treatment with SV. Mean follow-up was 7.0 ± 0.1 months. Forty-nine patients (11.5%) discontinued SV, and 12 (2.8%) died. SV discontinuation was associated with higher cardiovascular (hazard ratio 13.22, 95% confidence interval, 6.71-15.73, P < 0.001) and all-cause mortality (hazard ratio 13.51, 95% confidence interval 3.22-56.13, P < 0.001). Symptomatic hypotension occurred in 71 patients (16.6%). Baseline N-terminal pro-B-type natriuretic peptide levels, functional class, and left ventricular ejection fraction improved at the end of follow-up in patients who continued with SV (all P values ≤0.001). This improvement was not significant in patients with SV discontinuation. SV has a good tolerability in patients from daily clinical practice. SV withdrawal in patients with heart failure and reduced ejection fraction was independently associated with increased all-cause mortality. Patients who continued with SV presented an improvement in functional class left ventricular ejection fraction and N-terminal pro-B-type natriuretic peptide levels. Sin financiación 2.598 JCR (2019) Q2, 62/138 Cardiac & Cardiovascular Systems; Q3, 147/270 Pharmacology & Pharmacy 0.800 SJR (2019) Q2, 110/362 Cardiology and Cardiovascular Medicine, 112/331 Pharmacology No data IDR 2019 UEM

Heart failure (HF) is a syndromic condition with a high incidence in current medicine. When the symptoms of HF progress, and become refractory, cardiac transplant is the best therapeutic option. However, due to the shortage of donors and the long waiting lists, many of those patients are candidates for implantation of ventricular assist devices as a bridge to the cardiac transplant, or when this is not an option, as a definitive therapy. A series of four clinical cases of patients with ventricular assist devices that required surgical intervention, is presented. Three of them were assisted with long-term care: two EXCOR (pulsatile and para-corporeal) and one HEARTWARE (non-pulsatile and intra-corporeal), and the last one with short-term assistance; CentriMag biventricular Levitronix. There is no significant literature on the peri-operative implications of these patients when they undergo urgent or scheduled surgery. The experience in our centre leads us to raise the need to determine a series of aspects: operation of each device, emphasising the correct placement of the cannulas during the surgery; the proper management of any medication, emphasising the importance of anticoagulant and anti-platelet therapies; the Pathophysiological changes at cardiopulmonary level due to the implantation of these devices; and the importance of the administration of a correct antibiotic therapy. Given the complexity of these cases, the limited experience in this field, and the few cases that exist in these situations, it is recommended to create protocols to ensure their proper management.

Resumen La insuficiencia cardiaca constituye un cuadro sindromico de elevada incidencia en la medicina actual. Cuando los sintomas de la insuficiencia cardiaca progresan y se convierten en refractarios, la indicacion de trasplante cardiaco es la mejor opcion terapeutica. Sin embargo, debido a la escasez de donantes y a las largas listas de espera, muchos de estos pacientes necesitan la implantacion de dispositivos de asistencia ventricular como puente a este trasplante, o en algunos casos, cuando el trasplante no es una opcion, como terapia definitiva. En este articulo se presenta una serie de 4 casos clinicos en pacientes portadores de dispositivos de asistencia ventricular que precisaron intervencion quirurgica. Tres de ellos estaban asistidos con asistencias de larga duracion: 2 EXCOR (pulsatiles y paracorporea) y un HEARTWARE (no pulsatil e intracorporea) y el ultimo con una asistencia de corta duracion; CentriMag Levitronix biventricular. No existe bibliografia significativa sobre las implicaciones perioperatorias de estos pacientes cuando son sometidos a cirugia urgente o programada. La experiencia en nuestro centro nos lleva a plantear la necesidad de conocer una serie de aspectos: funcionamiento de cada dispositivo, recalcando la correcta colocacion de las canulas durante la cirugia; el manejo apropiado de la medicacion, recalcando la importancia de las terapias anticoagulantes y antiagregantes; los cambios fisiopatologicos a nivel cardiopulmonar debidos a la implantacion de estos dispositivos; y la importancia de la administracion de una correcta antibioterapia. Ante la complejidad que presentan estos casos, la escasa experiencia en este campo y los pocos casos que existen de estas situaciones se recomienda la creacion de protocolos para garantizar un manejo correcto de estos.

Aims Our objective was to better understand the genetic bases of dilated cardiomyopathy (DCM), a leading cause of systolic heart failure. Methods and results We conducted the largest genome-wide association study performed so far in DCM, with 2719 cases and 4440 controls in the discovery population. We identified and replicated two new DCM-associated loci on chromosome 3p25.1 [lead single-nucleotide polymorphism (SNP) rs62232870, P = 8.7 × 10−11 and 7.7 × 10−4 in the discovery and replication steps, respectively] and chromosome 22q11.23 (lead SNP rs7284877, P = 3.3 × 10−8 and 1.4 × 10−3 in the discovery and replication steps, respectively), while confirming two previously identified DCM loci on chromosomes 10 and 1, BAG3 and HSPB7. A genetic risk score constructed from the number of risk alleles at these four DCM loci revealed a 3-fold increased risk of DCM for individuals with 8 risk alleles compared to individuals with 5 risk alleles (median of the referral population). In silico annotation and functional 4C-sequencing analyses on iPSC-derived cardiomyocytes identify SLC6A6 as the most likely DCM gene at the 3p25.1 locus. This gene encodes a taurine transporter whose involvement in myocardial dysfunction and DCM is supported by numerous observations in humans and animals. At the 22q11.23 locus, in silico and data mining annotations, and to a lesser extent functional analysis, strongly suggest SMARCB1 as the candidate culprit gene. Conclusion This study provides a better understanding of the genetic architecture of DCM and sheds light on novel biological pathways underlying heart failure.

Introduction and objectives: The present report updates the main characteristics and outcomes of heart transplants in Spain to 2020. Methods: We describe the main features of recipients, donors, surgical procedure, and immunosuppression in 2020. We also analyze the temporal trends of these characteristics and outcomes (survival) for the period 2011 to 2019. Results: In 2020, 278 heart transplants were performed (7.3% decrease vs 2019). The findings in 2020 confirmed previous observations of an increase in pretransplant sternotomy, a slight decrease in urgent transplants carried out with ventricular assist devices, a slight decrease in donor age, an increase in the use of allografts with previous arrest, and a decrease in ischemia time. Survival continued to improve in recent triennia, reaching 82.0% at 1 year in the period 2017 to 2019. Conclusions: The slight decrease in the number of heart transplants performed in 2020 in Spain, most likely due to the SARS-CoV-2 pandemic, did not change the main characteristics of the procedure. No change was observed in the tendency to improved survival. (C) 2021 Sociedad Espanola de Cardiologia. Published by Elsevier Espana, S.L.U. All rights reserved.

Introducción y objetivos La amiloidosis cardiaca (AC) se produce por depósito de fibras de amiloide en el miocardio. Las formas más frecuentes son la amiloidosis por cadenas ligeras (AL) y por transtiretina (ATTR). Nuestro objetivo es describir la experiencia en el diagnóstico, tratamiento y pronóstico en un centro especializado español. Métodos Se incluyeron todos los pacientes diagnosticados de AC en el XXXX1 desde mayo de 2008 a septiembre de 2018 y se analizaron sus características clínicas, evolución y supervivencia. Resultados Se incluyeron 180 pacientes con AC, de los cuales, 64 (36%) eran AL (50% varones;65±11 años) y 116 ATTR (72% varones;79±11 años, 19 con ATTR hereditaria). La forma de presentación más frecuente fue la insuficiencia cardiaca en ambos grupos (81% AL y 45% ATTR;p

Heart retransplantation (ReHT) is controversial in the current era. The aim of this study was to describe and analyze the results of ReHT in Spain. Methods We performed a retrospective cohort analysis from the Spanish Heart Transplant Registry from 1984 to 2018. Data were collected on donors, recipients, surgical procedure characteristics, immunosuppression, and survival. The main outcome was posttransplant all-cause mortality or need for ReHT. We studied differences in survival according to indication for ReHT, the time interval between transplants and era of ReHT. Results A total of 7592 heart transplants (HT) and 173 (2.3%) ReHT were studied (median age, 52.0 and 55.0 years, respectively). Cardiac allograft vasculopathy was the most frequent indication for ReHT (42.2%) and 59 patients (80.8%) received ReHT > 5 years after the initial transplant. Acute rejection and primary graft failure decreased as indications over the study period. Renal dysfunction, hypertension, need for mechanical ventilation or intra-aortic balloon pump and longer cold ischemia time were more frequent in ReHT. Median follow-up for ReHT was 5.8 years. ReHT had worse survival than HT (weighted HR, 1.43; 95%CI, 1.17-1.44; P < .001). The indication of acute rejection (HR, 2.49; 95%CI, 1.45-4.27; P < .001) was related to the worst outcome. ReHT beyond 5 years after initial HT portended similar results as primary HT (weighted HR, 1.14; 95%CI, 0.86-1.50; P < .001). Conclusions ReHT was associated with higher mortality than HT, especially when indicated for acute rejection. ReHT beyond 5 years had a similar prognosis to primary HT. El retrasplante cardiaco (ReTC) representa un tema controvertido actualmente. Nuestro objetivo es describir y analizar los resultados del ReTC en España. Métodos Análisis retrospectivo del Registro Español de Trasplante Cardiaco de 1984 a 2018. Se recogieron datos sobre donante, receptor, cirugía, inmunosupresión y supervivencia. La mortalidad por todas las causas o la necesidad de ReTC postrasplante fueron el objetivo principal. Se estudiaron diferencias en supervivencia según indicación, tiempo entre trasplantes y época del ReTC. Resultados Se estudiaron en total 7.592 trasplantes cardiacos (TxC) y 173 (2,3%) ReTC (mediana de edad, 52,0 y 55,0 años respectivamente). La enfermedad vascular del injerto fue la indicación de ReTC más frecuente (42,2%) y 59 pacientes (80,8%) recibieron el ReTC más de 5 años después del trasplante inicial. El rechazo agudo y el fallo primario del injerto disminuyeron como indicaciones durante el periodo estudiado. La insuficiencia renal, la hipertensión, la necesidad de ventilación mecánica o balón intraaórtico y la mayor duración de la isquemia fría fueron más frecuentes en el ReTC. La mediana de seguimiento del ReTC fue 5,8 años. El ReTC tuvo peor supervivencia que el TxC (HR ponderado = 1,43; IC95%, 1,17-1,44; p < 0,001). El rechazo agudo (HR = 2,49; IC95%, 1,45-4,27; p < 0,001) se relacionó con el peor resultado. El ReTC más allá de 5 años del trasplante inicial presagia resultados similares a los del TxC primario (HR ponderado = 1,14; IC95%, 0,86-1,50; p < 0,001). Conclusiones El ReTC se asoció con mayor mortalidad que el TxC, especialmente por rechazo agudo. El pronóstico del ReTC realizado más de 5 años después es similar al del TxC primario. Sin financiación No data JCR 2021 0.385 SJR (2021) Q3, 1499/2489 Medicine (miscellaneous) No data IDR 2021 UEM

Heart transplantation from controlled donation after the circulatory determination of death (cDCDD) may help to increase the availability of hearts for transplantation. During 2020, four heart transplants were performed at three different Spanish hospitals based on the use of thoraco-abdominal normothermic regional perfusion (TA-NRP) followed by cold storage (CS). All donors were young adults

Introduction and objectives Sacubitril/valsartan (SV) is recommended in patients with heart failure and reduced left ventricular ejection fraction. However, the characteristics of the centers included in the pivotal clinical trial differ from real life. The present study aims to analyze the clinical profile of patients treated with SV according to hospital type and the potential role of these differences in drug safety and effectivity. Methods Prospective multicenter registry that included outpatients treated with SV. We compared baseline characteristics and treatments, drug titration, and data at 7-months follow-up according to hospital type. Results From 427 patients, 81 (19%) were included in medium/small size hospitals, 204 (48%) in heart transplant program centers and 142 (33%) in hospitals with ≥1000 beds without heart transplant program. A low starting dose of SV (50 mg bid) was more frequent in medium/small size hospitals than in hospitals with ≥1000 beds (51 [63.0%] vs 151 [43.6%] patients, P = .008). At 7 months, SV had been withdrawn more frequently in medium/small size hospitals than in hospitals ≥1000 beds (15 [18.5%] vs 34 [9.8%] patients, P = .001], in spite of a similar rate of adverse events (23 [28.4%)] vs 99 [28.6%] patients, P = .94). Conclusion In our registry, the use of SV in real life showed a low rate of events during follow-up. The above suggests that this drug is safe and beneficial in different clinical profiles of patients but drug withdrawal is more frequent in medium/small size hospitals. Resumen Introducción y objetivos Se recomienda el sacubitrilo-valstartán (SV) en pacientes con insuficiencia cardiaca y fracción de eyección reducida.. Sin embargo, las características de los centros incluidos en los ensayos clínicos fundamentales son diferentes a las de los centros en la vida real. El objetivo del presente estudio fue analizar el perfil clínico de los pacientes tratados con SV según el tipo de hospital y el papel potencial de estas diferencias en la seguridad y la efectividad de este fármaco. Métodos Registro multicéntrico prospectivo que incluyó a pacientes ambulatorios tratados con SV. Se compararon las características basales y los tratamientos, la dosificación del fármaco y los datos a los 7 meses de seguimiento. Resultados De 427 pacientes, 81 (19,0%) fueron incluidos en hospitales de tamaño medio/pequeño, 204 (48%) en centros con programa de trasplante cardiaco y 142 (33%) en hospitales con ≥ 1.000 camas sin trasplante cardíaco. Una dosis de inicio baja de SV fue más frecuente en hospitales de tamaño medio/pequeño que en hospitales con ≥ 1.000 camas (51 [63,0%] frente a 151 [43,6%] pacientes, p = 0,008). A los 7 meses, el SV se había retirado con mayor frecuencia en hospitales de tamaño medio/pequeño que en hospitales ≥ 1.000 camas (15 [18,5%] frente a 34 [9,8%] pacientes, p = 0,001], a pesar de una tasa similar de eventos adversos (23 [28,4%)] frente a 99 [28,6%] pacientes, p = 0,94). Conclusión En nuestro estudio, el uso de SV en la vida real mostró una tasa baja de eventos en el seguimiento. Esto sugiere que este fármaco es seguro y beneficioso en diferentes perfiles clínicos de pacientes, si bien el fármaco se suspendió con mayor frecuencia en los hospitales de tamaño medio/pequeño. Sin financiación No data JCR (2021) 0.130 SJR (2021) Q4, 317/356 Cardiology and Cardiovascular Medicine No data IDR 2021 UEM

Altres ajuts: Centro de Investigación en Red de Enfermedades Cardiovasculares (CIBERCV); Instituto de Salud Carlos III (ISCIII); Ministerio de Economía y Competitividad (MINECO). Biological differences between males and females change the course of different diseases and affect therapeutic measures' responses. Heart failure is not an exception to these differences. Women account for a minority of patients on the waiting list for heart transplantation or other advanced heart failure therapies. The reason for this under-representation is unknown. Men have a worse cardiovascular risk profile and suffer more often from ischemic heart disease. Conversely, transplanted women are younger and more frequently have non-ischemic cardiac disorders. Women's poorer survival on the waiting list for heart transplantation has been previously described, but this trend has been corrected in recent years. The use of ventricular assist devices in women is progressively increasing, with comparable results than in men. The indication rate for a heart transplant in women (number of women on the waiting list for millions of habitants) has remained unchanged over the past 25 years. Long-term results of heart transplants are equal for both men and women. We have analyzed the data of a national registry of heart transplant patients to look for possible future directions for a more in-depth study of sex differences in this area. We have analyzed 1-year outcomes of heart transplant recipients. We found similar results in men and women and no sex-related interactions with any of the factors related to survival or differences in death causes between men and women. We should keep trying to approach sex differences in prospective studies to confirm if they deserve a different approach, which is not supported by current evidence.