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Truncus arteriosus is a congenital heart disease in which a great artery, that is balanced positioned above a large ventricular septal defect, gives origin to the ascending aorta, the pulmonary arteries and the coronary circulation. The atrioventricular septum, both ventricles and the left ventricular outflow tract are usually normal. In most cases the common trunk gives rise to the main pulmonary artery which then bifurcates into branch pulmonary arteries generally of normal size. Truncal valve is usually dysplastic with thickened and deformed leaflets and in order from highest to lowest frequency it may be trileaflet, quadricuspid or bicuspid. The absence of one pulmonary artery is rare (1).

Resumen Introduccion y objetivos El recambio valvular pulmonar (RVP) es actualmente la intervencion mas frecuente de las cardiopatias congenitas del adulto. Existen numerosas controversias sobre cuando implantar una protesis y; sobre todo, acerca de cual es el sustituto ideal. El objetivo del estudio es revisar el resultado de 3 tipos de protesis biologicas implantadas en nuestro centro. Metodos Desde 2003 se implantaron 3 tipos de protesis biologicas en diferentes momentos: grupo 1-protesis Medtronic Freestyle (n = 14); grupo 2-protesis Sorin Soprano (n = 24), y grupo 3-protesis Carpentier Edwards (n = 15). Se analizaron datos clinicos, ecocardiograficos y de seguimiento, tanto pre como postoperatorios. Resultados El RVP se asocio a una mejoria de la New York Heart Association y a un aumento de la fraccion eyeccion ventriculo izquierdo postoperatoria en nuestra serie. Las caracteristicas preoperatorias fueron similares entre grupos. El grupo 1 presento un gradiente mayor inicial (p = 0,053), aunque estable en el tiempo. El grupo 2 se asocio a una mayor tasa de disfunciones protesicas tardias (p = 0,004). La degeneracion protesica global de la serie fue de 20 pacientes con 5 reintervenciones. No hubo mortalidad precoz y durante el seguimiento fallecieron 4 pacientes, 1 por causa cardiaca. T medio ± desviacion estandar de seguimiento global: 49,98 ± 42,79 meses. Conclusiones El RVP en nuestra serie se ha asociado a una baja tasa de mortalidad perioperatoria. Cuando comparamos segun el tipo de protesis, la protesis Medtronic Freestyle presento un mayor gradiente inicial y en el grupo Sorin Soprano se observa una degeneracion protesica superior a la esperada. Sin embargo, sera necesario mas seguimiento para definir el comportamiento de la protesis Carpentier Edwards.

Resumen: Introducción: El cierre quirúrgico del ductus arterioso persistente en el prematuro es una técnica agresiva y no exenta de complicaciones. Diseñamos un estudio con el objetivo de describir nuestra experiencia con una técnica menos invasiva, el abordaje extrapleural vía minitoracotomía posterior, y de comparar sus resultados con los del abordaje clásico transpleural. Pacientes y métodos: Estudio de cohortes retrospectivo de los neonatos prematuros a los que se les realizó cierre quirúrgico del ductus en un periodo de 10 años (marzo de 2005-marzo de 2015). Se compararon las complicaciones agudas, los resultados al alta y en el seguimiento entre los grupos de abordaje extrapleural y abordaje clásico transpleural. Se incluyó a 48 pacientes, 30 en el grupo de abordaje clásico y 18 en el grupo extrapleural. Resultados: Las características demográficas y preoperatorias fueron similares en ambos grupos. No se encontraron diferencias entre los 2 grupos en la incidencia de complicaciones postoperatorias agudas (56,6 vs. 44,4%), en la dependencia de oxígeno a las 36 semanas (33,3 vs. 55,5%), ni en la mortalidad hospitalaria (10 vs. 16,6%). En la evolución a corto plazo, el grupo extrapleural precisó menos días hasta la retirada del oxígeno suplementario (36,3 vs. 28,9) y hasta el alta hospitalaria (67,5 vs. 53,2), aunque solo el tiempo hasta la extubación alcanzó una diferencia estadísticamente significativa (11,5 vs. 2,7, p = 0,03). Conclusiones: El abordaje extrapleural por minitoracotomía posterior para el cierre quirúrgico del ductus en el prematuro es factible y podría conllevar algunos beneficios clínicos a corto plazo. Abstract: Introduction: Surgical closure of patent ductus arteriosus in premature neonates is an aggressive technique and is not free of complications. A study was designed with the aim of describing our experience with a less invasive technique, the extra-pleural approach via a posterior minithoracotomy, and to compare the results with the classic transpleural approach. Patients and methods: A retrospective cohort study was conducted on premature neonates on whom surgical closure of the ductus was performed during a ten-year period (March 2005 to March 2015). A comparison was made of the acute complications, the outcomes on discharge, and follow-up, between the extra-pleural approach and the classic transpleural approach. The study included 48 patients, 30 in the classical approach and 18 in the extra-pleural group. Results: The demographic and pre-operative characteristics were similar in both groups. No differences were found between the 2 groups in the incidence of acute post-operative complications (56.6 vs. 44.4%), on the dependence on oxygen at 36 weeks (33.3 vs. 55.5%), or in hospital mortality (10 vs. 16.6%). As regards the short-term progress, the extra-pleural group required fewer days until the withdrawal of supplementary oxygen (36.3 vs. 28.9) and until hospital discharge (67.5 vs. 53.2), although only the time until extubation achieved a statistically significant difference (11.5 vs. 2.7, P=.03). Conclusions: The extra-plural approach by posterior minithoracotomy for the surgical closure of ductus in the premature infant is viable and could bring some clinical benefits in the short-term.

We report a case of a 12-yr-old boy referred to our unit with congenital generalized lipodystrophy and dilated cardiomyopathy related to a lamin gene mutation. He progressively developed end-stage heart failure and was referred for heart transplant evaluation. The patient's lipid profile, glucose level, and renal function were normal, and vascular retinopathy was ruled out. He underwent orthotopic bicaval HT and had an uneventful recovery. He was discharged home two wk after surgery with good graft function. During follow-up, he developed hyperglycemia and dyslipidemia, which were controlled by increasing leptin dose and starting oral antidiabetic drugs. The patient is currently doing well two yr after transplantation.

Objetivo: Determinar la incidencia de deterioro valvular estructural (DVE) entre todos los modelos de bioprótesis aórtica Mitroflow (A12/LX/DL), así como definir los factores de riesgo implicados y el posible papel protector del tratamiento anticalcificación phospholipid reduction treatment (PRT) del modelo DL. Métodos: Estudio retrospectivo de 1.023 pacientes consecutivos operados de recambio valvular aórtico con bioprótesis Mitroflow entre 2001 y 2014. Resultados: El seguimiento medio fue de 5,4 ± 3,1 años. La incidencia acumulada global de DVE fue 0,48% a un año, 2,1% a 5 años y 6,8% a 10 años. El grupo PRT mostró menor incidencia acumulada de DVE (0,2% vs. 1,2% a 4 años) (p = 0,04). El análisis de regresión de riesgos competitivos confirmó: edad < 70 años (HR = 2,98 [1,5-5,89], p = 0,002), tamaño valvular de 19 mm (HR = 4,7 [2,29-9,64], p 30 mmHg en postoperatorio precoz (HR = 10,3 [5,3-19,9], p

Resumen: Introducción: Existe una creciente preocupación por las complicaciones neurológicas asociadas a las cardiopatías congénitas y a la cirugía cardiaca infantil. Material y métodos: Estudio observacional retrospectivo de casos y controles de las cirugías cardiacas infantiles y su postoperatorio en cuidados intensivos durante un período de 10 años. Se seleccionaron dos controles por cada caso, ajustados por el mismo grado de complejidad quirúrgica. Resultados: Se estudiaron 900 cirugías. Se detectaron 38 complicaciones neurológicas (4,2%), de las cuales 21 (55,3%) implicaban al sistema nervioso periférico y 17 (44,7%) al sistema nervioso central. Las complicaciones del sistema nervioso central (1,9% del total) fueron 8 convulsiones, 4 accidentes cerebrovasculares, 4 encefalopatías hipóxico-isquémicas y un déficit neurológico reversible. Un 35,3% mostró afectación al alta y un 17,6% falleció. Se encontró una diferencia estadísticamente significativa en el tiempo de circulación extracorpórea (p = 0,009), el tiempo de isquemia (p = 0,12), los días de estancia en cuidados intensivos (p = 0,001), días de ventilación mecánica (p = 0,004) y días de soporte inotrópico (p = 0,001). Conclusiones: La incidencia de complicaciones neurológicas en nuestra serie es similar a la descrita previamente. Las convulsiones son la manifestación clínica más común. Las complicaciones del sistema nervioso central se asocian con un aumento de la morbilidad, una mayor estancia hospitalaria y un mayor consumo de recursos. Es necesario establecer medidas en el pre y post-operatorio, así como durante la cirugía, encaminadas a su prevención y diagnóstico precoz. Abstract: Introduction: There has been an increasing concern over the neurological complications associated with congenital heart disease and cardiac surgery. Material and methods: We performed a retrospective, case-control, observational review of the postoperative period in the intensive care unit of patients undergoing cardiac surgery over the past 10 years. We selected 2 control patients for each case, matched for surgical complexity. Results: A total of 900 patients were reviewed. We found 38 neurological complications (4.2%), of which 21 (55.3%) were in the peripheral nervous system and 17 (44.7%) in the central nervous system. The complications involving the central nervous system (1.9% of total) consisted of 8 seizures, 4 cerebrovascular accidents, 4 hypoxic-ischemic encephalopathy events, and 1 reversible neurological deficit. At the time of discharge, 35.3% were symptomatic and 17.6% had died. Patients with neurological complications had a longer bypass time (P=.009), longer aortic cross time (P=.012), longer hospitalization in intensive care (P=.001), longer duration of mechanical ventilation (P=.004) and an increased number of days under inotropic support (P=.001). Conclusions: Our incidence of neurological complications after cardiac surgery is similar to that previously described. Clinical seizures are the most common complication. Central nervous system complications are associated with a higher morbidity and hospitalization time. Units caring for patients with congenital heart disease must implement neurological monitoring during and after cardiac surgery to prevent and to detect these complications earlier.

ResuménLa cirugía cardiovascular (CCV) es una especialidad de la medicina que se ocupa de los trastornos y enfermedades del sistema cardiocirculatorio que requieren una terapéutica quirúrgica. El presente documento está dedicado a los aspectos definitorios de la especialidad y a los organizativos de la actividad profesional incluyendo la acreditación y homologación. se define asimismo la sociedad profesional, la sociedad Española de Cirugía Torácica-Cardiovascular (SECTCV) en sus aspectos relacionados con la profesión, con la formación continuada así como la comunicación en el seno y fuera de la misma al igual que la necesidad y existencia de la fundación Cirugía y Corazón (FCC). También se focaliza en la actividad en CCV y los requerimientos estructurales mínimos para llevar a cabo el componente de intervención de la CCV. Por último, se confirman las recomendaciones para su práctica y se presentan los requerimientos de calidad y del programa docente de la especialidad. Este documento de la junta directiva de la SECTCV y asesores externos de la misma confirma la realidad de la especialidad y sus contenidos como señas de identidad inalienables.AbstractCardiovascular surgery (CVS) is a branch of medicine related to the diseases of the cardio-circulatory system requiring surgical treatment. this current document is dedicated to all aspects defining the specialty and the organization of the professional activity including accreditation and credentialing. The professional society, the spanish society of thoracic-Cardiovascular surgery (SECTCV) is also defined and insights on its professional, continuing education and communication given as well as the background for the Cirugía y Corazón foundation. Attention is also given to the activity in CVS and the core requirements for practice. The recommendations for practice and quality requirements and the postgraduate teaching programme are also presented. This document of the Council of the SECTCV and its external advisors confirms the reality of the specialty and its contents as sound signs of identity.

La cirugia de reparacion valvular mitral con neocuerdas de politetrafluoroetileno (PTFE) se ha mostrado en los ultimos anos como una tecnica eficaz en la correccion del prolapso de los velos mitrales debido a rotura o elongacion de cuerdas tendinosas. En el presente estudio hemos analizado la experiencia inicial con esta tecnica en nuestro centro, con un total de 21 pacientes. La mediana (intervalo) de seguimiento fue de 9 (1-26) meses. No se produjo mortalidad hospitalaria. En el seguimiento se evidencio mejoria clinica (el 95,2% de los pacientes en clase funcional NYHA ≤ II) y ecocardiografica (el 85,7% de los pacientes con area de regurgitacion mitral

Introduction: Surgical closure of patent ductus arteriosus in premature neonates is an aggressive technique and is not free of complications. A study was designed with the aim of describing our experience with a less invasive technique, the extra-pleural approach via a posterior minithoracotomy, and to compare the results with the classic transpleural approach. Patients and methods: A retrospective cohort study was conducted on premature neonates on whom surgical closure of the ductus was performed during a ten-year period (March 2005 to March 2015). A comparison was made of the acute complications, the outcomes on discharge, and follow-up, between the extra-pleural approach and the classic transpleural approach. The study included 48 patients, 30 in the classical approach and 18 in the extra-pleural group. Results: The demographic and pre-operative characteristics were similar in both groups. No differences were found between the 2 groups in the incidence of acute post-operative complications (56.6 vs. 44.4%), on the dependence on oxygen at 36 weeks (33.3 vs. 55.5%), or in hospital mortality (10 vs. 16.6%). As regards the short-term progress, the extra-pleural group required fewer days until the withdrawal of supplementary oxygen (36.3 vs. 28.9) and until hospital discharge (67.5 vs. 53.2), although only the time until extubation achieved a statistically significant difference (11.5 vs. 2.7, p = .03). Conclusions: The extra-plural approach by posterior minithoracotomy for the surgical closure of ductus in the premature infant is viable and could bring some clinical benefits in the short-term. Resumen: Introducción: El cierre quirúrgico del ductus arterioso persistente en el prematuro es una técnica agresiva y no exenta de complicaciones. Diseñamos un estudio con el objetivo de describir nuestra experiencia con una técnica menos invasiva, el abordaje extrapleural vía minitoracotomía posterior, y de comparar sus resultados con los del abordaje clásico transpleural. Pacientes y métodos: Estudio de cohortes retrospectivo de los neonatos prematuros a los que se les realizó cierre quirúrgico del ductus en un periodo de 10 años (marzo de 2005-marzo de 2015). Se compararon las complicaciones agudas, los resultados al alta y en el seguimiento entre los grupos de abordaje extrapleural y abordaje clásico transpleural. Se incluyó a 48 pacientes, 30 en el grupo de abordaje clásico y 18 en el grupo extrapleural. Resultados: Las características demográficas y preoperatorias fueron similares en ambos grupos. No se encontraron diferencias entre los 2 grupos en la incidencia de complicaciones postoperatorias agudas (56,6 vs. 44,4%), en la dependencia de oxígeno a las 36 semanas (33,3 vs. 55,5%), ni en la mortalidad hospitalaria (10 vs. 16,6%). En la evolución a corto plazo, el grupo extrapleural precisó menos días hasta la retirada del oxígeno suplementario (36,3 vs. 28,9) y hasta el alta hospitalaria (67,5 vs. 53,2), aunque solo el tiempo hasta la extubación alcanzó una diferencia estadísticamente significativa (11,5 vs. 2,7, p = 0,03). Conclusiones: El abordaje extrapleural por minitoracotomía posterior para el cierre quirúrgico del ductus en el prematuro es factible y podría conllevar algunos beneficios clínicos a corto plazo.

OBJECTIVES Lower mini-sternotomy represents a minimally invasive surgical technique that has been utilized for the repair of a wide variety of congenital heart defects with excellent surgical and cosmetic outcomes. However, clinical improvements provided for this technique beyond cosmetic results are controversial. The aim of our study is to report our results with lower mini-sternotomy for the repair of congenital heart malformations and compare them with a matched group with a full median sternotomy approach. METHODS From 2010 through 2013, 105 consecutive congenital patients (81 paediatric) underwent lower mini-sternotomy at our centre (Group 1). We analysed in-hospital and follow-up outcomes, and compare them with an age-sex-diagnosis-type of surgery-matched group (Group 2). Both groups were managed following the same clinical protocols. RESULTS In Group 1, age at the time of surgery was 12 ± 17 years (range from 0.2 to 64.6 years). In this group, 81 patients were paediatric and 62 were female. Operative techniques were atrial septal defect (n = 72), ventricular septal defect (n = 24) and atrioventricular canal repairs (n = 9). There were no deaths or major in-hospital complications. Two adult patients required conversion to full median sternotomy. For a medium follow-up of 1.5 years (range from 1 month to 5 years), there were no deaths, reinterventions or reoperations and no significant residual defects were found. Compared with Group 2, patients in Group 1 had longer cardiopulmonary bypass times (58.71 ± 19.08 vs 45.39 ± 20.45, P < 0.001) and cross-clamp times (32.75 ± 13.11 vs 23.22 ± 13.93, P < 0.001), higher rate of early extubation (96 vs 85%, P = 0.018) and lower rate of postoperative complications (11.6 vs 22.3%, P = 0.034). CONCLUSIONS At our centre, lower mini-sternotomy represents a safe alternative for the repair of congenital heart defects in paediatric and adult populations. Cardiopulmonary bypass and cross-clamp times were longer in the mini-sternotomy group. However, these patients showed earlier extubation and less postoperative complications when compared with patients with a full sternotomy approach. Combined with improved cosmetic outcomes, lower mini-sternotomy could represent the technique of choice for these populations.

Junctional ectopic tachycardia (JET) is a complication of the repair of congenital cardiac malformations that responds poorly to conventional treatment. We report our experience with the use of moderate hypothermia in its management. Twelve infants with postoperative JET treated with hypothermia were reviewed. The mean interval between the diagnosis of JET and initiation of hypothermia was 1.5 ± 0.5 hours. In the first 24 hours of hypothermia, central temperature and heart rate decreased significantly. Arterial pressure and diuresis tended to increase and central venous pressure tended to decrease. No direct adverse events occurred. All the patients but one survived and are alive and free of neurological deficits after 15 ± 12 months.

In recent years, mitral valve repair using polytetrafluoroethylene (PTFE) neochordae has been shown to be an effective technique for correcting valve prolapse due to chordal rupture or elongation. In this study, we evaluated our experience using this technique in a group of 21 patients. The median (range) follow-up period was 9 (1-26) months. There was no in-hospital mortality. During follow-up, improvement in both clinical and echocardiographic parameters were observed: 95.2% of patients were in I-II NYHA class and the mitral regurgitation jet area was less than 4 cm 2 in 85.7% of patients. These findings are consistent with those previously reported in the literature. However, these results relate to our initial experience, and long-term follow-up is still required.

Junctional ectopic tachycardia (JET) is a complication of the repair of congenital cardiac malformations that responds poorly to conventional treatment. We report our experience with the use of moderate hypothermia in its management. Twelve infants with postoperative JET treated with hypothermia were reviewed. The mean interval between the diagnosis of JET and initiation of hypothermia was 1.5 0.5 hours. In the first 24 hours of hypothermia, central temperature and heart rate decreased significantly. Arterial pressure and diuresis tended to increase and central venous pressure tended to decrease. No direct adverse events occurred. All the patients but one survived and are alive and free of neurological deficits after 15 12 months.

A 53-year-old man was admitted to our institution because of palpitations and epigastric pain. He had a history of moderate mitral regurgitation secondary to a prolapse of the posterior leaflet diagnosed 6 months before in a routine echocardiographic examination during the study of a cardiac murmur. Eight hours after admission he experienced acute onset of dyspnoea with signs of heart failure on physical examination. Within a few …

ResumenEn este capítulo se realizará una revisión general de la embriología y la anatomía quirúrgica de las cardiopatías congénitas, ofreciendo un pequeño esbozo del paralelismo natural de la evolución del aparato circulatorio y ofreciendo finalmente un análisis de los sistemas más empleados en la descripción de esta patología.AbstractA systematic review will be done in this chapter about embryology and surgical anatomy in congenital heart disease. The circulatory system evolution will be compared with some aspects in this field. Finally, a segmental analisys will be shown to describe different heart malformations.

ResumenEn este capítulo se realizará una revisión general de la regurgitación aórtica como parte del capítulo de lesiones del tracto de salida del ventrículo izquierdo, incidiendo de manera especial en las técnicas reparadoras como primera elección.AbstractA systematic review will be done in this chapter about aortic regurgitation as a part of the left ventricular outflow tract pathology chapter, focusing in reparative techniques as first line strategy