Wolff Parkinson white pattern in Danon disease: When preexcitation is not what it seems.

Danondisease is a rare genetic disorder with an X-linked dominant inheritance affecting both skeletal and cardiac muscle. Its characteristic cardiac phenotype consists on a severe, non-obstructive and concentric hypertrophic cardiomyopathy (HCM) usually associated with a Wolff-Parkinson-White (WPW) type preexcitation pattern. Whether this corresponds to the presence of an AV or another type of accessory pathways, such as fasciculoventricular pathways (FVP) remains controversial in the literature. However, we describe the case of a teenager with Danon disease and preexcitation who develops a first degree AV block without any change in his QRS morphology, fact that favors the hypothesis of the presence of a FVP. This finding has important clinical implications in the management and prognosis of these complex patients. The absence of an AV accessory pathway decreases their risk of potential SCD in the context of a fast atrial arrhythmia and their chances of having a reentrant AV tachycardia.

Echocardiographic two-dimensional view of the pulmonary valve in infants: the high transsternal view.

Neonates and small infants have unique characteristics that make it possible to obtain echocardiographic views that are inaccessible in older patients. A high transsternal approach through the cartilaginous sternum and the thymus gland allows visualisation of a short-axis view of the pulmonary valve. This view should be included as part of routine protocols for echocardiographic examinations performed in this age group.

Pulmonary vasodilator therapy and early postoperative outcome after modified Fontan operation.

Although mortality is low after the modified Fontan procedure, there is a significant percentage of patients with prolonged postoperative recovery. The objective of this study is to evaluate the usefulness of postoperative administration of oral sildenafil and inhaled nitric oxide on early postoperative outcome. A prospective interventional and comparison study with a historical cohort was conducted. Between January, 2010 and March, 2013, 16 patients received oral sildenafil during immediate modified Fontan postoperative period. Inhaled nitric oxide was also administered if the patient was kept intubated 12 hours after surgery. Early postoperative outcome was compared with a historical cohort of 32 patients on whom the modified Fontan procedure was performed between March, 2000 and December, 2009. Postoperative administration of sildenafil and nitric oxide had no influence on early postoperative outcome after the modified Fontan procedure in terms of duration of pleural effusions, mechanical ventilation time, length of stay in the ICU, and length of hospital stay.

Operación de Fontan. Estudio de los factores hemodinámicos asociados a la evolución postoperatoria / Fontan operation. Hemodynamic factors associated with postoperative outcomes

Introducción y objetivos. Los pacientes con corazón univentricular son sometidos a una serie de intervenciones paliativas que culminan en la operación de Fontan. El objetivo de este trabajo es revisar las características clínicas y hemodinámicas de un grupo de pacientes con fisiología univentricular paliados previamente con operación de Glenn bidireccional e identificar los factores de riesgo que influyen en la evolución postoperatoria de la cirugía de Fontan. Métodos. Estudio retrospectivo de 32 pacientes sometidos a cirugía de Fontan entre marzo de 2000 y diciembre de 2009. Se revisaron las características clínicas, los datos derivados del cateterismo, el tipo de cirugía y los tiempos quirúrgicos y se buscó su relación con la evolución postoperatoria. Resultados. La mortalidad hospitalaria fue del 3%. Tras una mediana [intervalo intercuartílico] de seguimiento de 44 meses [32-79], la supervivencia es del 90%. La presión media en arteria pulmonar medida en el cateterismo se relacionó con la mortalidad tardía. De las demás variables estudiadas, las que mejor se relacionaron con la evolución postoperatoria fueron los índices de Nakata y McGoon y el tiempo de circulación extracorpórea. En el 42% de los pacientes se realizó cateterismo intervencionista previo a la operación de Fontan. Conclusiones. Realizamos la operación de Fontan con muy baja mortalidad hospitalaria. El cateterismo previo a la operación de Fontan permite seleccionar a los pacientes de alto riesgo para la cirugía así como realizar procedimientos intervencionistas que podrían mejorar la evolución postoperatoria (AU)

Introduction and objectives. The Fontan operation is usually the final palliative procedure in patients with univentricular heart. The objectives of this study were, firstly, to describe the clinical and haemodynamic characteristics of a group of patients with univentricular physiology who had previously been palliated with a bidirectional Glenn procedure and, secondly, to identify risk factors that can influence postoperative outcomes after the Fontan operation. Methods. Retrospective study with 32 patients who underwent a Fontan operation between March 2000 and December 2009. Clinical characteristics, catheterization data, type and duration of surgery were revised and analyzed as predictors of postoperative outcome. Results. Hospital mortality was 3%. After a median follow-up of 44 months (interquartile range, 32-79), survival was 90%. Preoperative mean pulmonary arterial pressure (measured during catheterization) was correlated with late mortality. Of the remaining variables analyzed, the Nakata and McGoon indices, and duration of cardiopulmonary bypass showed the highest correlations with postoperative outcomes. Interventional catheterization before the Fontan operation was performed in 42% of patients. Conclusions. Hospital mortality after the Fontan operation was very low. The performance of a haemodynamic study before the Fontan operation made it possible to select high-risk patients for surgery as well as permitting the performance of interventional procedures that could improve postoperative outcome in these patients (AU)

Fontan operation. Hemodynamic factors associated with postoperative outcomes.

INTRODUCTION AND OBJECTIVES: The Fontan operation is usually the final palliative procedure in patients with univentricular heart. The objectives of this study were, firstly, to describe the clinical and haemodynamic characteristics of a group of patients with univentricular physiology who had previously been palliated with a bidirectional Glenn procedure and, secondly, to identify risk factors that can influence postoperative outcomes after the Fontan operation. METHODS: Retrospective study with 32 patients who underwent a Fontan operation between March 2000 and December 2009. Clinical characteristics, catheterization data, type and duration of surgery were revised and analyzed as predictors of postoperative outcome. RESULTS: Hospital mortality was 3%. After a median follow-up of 44 months (interquartile range, 32-79), survival was 90%. Preoperative mean pulmonary artery pressure (measured during catheterization) was correlated with late mortality. Of the remaining variables analyzed, the Nakata and McGoon indices, and duration of cardiopulmonary bypass showed the highest correlations with postoperative outcomes. Interventional catheterization before the Fontan operation was performed in 42% of patients. CONCLUSIONS: Hospital mortality after the Fontan operation was very low. The performance of a haemodynamic study before the Fontan operation made it possible to select high-risk patients for surgery as well as permitting the performance of interventional procedures that could improve postoperative outcome in these patients.

Método para la bbtención de lacturo de alta purza a partir de la depolimerizacción de poli(ácido láctico) / A methd to obtaiin high purity lactide by the depolymerization of poly(lactic acid)

Se sintetizó lacturo por medio de la policondensación de ácido láctico a 180 °C y 5,3 kPa durante 120 minutos, y una posterior depolimerización a 220 °C y 5,3 kPa durante 40 minutos usando cloruro de estaño dihidratado como catalizador. Se utilizó un sistema novedoso de reacción y separación, recolectando el lacturo sólido y dejando en fase vapor el agua y el ácido láctico, garantizando así una alta pureza del producto y evitando reacciones reversibles.

Lactide was synthesized by polycondenzation of lactic acid at 180 °C and a 5.3 kPa for 120 minutes, and a subsequent depolymerization at 220 °C and 5.3 kPa for 40 minutes using dehydrated tin chloride as catalyst. A new reaction separation system was used, collecting the lactide in solid form and leaving water and lactic acid as vapor. This guarantees a high purity product and avoids any reversible reaction of lactide.

Cirugía biliar / Biliary surgery

La revision de 751 casos nos permite concluir que la incidencia de enfermedades del árbol biliar fue del 15% de todas las internaciones en nuestro servicio y afecta a personas entre 50 y 59 años de edad. La relación mujer hombre fue de 6 a 1. La frecuencia de enfermdades producidas por litiasis es extraordinaria comparada con afecciones malignas. El método diagnóstico más utilizado fue la ecografía. Los diagnósticos más comunes fueron: colecistitis aguda y crónica. La operación más común fue la colecistectomía. El uso rutinario de la colangiografía y/o coledocoscopía intraoperatoria son propuestos para disminuir la incidencia de litiasis residual del coledoco. Nuestra mobilidad fue relativamente alta, pero la mortalidad para enfermedades benignas fue baja. Creemos que las enfermedades banignas y malignas del árbol biliar son graves y su frecuencia es bastante elevada en nuestra comunidad. El éxito obtenido fue el resultado del esfuerzo, idoneidad y equipamiento de especialistas de diferentes ramas médicas trabajando en conjunto para resolver los problemas diagnósticos y terapéuticos presentados por los pacientes. La consulta temprana, la atención médica organizada y eficiente y el excelente entrenamiento de los médicos beneficiará a la comunidad que servimos