Significant heterogeneity in the diagnosis and long-term management of Wilson disease: Results from a large multicenter Spanish study.

There is uncertainty regarding Wilson's disease (WD) management. OBJECTIVES: To assess, in a multicenter Spanish retrospective cohort study, whether the approach to WD is homogeneous among centers. METHODS: Data on WD patients followed at 32 Spanish hospitals were collected. RESULTS: 153 cases, 58% men, 20.6 years at diagnosis, 69.1% hepatic presentation, were followed for 15.5 years. Discordant results in non-invasive laboratory parameters were present in 39.8%. Intrahepatic copper concentration was pathologic in 82.4%. Genetic testing was only done in 56.6% with positive results in 83.9%. A definite WD diagnosis (Leipzig score ≥4) was retrospectively confirmed in 92.5% of cases. Chelating agents were standard initial therapy (75.2%) with frequent modifications (57%), particularly to maintenance zinc. Enzyme normalization was not achieved by one third, most commonly in the setting of poor compliance, lack of genetic mutations and/or presence of cardiometabolic risk factors. Although not statistically significant, there were trends for sex differences in number of diagnosed cases, age at diagnosis and biochemical response. CONCLUSIONS: Significant heterogeneity in diagnosis and management of WD patients emerges from this multicenter study that includes both small and large reference centers. The incorporation of genetic testing will likely improve diagnosis. Sex differences need to be further explored.

Cardiac surgery simulation: Advances in transvenous lead extraction.

The clinical importance of procedures for transvenous lead extraction has increased greatly because the volume of cardiac implantable electronic devices has increased. Consequently, the number of device-related complications requiring lead extraction has also been increasing. Great improvements in lead extraction techniques have occurred in recent years with the development of transvenous lead extraction tools and techniques. Experienced centres can achieve high success rates with infrequent complications. However, even in experienced hands, life-threatening complications can occur, and any physician performing these procedures must be prepared and trained for such eventualities. Moreover, because the occurrence of these stressful situations cannot be accurately predicted, training obtained in simulated environments can be decisive. We have developed a training module that simulates all the ordered steps of a transvenous lead extraction procedure and thus is able to help surgeons refine their surgical techniques and improve their performance.

Changes in frailty status after cardiac surgery. A prospective cohort study.

BACKGROUND: The concept of frailty has become increasingly familiar in cardiac surgery. Since one of the characteristics of frailty is its reversible nature, efforts are being made to preoperatively decrease the level of frailty. However, the effect that the surgery itself may have in the postoperative frailty status remains still unknown. METHODS: A prospective cohort of 137 patients aged 70 or more undergoing major cardiac surgery were recruited at three hospitals in Spain. Frailty status was assessed preoperatively and six months after surgery using the Fried scale and the Clinical Frailty Scale (CFS). We analyzed the changes in these frailty scores and the transitions between frailty status after surgery. RESULTS: Mean age of participants was 78.3 years. 47.7% were female. Prevalence of frailty was 27.5% with the Fried Scale and 11% with the CFS. Up to 50% of frail patients improved their frailty status after surgery. The distribution of frailty among the study groups at baseline and six months after surgery changed significantly, decreasing the overall burden of frailty after surgery: 27.5% vs 17.4% of frail patients with the Fried Scale and 50.5% vs 29.4% of vulnerable or frail patients with the CFS (p<0.01). CONCLUSIONS: Frailty is a dynamic condition that can be significantly reduced after cardiac surgery, suggesting that cardiac pathology is an important contributor to the preoperative patient's frailty status.

Digital onboarding in finance: a novel model and related cybersecurity risks.

Over the last few years, the financial sector has undergone a digital revolution that has had a severe impact on different related areas such as, the entities, the cybersecurity of systems, regulations and, of course, customers. SOTER project takes the challenge providing a complete set of tools to enhance the cybersecurity levels by implementing, in addition to non-technological tools, an innovative onboarding process has been implemented with the goals of increasing security, improving the user experience and integrity in the sector, and facilitating the customer entry into the digital marketplace by combining a set of breakthrough technologies. The cybersecurity research plays a crucial role in the conception and implementation of the onboarding process and for this kind of processes it has to be studied as an individual area by itself, as it is necessary to analyze the possible threats that can affect them, their origin and the solution(s) that can be taken to address them. Therefore, the SOTER project presents a fully digital onboarding process, innovative, adaptable to both current and future market needs and to possible changes in regulation, based on the most advanced technologies available today, and above all guaranteeing the cybersecurity of both entities and end users.

[Resultados a corto y medio plazo de la técnica de Florida]. / Early and medium-term results of the Florida Sleeve technique for aortic root ectasia

Introduction: Aortic root dilatation is a frequent disease affecting mostly young patients that often requires surgical repair. Surgical techniques in young patients include aortic valve-sparing procedures to avoid prosthetic valve implant. Objective: The aim of this paper is to describe the results obtained in three patients with aortic root dilatation using the Florida Sleeve technique. Methods: From November 2015 to January 2017, three patients with severe aortic regurgitation due to aortic anuloectasic were intervened applying the Florida Sleeve technique. Results: Excellent postoperative results were obtained in the three cases including freedom of aortic regurgitation and any cause re-operation during three years of follow-up. Conclusion: The Florida Sleeve technique is a safe, reproducible technique with a learning curve and lower surgical times than traditional techniques. The medium-term clinical outcomes in terms of morbidity and mortality are good.

Introducción: La dilatación de la raíz aórtica es una patología frecuente que afecta a pacientes jóvenes y su tratamiento es quirúrgico. Las técnicas quirúrgicas de elección en pacientes jóvenes son las de preservación de la válvula aórtica para evitar el implante de una prótesis valvular. Objetivo: El presente trabajo tiene como objetivo describir la técnica de Florida Sleeve y los resultados de nuestra experiencia. Método: Entre noviembre de 2015 y enero de 2017 fueron intervenidos en nuestro centro tres casos de pacientes con insuficiencia aórtica severa secundaria a anulectasia aórtica en los que se realizó la técnica de Florida Sleeve. Resultados: En los tres casos se obtuvieron excelentes resultados postoperatorios, libres de insuficiencia aórtica residual y de reoperación por cualquier causa, resultados que se mantuvieron a los tres años de seguimiento. Conclusiones: La técnica de Florida Sleeve es una técnica segura, reproducible con una curva de aprendizaje y tiempos quirúrgicos inferiores a las técnicas tradicionales. Los resultados clínicos a medio plazo en términos de morbimortalidad son buenos.

Propionibacterium acnes Pacemaker Endocarditis in a Patient With a Redundant Loop of the Ventricular Lead: PET/CT Findings.

A 59-year-old man with a pacemaker who was admitted for evaluation of fever was found to have a redundant loop of the ventricular lead and a vegetation attached to it. An FDG PET/CT revealed a focus of FDG uptake at the ventricular lead loop. Surgical extraction of the pacemaker device confirmed a Propionibacterium acnes pacemaker endocarditis. We hypothesize that the redundant loop of the ventricular lead induced a chronic mechanical stress both to the tricuspid valve and to the lead itself, facilitating the bacterial colonization of the lead.

Long-term prognosis after surgery for infective endocarditis: Distinction between predictors of early and late survival.

OBJECTIVES: Cardiac surgery is a life-saving procedure in patients diagnosed with infective endocarditis (IE). There are several validated risk scores developed to predict early-mortality; nevertheless, long-term survival has been less investigated. The aim of the present study is to analyze the impact of IE-specific risk factors for early and long-term mortality. METHODS: An observational retrospective study was conducted that included all patients who underwent surgery for IE from 2002 to 2016. Median follow-up time after surgery was 53.2 months (IQI 26.2-106.8 months). In-hospital mortality was analyzed using multiple logistic regression. Long-term survival was analyzed after one, two and five years. Cox proportional hazards regression was employed to identify risk factors related to long-term mortality. RESULTS: Of the 180 patients underwent cardiac surgery, 133 were discharged alive (in-hospital mortality was 26.11%). 6 variables were identified as independent factors associated with in-hospital mortality, most of them closely related to the severity of IE: age, multivalvular involvement, critical preoperative status, preoperative mechanical ventilation, abscess and thrombocytopenia. Long-term survival in patients discharged alive was 89.1%, 87.4% and 77.6% after one, two and five years. Long-term mortality was independent of specific IE factors and 86.51% of deaths were not related to cardiovascular or infectious diseases. CONCLUSION: Despite the high perioperative mortality rate after surgical treatment for active IE, long-term survival after hospital discharge was acceptable, regardless of the severity of the endocarditis episode. Although in-hospital survival depended mainly on several IE factors, long-term survival was not related to the severity of endocarditis baseline affection.

Which type of valve should we use in tricuspid position? Long-term comparison between mechanical and biological valves.

BACKGROUND: Nowadays, tricuspid valve replacement (TVR) is much less common than aortic or mitral valve replacement, since repair is almost always preferable. Prosthetic tricuspid valves are associated with high mortality and morbidity, mostly due to thrombotic or hemorrhagic events. Nevertheless, there is lack of evidence of which is the optimal type of valve (biological versus mechanical) in tricuspid position. METHODS: We analyzed all the patients who underwent TVR in our Institution, from 2005 to 2015. Patient baseline characteristics were recorded (such as functional class, previous cardiac surgery, right ventricular dysfunction or pulmonary hypertension), and a clinical long-term follow-up was conducted. We compared the outcomes between mechanical and biological prostheses: in-hospital mortality, long-term mortality, need for reintervention and adverse events (such as stroke or valve thrombosis). RESULTS: During the study period 120 tricuspid prosthetic valves were implanted in 111 patients. 81 of them (67.5%) were bioprostheses, and 39 (32.5%) mechanical valves. 73 patients (60.8%) had undergone a previous cardiac surgery (28.4% had previous tricuspid surgery). Most of the patients (87.1%) were in high functional class (grade III-IV of the NYHA classification), and 85% had moderate to severe pulmonary hypertension. Mean logistic EuroSCORE I was 14.80%. Only 37 cases were isolated TVR (30.6%), as most of the cases were TVR concomitant to mitral valve replacement. In-hospital mortality was 21.7%, and during the follow-up (mean follow-up of 7 years) reached 37.5%. Three mechanical tricuspid valves (7.7%) had to be replaced due to thrombosis, while 7 biological valves (8.6%) had to be replaced due to valve deterioration. The incidence of stroke was 7.5%. CONCLUSIONS: Tricuspid valve replacement is an infrequent procedure with a high incidence of perioperative morbidity and mortality. Biological or mechanical valves have similar mortality, and a reasonably low incidence of need for reintervention due to thrombosis or valve deterioration.

Long-term prognosis after surgery for infective endocarditis: Distinction between predictors of early and late survival / Pronóstico a largo plazo tras cirugía cardiaca en la endocarditis infecciosa: diferencias entre predictores de supervivencia precoz y tardía

OBJECTIVES: Cardiac surgery is a life-saving procedure in patients diagnosed with infective endocarditis (IE). There are several validated risk scores developed to predict early-mortality; nevertheless, long-term survival has been less investigated. The aim of the present study is to analyze the impact of IE-specific risk factors for early and long-term mortality. METHODS: An observational retrospective study was conducted that included all patients who underwent surgery for IE from 2002 to 2016. Median follow-up time after surgery was 53.2 months (IQI 26.2-106.8 months). In-hospital mortality was analyzed using multiple logistic regression. Long-term survival was analyzed after one, two and five years. Cox proportional hazards regression was employed to identify risk factors related to long-term mortality. RESULTS: Of the 180 patients underwent cardiac surgery, 133 were discharged alive (in-hospital mortality was 26.11%). 6 variables were identified as independent factors associated with in-hospital mortality, most of them closely related to the severity of IE: age, multivalvular involvement, critical preoperative status, preoperative mechanical ventilation, abscess and thrombocytopenia. Long-term survival in patients discharged alive was 89.1%, 87.4% and 77.6% after one, two and five years. Long-term mortality was independent of specific IE factors and 86.51% of deaths were not related to cardiovascular or infectious diseases. CONCLUSION: Despite the high perioperative mortality rate after surgical treatment for active IE, long-term survival after hospital discharge was acceptable, regardless of the severity of the endocarditis episode. Although in-hospital survival depended mainly on several IE factors, long-term survival was not related to the severity of endocarditis baseline affection

OBJETIVOS: La cirugía cardíaca es un procedimiento fundamental en pacientes diagnosticados de endocarditis infecciosa (EI). Existen varias escalas de riesgo para predecir la mortalidad temprana; sin embargo, la supervivencia a largo plazo ha sido menos estudiada. El objetivo es analizar el impacto de los factores de riesgo específicos de EI en la mortalidad temprana y a largo plazo. MÉTODOS: Estudio observacional retrospectivo que incluyó a todos los pacientes operados por EI entre 2002 y 2016. La mediana del tiempo de seguimiento fue de 53,2 meses (IQI: 26,2-106,8 meses). La mortalidad intrahospitalaria se analizó mediante regresión logística múltiple. La supervivencia se analizó a uno, 2 y 5 años. Los factores de riesgo de mortalidad tardía se analizaron mediante regresión de Cox. RESULTADOS: De los 180 pacientes operados, 133 sobrevivieron al postoperatorio inmediato (26,11% de mortalidad intrahospitalaria). Encontramos 6 factores asociados a la mortalidad hospitalaria: edad, afectación multivalvular, estado preoperatorio crítico, ventilación mecánica preoperatoria, absceso y trombopenia. La supervivencia a largo plazo fue del 89,1, 87,4 y 77,6% después de uno, 2 y 5 años. La mortalidad a largo plazo fue independiente de factores específicos de la EI, y el 86,51% no se relacionó con enfermedades cardiovasculares o infecciosas. CONCLUSIÓN: A pesar de la alta tasa de mortalidad peri-operatoria tras cirugía, la supervivencia a largo plazo fue aceptable, independientemente de la gravedad del episodio de endocarditis. Aunque la supervivencia intrahospitalaria guardó relación con factores específicos de endocarditis, y la supervivencia a largo plazo no se correlacionó con la gravedad de la afectación inicial

Significant heterogeneity in the diagnosis and long-term management of Wilson disease: Results from a large multicenter Spanish study / Elevada heterogeneidad en el diagnóstico y tratamiento a largo plazo en la enfermedad de Wilson: resultados de un estudio multicéntrico español

There is uncertainty regarding Wilson's disease (WD) management. Objectives: To assess, in a multicenter Spanish retrospective cohort study, whether the approach to WD is homogeneous among centers. Methods: Data on WD patients followed at 32 Spanish hospitals were collected. Results: 153 cases, 58% men, 20.6 years at diagnosis, 69.1% hepatic presentation, were followed for 15.5 years. Discordant results in non-invasive laboratory parameters were present in 39.8%. Intrahepatic copper concentration was pathologic in 82.4%. Genetic testing was only done in 56.6% with positive results in 83.9%. A definite WD diagnosis (Leipzig score ≥4) was retrospectively confirmed in 92.5% of cases. Chelating agents were standard initial therapy (75.2%) with frequent modifications (57%), particularly to maintenance zinc. Enzyme normalization was not achieved by one third, most commonly in the setting of poor compliance, lack of genetic mutations and/or presence of cardiometabolic risk factors. Although not statistically significant, there were trends for sex differences in number of diagnosed cases, age at diagnosis and biochemical response. Conclusions: Significant heterogeneity in diagnosis and management of WD patients emerges from this multicenter study that includes both small and large reference centers. The incorporation of genetic testing will likely improve diagnosis. Sex differences need to be further explored. (AU)

Existe incertidumbre con respecto al manejo de la enfermedad de Wilson (EW). Objetivos: Evaluar, en un estudio de cohorte retrospectivo español multicéntrico, si el abordaje de la EW es homogéneo entre los centros. Métodos: Se recogieron datos sobre pacientes con EW seguidos en 32 hospitales españoles. Resultados: Un total de 153 casos, 58% hombres, 20,6 años al diagnóstico, 69,1% presentación hepática, fueron seguidos durante 15,5 años. Se objetivaron resultados discordantes en parámetros de laboratorio no invasivos en el 39,8%. La concentración intrahepática de cobre fue patológica en el 82,4%. Las pruebas genéticas solo se realizaron en el 56,6% con resultados positivos en el 83,9%. Un diagnóstico definitivo de EW (puntuación de Leipzig ≥4) se confirmó retrospectivamente en el 92,5% de los casos. Los agentes quelantes fueron la terapia inicial estándar (75,2%) con modificaciones frecuentes (57%), particularmente hacia zinc de mantenimiento. La normalización enzimática no se logró en un tercio, más comúnmente en el contexto de un cumplimiento deficiente, ausencia de mutaciones genéticas y/o presencia de factores de riesgo cardiometabólicos. Aunque sin alcanzar significación estadística, observamos diferencias entre hombres y mujeres en el número de casos, edad en el momento del diagnóstico y la respuesta bioquímica. Conclusiones: De este estudio multicéntrico que incluye centros de referencia pequeños y grandes se desprende una heterogeneidad significativa en el diagnóstico y manejo de los pacientes con EW. La incorporación de pruebas genéticas ha mejorado el diagnóstico. Las diferencias de sexo deben explorarse más a fondo en estudios futuros. (AU)

Rotura iatrogénica de electrodos de marcapasos permanente en la realización de esternotomía media: presentación de un caso / Iatrogenic rupture of permanent pacemaker electrodes while performing a median sternotomy: a case report

RESUMEN La esternotomía media continúa siendo el gold-standart de abordaje quirúrgico en cirugía cardiaca. Aún realizada correctamente, la apertura media del esternón presenta complicaciones en el 1-5% de los casos que varían en su gravedad desde la infección de la herida quirúrgica a la lesión de estructuras intratorácicas. En el presente estudio se describe un caso de rotura de electrodos de marcapasos tras esternotomía media por retracción y fibrosis del tronco venoso braquiocefálico. Debido a la ausencia de artículos semejantes en la literatura y al extendido empleo de la esternotomía en pacientes portadores de marcapasos permanente, se pretende disminuir las lesiones asociadas mediante la publicación de un infrecuente caso de lesión iatrogénica durante la apertura esternal, con escasos precedentes en la literatura publicada hasta la fecha. MÉD.UIS. 2016;29(3):95-9.

ABSTRACT Median sternotomy is still the gold-standard surgical approach in cardiac surgery. Even correctly performed, the sternum median opening presents complications in an incidence of 1-5% of cases comprising in severity from infection of the surgical wound to the injury of intrathoracic structures. We describe a case in which the pacemaker leads were broken after median sternotomy because of the brachiocephalic vein fibrosis. Due to the absence of similar items in the literature and the widespread use of sternotomy in patients with permanent pacemakers, we intended to reduce associated injuries by posting a rare case of iatrogenic injury during sternal opening with few precedents in the literature. MÉD.UIS. 2016;29(3):95-9.

Aplicación de la metodología de la FAO/OMS/UNU para estimar necesidades de energía de la población venezolana / Application of FAO/WHO/UNU methodology for estimating the energy needs of the Venezuelan population

Este trabajo tiene como propósito determinar si la metodología propuesta por el comité de expertos FAO-OMS-UNU en 1985 para la estimación de las necesidades de energía es factible de ser aplicada para la estimación de estas necesidades en la población venezolana. Esta metodología se basa principalmente en calcular la tasa de metabolismo basal (TMB) mediante unas ecuaciones lineales que incluyen la edad, el sexo y el peso corporal y el gasto energético total, multiplicando la TMB por un factor que cubre el costo energético de incrementar el tono muscular, la actividad física y el efecto térmico de los alimentos. Cuando sea pertinente se incluyen las necesidades adicionales en caso de crecimiento y lactancia. Con esta metodología, en la población venezolana se obtuvieron los siguientes valores: 1.502 Kcal en los niños menores de 10 años; 2.430 Kcal en los varones de 10 a 17 años; 2.121 Kcal en las niñas de 10 a 17 años;2.798 Kcal en los varones de 18 años y más; 1.995 Kcal, en las mujeres de 18 años y más y un requerimiento energético promedio por persona día de 2.153 Kcal que aumenta a 2.207 Kcal, si se corrige por el contenido de fibra dietética en la dieta. Estas cifras presentan diferencias con las obtenidas anteriormente para Venezuela en 1981, siendo ahora más bajas en los menores de 10 años, en los varones de 10 años y más y en las niñas de 10 a 15 años y más elevadas en las mujeres de 16 años y más. Estos valores pueden ser revisados en la medida en que se disponga de mayor información sobre patrones nacionales de peso y talla, actividades ocupacionales y discrecionales de la población y, además, se prevé que las necesidades energéticas aumentarán en la medida que la población menor de 10 años logre un desarrollo físico más acorde con su potencial genético y la población en general aumente su actividad física