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Resumen: El trasplante en cardiopatías congénitas supone alrededor de un 10% del total. Aunque básicamente se trata de un procedimiento similar al de un trasplante convencional en un adulto, incluye peculiaridades técnicas propias de la cirugía infantil. Indicaciones, tamaños y estrategias son más variados. Presentamos un repaso donde detallamos aspectos específicos, con algoritmos de decisión y esquemas que facilitan la evaluación de los pacientes. Finalmente, apuntamos las tendencias previsibles de aumento de re-intervenciones, así como trasplantes en menores de un año y congénitos adultos. Abstract: Congenital heart disease accounts for 10% of overall transplants. Unlike in adults procedures, childreńs transplants may become a real challenge. Indications, sizes and strategies are wide enough. This paper gathers several tips, decisión-making trees and simple drawings which enable patients handling. The authors foresee an increase in re-do procedures, as well as a rise in infants and grown-up transplant candidates in the future. Palabras clave: Trasplante, Congénitos, Asistencia circulatoria, Univentricular, Keywords: Transplant, Congenital, Assist-device, Single-ventricle

Neonatal cardiac lupus is a rare, passively acquired autoimmune disease. We report a case of in utero myocarditis, confirmed postnatally, with papillary muscle rupture and severe tricuspid regurgitation after birth in the absence of conduction disturbances. Tricuspid repair was successfully performed with polytetrafluoroethylene neochordae. In this article, we discuss the pathophysiology, medical and surgical management, and implications at follow-up in this unique scenario.

Introducción y objetivos: El trasplante cardíaco pediátrico constituye un reto multidisciplinar y son escasas las series amplias a nivel mundial. Presentamos evolución, tendencias y resultados de nuestra serie y perspectivas futuras. Material y métodos: Desde inicio del programa (1990) hasta 2015, se han realizado 163 trasplantes (158 pacientes): 50,3% (n = 82) cardiopatías congénitas (80,5% [n = 66] univentriculares); 33,7% (n = 55) miocardiopatía dilatada; 4,3% (n = 7) miocardiopatía hipertrófica; 3,6% (n = 5) retrasplante, y 7,9% (n = 14) otras causas. La edad media fue 5,1 ± 3,2 años (rango 11 días-17 años) y peso medio 17,2 ± 3 kg (rango 2,8-70). Se realizaron análisis descriptivo, comparativo (períodos históricos) y análisis multivariante y de seguimiento (Software-R-Core-Team-2013). Resultados: A partir del 2003, se incrementa el número global de trasplantes cardíacos, convirtiéndose la fisiología univentricular en estadio Glenn (n = 14) y Fontan (n = 10) la cardiopatía congénita más prevalente, precisando reconstrucciones complejas preimplante. La mortalidad global a 30 días fue del 20,2% (n = 33), descendiendo en el segundo período (14,4% vs. 30,5%, p

ResumenEl drenaje venoso pulmonar anómalo parcial es una cardiopatía infrecuente especialmente cuando afecta a las venas pulmonares izquierdas. El abordaje quirúrgico más utilizado en cirugía cardiaca es vía esternotomía media y circulación extracorpórea. Pero existen ciertas patologías en las que puede ser más recomendable otro tipo de acceso (toracotomía) y no siempre es preciso el uso de la circulación extracorpórea. Presentamos un caso de un paciente de 3 años con drenaje venoso pulmonar anómalo izquierdo que drena a una vena vertical que desemboca en la vena innominada. Para su reparación realizamos un abordaje a través de una toracotomía posterolateral y realizamos el procedimiento sin circulación extracorpórea.El propósito de esta publicación es contribuir a la difusión de esta técnica.AbstractPartial anomalous pulmonary venous drainage is a rare condition, especially that of left pulmonary veins. Median sternotomy and cardiopulmonary bypass is the most common surgical approach in cardiac surgery, although there are certain conditions in which a different approach (thoracotomy) would be more advisable, and where it is not always necessary to perform a cardiopulmonary bypass. The case is presented of a 3 year-old child with a left-sided partial anomalous pulmonary venous drainage with a vertical vein connecting the superior left pulmonary vein to the innominate vein. An off-pump repair through a left thoracotomy was chosen. The purpose of this publication is to present this technique.

Resumen: Introducción: Los abordajes miniinvasivos en cirugía cardiaca infantil no son habituales. Pocos grupos presentan programas y experiencia, quedando limitados a cardiopatías simples. Presentamos una casuística recopilada a lo largo de 15 años y más de 200 pacientes. Material y métodos: En el año 2000 comenzamos un programa de acceso submamario, ampliándolo progresivamente a casos complejos y edades menores. En 2009 incorporamos el abordaje axilar, con idénticas pautas. En 2013 iniciamos la cirugía por miniesternotomía inferior, ampliando la cartera de servicios. Entre julio del 2000 y diciembre del 2014 se intervino a 203 pacientes: 102 por vía submamaria, 50 por axilar, 44 por miniesternotomía inferior, 4 por toracotomía lateral-posterior y 3 por miniesternotomía superior. Resultados: Por patologías, la más frecuente fue la comunicación interauricular ostium secundum (128), seguida del seno venoso (20), comunicación interventricular (20), ostium primum (16) y otras (19). Un caso fue reconvertido a esternotomía. No hubo eventos neurológicos. Las medias de edad fueron 7,8/3,7 y 1,8 años, con medias de peso de 28,1/16,1 y 9,4 kg en los accesos submamario, axilar y miniesternotomía, respectivamente. Los resultados estéticos han sido excelentes. Conclusiones: Tras 15 años de experiencia acumulada, la cirugía miniinvasiva es segura y eficaz, con resultados estéticos excelentes. La introducción gradual de diferentes accesos alternativos permite establecer pautas de aprendizaje. La versatilidad de accesos (submamario, axilar, miniesternotomía) facilita la selección de abordajes en función de cardiopatía y edad/peso. Abstract: Introduction: The minimally invasive approach is seldom reported in paediatric cardiac surgery. Teams gathering experience are scarce, with programs focused on simple cases. The experience is presented on a series of over 200 cases operated on in the past 15 years. Material and methods: A sub-mammary approach program was started in 2000, which was gradually extended to include more complex and younger patients. The axillary incision was adopted in 2009, following the same steps. In 2013, the mini-sternotomy incision was introduced, increasing our armamentarium. From July 2000 until December 2014, 203 patients were operated on. The sub-mammary approach was used in 102 cases, axillary in 50 patients, mini-sternotomy in 44, postero-lateral thoracotomy in 4 cases, and upper mini-sternotomy in 3. Results: By diagnosis, ostium secundum atrial septal defect was the most common (128), followed by sinus venosus (20), ventricular septal defect (20), ostium primum (16), and others (19). One patient was converted to sternotomy. No neurological events were detected. The mean age was 7.8/3.7 and 1.8 years, and the mean weight was 28.1/16.1 and 9.4 Kg. in the sub-mammary, axillary and mini-sternotomy approaches, respectively. The aesthetic results were excellent. Conclusions: Based on our 15 years of experience, minimally invasive surgery is safe and yields excellent cosmetic results. The gradual introduction of alternative approaches (sub-mammary, axillary, mini-sternotomy) allowed us to set-up guidelines and learning curves. The wide range of incisions enables the most appropriate one to be selected depending on age/weight and cardiac condition.

Introduction: The minimally invasive approach is seldom reported in paediatric cardiac surgery. Teams gathering experience are scarce, with programmes focused on simple cases. The experience is presented on a series of over 200 cases operated on in the past 15 years. Material and methods: A sub-mammary approach programme was started in 2000, which was gradually extended to include more complex and younger patients. The axillary incision was adopted in 2009, following the same steps. In 2013, the mini-sternotomy incision was introduced, increasing our armamentarium. From July 2000 until December 2014, 203 patients were operated on. The sub-mammary approach was used in 102 cases, axillary in 50 patients, mini-sternotomy in 44, postero-lateral thoracotomy in 4 cases, and upper mini-sternotomy in 3. Results: By diagnosis, ostium secundum atrial septal defect was the most common (128), followed by sinus venosus (20), ventricular septal defect (20), ostium primum (16), and others (19). One patient was converted to sternotomy. No neurological events were detected. The mean age was 7.8/3.7 and 1.8 years, and the mean weight was 28.1/16.1 and 9.4 kg, in the sub-mammary, axillary and mini-sternotomy approaches, respectively. The aesthetic results were excellent. Conclusions: Based on our 15 years of experience, minimally invasive surgery is safe and yields excellent cosmetic results. The gradual introduction of alternative approaches (sub-mammary, axillary, mini-sternotomy) allowed us to set-up guidelines and learning curves. The wide range of incisions enables the most appropriate one to be selected depending on age/weight and cardiac condition. Resumen: Introducción: Los abordajes miniinvasivos en cirugía cardiaca infantil no son habituales. Pocos grupos presentan programas y experiencia, quedando limitados a cardiopatías simples. Presentamos una casuística recopilada a lo largo de 15 años y más de 200 pacientes. Material y métodos: En el año 2000 comenzamos un programa de acceso submamario, ampliándolo progresivamente a casos complejos y edades menores. En 2009 incorporamos el abordaje axilar, con idénticas pautas. En 2013 iniciamos la cirugía por miniesternotomía inferior, ampliando la cartera de servicios. Entre julio del 2000 y diciembre del 2014 se intervino a 203 pacientes: 102 por vía submamaria, 50 por axilar, 44 por miniesternotomía inferior, 4 por toracotomía lateral-posterior y 3 por miniesternotomía superior. Resultados: Por patologías, la más frecuente fue la comunicación interauricular ostium secundum (128), seguida del seno venoso (20), comunicación interventricular (20), ostium primum (16) y otras (19). Un caso fue reconvertido a esternotomía. No hubo eventos neurológicos. Las medias de edad fueron 7,8/3,7 y 1,8 años, con medias de peso de 28,1/16,1 y 9,4 kg en los accesos submamario, axilar y miniesternotomía, respectivamente. Los resultados estéticos han sido excelentes. Conclusiones: Tras 15 años de experiencia acumulada, la cirugía miniinvasiva es segura y eficaz, con resultados estéticos excelentes. La introducción gradual de diferentes accesos alternativos permite establecer pautas de aprendizaje. La versatilidad de accesos (submamario, axilar, miniesternotomía) facilita la selección de abordajes en función de cardiopatía y edad/peso.

WBS is a rare disorder caused by mutations in the chromosomal sub-band 7q11.23 involving the elastin gene. The clinical features (craniofacial, developmental, and cardiovascular abnormalities) are variable. The association with cardiac anomalies is a well-recognized feature, and SVAS is the most common cardiac defect found. End-stage ischemic heart disease is unusual in this setting but when it occurs, OHT remains the final therapeutic option. This decision can be difficult to determine, and it must be tailored to the individual patient based on the clinical status and concomitant cardiovascular and multisystem lesions. To date, no cases of OHT in patients with WBS have been described. We present a 14-month-old patient with WBS who developed severe LV dysfunction secondary to ischemia following a complex staged surgery for SVAS repair. He underwent successful OHT with no post-operative complications, and at three-month follow-up, he remains asymptomatic on standard immunosuppressive therapy. This case constitutes the first demonstration that OHT may be indicated for extended survival in selected children with WBS and we discuss the basic principles for extending the indication for OHT to this scenario as well as the particularities for post-transplant care.

Objectives Aortic arch repair has been shifted from deep hypothermia plus circulatory arrest to cerebral perfusion at tepid temperatures. A step forward is a simultaneous brain-coronary perfusion, allowing beating-heart arch surgery. Methods A 'Y' cannula from the arterial line delivers oxygenated blood to brain and heart. The arch is repaired on a beating heart at 25°C. Intracardiac repair is performed after running cardioplegia through the root line. Fifty patients are classified into 3 groups: A, Norwood (8 neonates); B, aortic arch (14 children) and C, aortic arch plus intracardiac repair (28 patients). Associated anomalies in Group C are as follows: ventricular septal defect (10), arterial switch (5), atrial septal defect (4), cor triatriatum (3), aortic commissurotomy (2), comprehensive repair (2), ostium primum (1) and Yasui (1). Results The mean bypass time was 161 ± 54.44 (range 93-312) min. Mean brain-coronary perfusion was 37.26 ± 10.54 (18-60) min. Mean coronary ischaemia was 31 ± 32.40 (0-160) min. The heart was not arrested in Group B patients. Follow-up was complete for a mean of 30 (1-48) months. Four patients died in the postoperative period. Two required angioplasty for recoarctation. Conclusions Selective brain-coronary perfusion is feasible and easy to switch to conventional cardioplegia delivery. Coronary ischaemia can be notably reduced and even 0 min in isolated arch surgery.

We describe a neonate with aortic atresia and hypoplastic aorta, listed for heart transplant after extracorporeal membrane oxygenation resuscitation and ductal stenting. The donor aorta was detached from the graft, after an isolated arch reconstruction prior to the transplant itself in a routine fashion. To the best of our knowledge, this is the first reported case of neonatal arch reconstruction before transplantation performed with grafts from the same donor in a split-way strategy.

A partial left ventriculectomy (Batista operation) is extremely unusual in infants. Follow-up data are unknown. We report the findings at long-term follow-up in an infant after the Batista procedure, following reimplantation of an anomalous coronary artery. The initial approach and options for management are discussed, along with late cardiac remodeling. doi: 10.1111/jocs.12775 (J Card Surg 2016;31:556-558).

The techniques and outcomes of heart transplantation in the pediatric population continue to improve over the years, although the supply of organs remains limited. Donor-to-recipient size matching is critical, especially in neonates and small infants. We present a novel strategy for heart transplantation that includes the Lecompte maneuver because of the features of the donor allograft available in a 8-month-old patient with a cardiac fibroma. We discuss the basis principles for extending the indication of this procedure to exceptional transplantation scenarios and describe the results at long-term follow-up.

Background: Mid-line sternotomy is the commonest incision for cardiac surgery. Alternative approaches are becoming fashionable in many centres, amidst some reluctance because of learning curves and overall complexity. Our recent experience in starting a new program on minimally invasive pediatric cardiac surgery is presented. The rationale for a stepwise onset and the short-medium term results for a three-year span are displayed. Methods: A three-step schedule is planned: First, an experienced surgeon (A) starts performing simple cases. Second, new surgeons (B, C, D, E) are introduced to the minimally invasive techniques according to their own proficiency and skills. Third, the new adopters are enhanced to suggest and develop further minimally invasive approaches. Two quality markers are defined: conversion rate and complications. Results: In part one, surgeon A performs sub-mammary, axillary and lower mini-sternotomy approaches for simple cardiac defects. In part two, surgeons B, C, D and E are customly introduced to such incisions. In part three, new approaches such as upper mini-sternotomy, postero-lateral thoracotomy and video-assisted mini-thoracotomy are introduced after being suggested and developed by surgeons B, C and E, as well as an algorithm to match cardiac conditions and age/weight to a given alternative approach. The conversion rate is one out of 148 patients. Two major complications were recorded, none of them related to our alternative approach. Four minor complications linked to the new incision were registered. The minimally invasive to mid-line sternotomy ratio rose from 20% in the first year to 40% in the third year. Conclusions: Minimally invasive pediatric cardiac surgery is becoming a common procedure worldwide. Our schedule to set up a program proves beneficial. The three-step approach has been successful in our experience, allowing a tailored training for every new surgeon and enhancing the enthusiasm in developing further strategies on their own. Recording conversion-rates and complications stands for quality standards. A twofold increase in minimally invasive procedures was observed in two years. The short-medium term results after three years are excellent.

We present the first reported case of totally video-assisted thoracoscopic surgery for combined ablation and external exclusion without appendectomy of the left atrial appendage. We encourage the use of video-assisted thoracoscopic surgery for a combined procedure of epicardial radiofrequency ablation and external exclusion without appendectomy to achieve a safe and complete electrical isolation of the left atrial appendage in children with focal atrial tachycardia. In our opinion, the external exclusion of the appendage has several advantages: it presents minimal risk of bleeding (especially in the pediatric population), and the clip can be retrieved if the circumflex artery is distorted.

ResumenIntroducción y objetivosEl trasplante cardiaco tras un Fontan supone un reto quirúrgico. Presentamos a 10 pacientes, destacando las variantes técnicas en las anastomosis previas a la implantación del injerto.MétodosRecogemos 8 casos de Fontan y 2 «take-down» consecutivos durante 32 meses, con medianas de edad (9 años), peso (30 kg) e intervalo entre Fontan y trasplante de 3 años. Siete pacientes presentaban conducto extracardiaco (diámetro 18/20), 2 conexión aurículo-pulmonar y uno, túnel lateral. Tres niños desarrollaron enteropatía pierde-proteínas. Nueve «stents» fueron implantados previamente.ResultadosHubo modificaciones en las 5 anastomosis. Una vez en la aurícula izquierda (sutura en ambas aurículas del receptor), 2 en la aorta (hemiarco), 2 en vena cava superior (plastia en vena innominada), 7 veces en la arteria pulmonar (parche de aorta/pericardio donante de hilio a hilio tras retirada de «stents»), vena cava inferior en 7 ocasiones (manguito de conducto). El seguimiento fue completo con una mediana de 25 meses (rango 3-34). Un paciente falleció. Dos precisaron extracorporeal membrane oxygenation por hipertensión pulmonar. Tres pacientes requirieron cierre de colaterales en hemodinámica, incluyendo colocación de «stent» en cava superior (1) y aorta (1). Se resolvió la enteropatía pierde-proteínas en 2 pacientes.ConclusionesTrasplantar a pacientes con Fontan previo es un reto. Podemos anticipar variantes en cada una de las 5 anastomosis previstas. Es recomendable obtener tejido extra del donante (aorta y arco, vena cava superior e innominada, pericardio). Los resultados pueden superponerse a otras series de trasplante cardiaco.AbstractIntrodcution and objectivesHeart transplantation after a Fontan procedure poses a unique challenge from a surgical point of view. The cases of ten patients are presented, stressing the technical variations performed in the five anastomoses in order to match the graft in the recipient.MethodsEight Fontan and two takedown patients were included over 32 consecutive months. The median age was 9 years, median weight was 30 Kg, with a median time interval of 3 years between Fontan and transplant. An extra-cardiac conduit (size 18/20) was implanted in seven patients, whereas an atriopulmonary connection was found in two, and lateral tunnel in one. Three patients developed protein-losing enteropathy. Nine stents were previously implanted.ResultsThe five anastomoses underwent some changes. Left atrium once (enlargement with recipient both atria), aorta twice (hemi-arch repair), superior vena cava twice (innominate donor plasty), pulmonary branches seven times (hilum to hilum plasty with donor aorta/pericardium patch after complete stent removal), and inferior vena cava seven times (conduit sleeve anastomoses). The patients were followed-up for a median of 25 months (range 5-34). One patient died. Extracorporeal membrane oxygenation was needed in two cases due to pulmonary hypertension. Three patients had collateral vessels occluded in the catheter laboratory, and stents placed in superior vena cava (1) and aorta (1). The protein-losing enteropathy was resolved in two children.ConclusionsTransplant in Fontan patients is currently challenging. Variations in each of the five proposed anastomoses must be anticipated. Extra tissue from the donor (innominate vein, aortic arch, pericardium) is strongly advisable. Overall results can match other transplants cohorts.