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Resumen La coartacion de aorta primaria representa aproximadamente del 6 al 8% de los defectos cardiacos congenitos. La coartectomia es el tratamiento de eleccion en neonatos, con una morbimortalidad del 1-2%. Los neonatos tienen desarrollado un buen sistema de circulacion colateral que actua como mecanismo de proteccion frente a la isquemia medular, por lo que un pinzamiento aortico directo sin necesidad de circulacion extracorporea suele ser lo habitual en la reparacion quirurgica. Presentamos el caso de un neonato de 8 dias de vida que fue sometido a una coartectomia a traves de toracotomia lateral izquierda, se realizo un by-pass parcial izquierdo, debido a la friabilidad de los tejidos que impidio completar la anastomosis, permitiendo cambiar de estrategia quirurgica a un avance de arco a traves de una esternotomia media sin suponer un tiempo prolongado de isquemia tisular.

Resumen: Introducción: La cirugía del arco aórtico en neonatos ha pasado de la hipotermia profunda con paro circulatorio a la perfusión cerebral en 25 °C. Un paso más supone la perfusión cerebral y miocárdica simultánea, facilitando la cirugía a corazón latiendo. Métodos: Una cánula en «Y» desde la línea arterial proporciona sangre oxigenada al cerebro y al corazón. El arco es reparado latiendo. A continuación se administra cardioplejía por raíz para la corrección intracardíaca. Sesenta y ocho pacientes son distribuidos en 3 grupos: a) Norwood (9 neonatos); b) arco aórtico (20 niños); y c) arco más afectación intracardíaca (39 pacientes). Cardiopatía asociada en el grupo c): comunicación interventricular (15), switch arterial (9), comunicación interauricular (6), cor triatriatum (3), comisurotomía aórtica (2), comprehensive repair (2), ostium primum (1) y Yasui (1). Catorce de los últimos pacientes recibieron una canulación adicional en la aorta descendente, alcanzando una perfusión corporal total. Resultados: El tiempo medio de circulación extracorpórea fue de 155 minutos (63-353). La perfusión cerebral-miocárdica duró 36 minutos (18-60) y la isquemia miocárdica alcanzó 34 minutos (0-160), siendo de cero en el grupo «b». El seguimiento fue completo con una media de 52 meses (2-78). Cinco pacientes fallecieron y 3 precisaron angioplastia por recoartación. Conclusiones: La perfusión cerebral-miocárdica selectiva es factible y fácil de reconvertir a protección cardiopléjica convencional. Notablemente se reduce la isquemia miocárdica, siendo nula en casos de cirugía aislada del arco. Mediante la perfusión corporal total puede reducirse la morbilidad en cirugía neonatal del arco. Abstract: Introduction: Aortic arch repair has shifted from deep hypothermia plus circulatory arrest to cerebral perfusion at tepid temperatures. A step forward is simultaneous brain-coronary perfusion, allowing beating-heart arch surgery. Methods: A “Y” cannula from the arterial line delivers oxigenated 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. 68 patients are classified into three groups. A: Norwood (9 neonates); B: aortic arch (20 children); C: aortic arch plus intracardiac repair (39 patients). Associated anomalies in group C: ventricular septal defect (15), arterial switch (9), atrial septal defect (6), cor triatriatum (3), aortic commissurotomy (2), comprehensive repair (2), ostium primum (1), Yasui (1). Fourteen lately patients had their descending aorta additionally cannulated to achieve a total body perfusion strategy. Results: Mean by-pass time was 155 minutes (range 63-353). Mean brain-coronary perfusion was 36 minutes (18-60). Mean coronary ischemia was 34 minutes (0-160). The heart was not arrested in group B patients. Follow-up was complete for a mean of 52 months (2-78). Five patients died in the postop. Three requiered angioplasty for recoarctation. Conclusions: Selective brain-coronary perfusion is feasible and easy to switch to conventional cardioplegia delivery. Coronary ischemia can be notably reduced, being even zero minutes in isolated arch surgery. Total body perfusion may reduce morbidity in neonatal arch surgery.

CD25, the alpha chain of the IL-2 receptor, is expressed on activated effector T cells that mediate immune graft damage. Induction immunosuppression is commonly used in solid organ transplantation and can include antibodies blocking CD25. However, regulatory T cells (Tregs) also rely on CD25 for their proliferation, survival, and regulatory function. Therefore, CD25-blockade may compromise Treg protective role against rejection. We analysed in vitro the effect of basiliximab (BXM) on the viability, phenotype, proliferation and cytokine production of Treg cells. We also evaluated in vivo the effect of BXM on Treg in thymectomized heart transplant children receiving BXM in comparison to patients not receiving induction therapy. Our results show that BXM reduces Treg counts and function in vitro by affecting their proliferation, Foxp3 expression, and IL-10 secretion capacity. In pediatric heart-transplant patients, we observed decreased Treg counts and a diminished Treg/Teff ratio in BXM-treated patients up to 6-month after treatment, recovering baseline values at the end of the 12-month follow up period. These results reveal that the use of BXM could produce detrimental effects on Tregs, and support the evidence suggesting that BXM induction could impair the protective role of Tregs in the period of highest incidence of acute graft rejection.

Introduction:Heart transplant after Fontan completion poses a unique surgical challenge. Twenty patients are presented, stressing the technical hints performed in the five anastomoses to match the graft in the recipient.Methods:Data are collected from 20 Fontan patients between 2013 and 2019. Age (13 years), weight (37 kg.), and time interval between Fontan and transplant (7 years) are presented as median. Extracardiac conduit (size 18/20) was implanted in 15 patients, whereas atrio-pulmonary connection was performed in 4 and lateral tunnel in 1. Six patients developed protein-losing enteropathy. Seventeen stents had been previously deployed.Results:The five anastomoses underwent some changes. Left atrium once, aorta 9 times, superior vena cava 7 times, pulmonary branches 15 times, and inferior vena cava 12 times. Follow-up was complete for a median of 42 months (range 6–84). Two patients died. ECMO was needed in six cases for pulmonary hypertension. Four patients had collateral vessels occluded in the cath lab, and stents were placed in superior vena cava (1) and aorta (1) post-transplant. Protein-losing enteropathy was resolved in five patients. Interestingly, one patient was on a systemic assist device before transplant (Levitronix) and right assistance (ECMO) afterwards.Conclusions:Transplant in Fontan patients is actually challenging. Hints in every of the five proposed anastomoses must be anticipated, including stents removal. Extra tissue from the donor (innominate vein, aortic arch, and pericardium) is strongly advisable. ECMO for right ventricular dysfunction was needed in nearly one-third of the cases. Overall results can match other transplant cohorts.

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.

Background Donation after Circulatory death is gaining worldwide acceptance. Most protocols regard their first cases to be performed with donor and recipient in the same institution. Few records of children or distant procurement have been published. Methods Our institution was offered a heart from a 3-day-old, 3.4-kg child, blood group A, suffering irreversible encephalopathy. Parents accepted withdrawal of life-sustaining therapy and agreed to donation. The donor hospital was located 340 km away. Concomitantly, a 2-month-old, 3.1 kg, blood group type B and with non-compaction ventricles was awaiting for the heart transplant in our unit. Results Thirty-seven minutes after withdrawal of life-sustaining therapy, the heart arrested. Five minutes afterwards, a sternotomy was performed. The supra-aortic vessels were clamped altogether. Aorta and right appendage were cannulated and connected to heart-lung machine. The innominate artery above the clamp was severed. The heart resumed spontaneous rhythm in less than 1 min. Ventilation was restored and extracorporeal circulation was maintained for 32 min. Upon cardiologic arrest, the graft was harvested as routinely. The heart was cold-stored and transported by plane to our Hospital. An orthotopic bicaval transplant was performed. Overall cold ischaemia was 245 min. Ten weeks later, the child was discharged home in good condition. Conclusion Donation in circulatory death could increase the pool in neonates. Extracorporeal circulation proves successful for procurement in neonates. Distant procurement plus cold storage for donation in circulatory death is feasible. Donation in circulatory death and ABO non-compatible strategies are complementary to each other.

Resumen: Introducción y objetivos: El trasplante cardiaco ABO incompatible se inició en el año 2001 y desde entonces son varios los centros que han adoptado esta estrategia. Describimos los 2 primeros pacientes trasplantados en España con donante ABO incompatible, así como el protocolo utilizado. Métodos: El requisito fundamental para poder ser incluido en lista de trasplante cardiaco ABO incompatible establecido en nuestro centro, y de acuerdo con otros centros de más experiencia, es tener un título de isohemaglutininas menor o igual a 1:8. Son precisas determinaciones periódicas de isohemaglutininas y puede ser necesario realizar varias exanguinotransfusiones durante el trasplante. Tras el trasplante se mantendrá la siguiente política transfusional de por vida: hematíes del grupo receptor o grupo 0, plasma AB y plaquetas AB o lavadas. Resultados: Realizamos 2 trasplantes en nuestro centro con donante ABO incompatible. Ambos receptores presentaban un síndrome de cavidades izquierdas hipoplásicas tratados con procedimiento híbrido (stent en ductus y banding pulmonar). El primero evolucionó muy favorablemente, sin ninguna complicación durante su ingreso. El segundo presentó un rechazo cardiaco con compromiso hemodinámico que precisó asistencia en ECMO en el contexto de bajos niveles de tacrolimus y no relacionado con la incompatibilidad de grupo. Se realizaron 4 ciclos de plasmaféresis. La evolución posterior fue satisfactoria. Conclusiones: El trasplante ABO incompatible en pacientes con títulos de isohemaglutininas inferior o igual a 1:8 es una técnica segura que permite aumentar las probabilidades de trasplante, reduciendo su tiempo en lista de espera y, con ello, sus posibles complicaciones y mortalidad. Abstract: Introduction and objectives: ABO-incompatible cardiac transplantation began in 2001 and since then there have been several centers that have adopted this strategy. We describe the first 2 patients transplanted in Spain with incompatible ABO donor as well as the protocol used. Methods: The fundamental requirement to be included in the ABO-incompatible cardiac transplant list established in our center and according to other more experienced centers is to have an isohemagglutinin titre less than or equal to 1: 8. Periodic determinations of isohemagglutinins are required and it may be necessary to perform several exchange transfusions at the time of transplantation. After transplantation, the following transfusion policy will be maintained for life: red blood cells from the recipient group or group 0, plasma AB and platelets AB or washed. Results: We performed 2 transplants in our center with an incompatible ABO donor. Both recipients presented a hypoplastic left heart syndrome treated with a hybrid procedure (ductus stent and pulmonary banding). The first evolved very favorably without any complications during his admission. The second one presented a cardiac rejection with hemodynamic compromise that required ECMO assistance in the context of low levels of tacrolimus and not related to the group incompatibility. Four plasmapheresis cycles were performed. The subsequent evolution was satisfactory. Conclusions: ABO-incompatible transplantation in patients with isohemagglutinin titrers less than or equal to 1:8 is a safe technique that increases the chances of transplantation, reducing their waiting list time and thus its possible complications and mortality. Palabras clave: Trasplante cardiaco, Pediátrico, ABO incompatible, Isohemaglutininas, Exanguinotransfusión, Lista de espera, Keywords: Cardiac transplant, Pediatric, ABO-incompatible, Isohemagglutinins, Exchange transfusion, Waiting list

Atopic dermatitis (AD) has a high incidence in heart-transplant children, and the reason why there is more AD after transplantation is still unknown. We conducted a cross-sectional study comparing 11 AD and 11 non-AD age-matched heart-transplant children, to assess which immune alterations are related to AD in these patients. AD patients had been transplanted at a younger age compared to non-AD, indicating that age at transplant may be determinant in the onset of AD. The earlier thymectomy in AD heart-transplant children favored the presence of more differentiated phenotypes in the T cell compartment. We observed a clear reduction in the T-helper 1/T-helper 2 (Th1/Th2) ratio in AD children. This Th2 polarization was related to eosinophilia and high immunoglobulin E levels, but also to an impaired regulatory T cell (Treg) suppression, which could be secondary to an exhaustion of the Treg compartment. Interestingly, AD patients were free of rejection episodes (0/11) in comparison to non-AD children (4/11). We propose that a predominant Th2 phenotype may prevent the emergence of Th1 responses associated with graft rejection. A more differentiated Treg phenotype could also play a role in preventing acute rejection in the first year posttransplant. Our findings provide useful insights and knowledge for the better understanding of atopic disorders in transplanted children.

Background: Collaboration between cardiac surgeons and cardiologists can offer interventions that each specialist may not be able to offer on their own. This type of collaboration has been demonstrated with the hybrid Stage I in patients with hypoplastic heart syndrome. Since that time, a hybrid approach to cardiac interventions has been expanded to an incredible variety of potential indications.Methods: Seventy-one patients were scheduled for a hybrid procedure along 8 years. This was defined as close collaboration between surgeon and cardiologist working together in the same room, either cath-lab (27 patients) or theater (44 patients).Results: Six groups were arbitrarily defined. A: vascular cut-down in the cath-lab (27 neonates); B: bilateral banding (plus ductal stent) in hypoplastic left heart syndrome or alike (15 children); C: perventricular closure of muscular ventricular septal defect (10 cases); D: balloon/stenting of pulmonary branches along with major surgical procedure (12 kids); E: surgical implantation of Melody valve (six patients) and others (F, one case). Two complications were recorded: left ventricular free wall puncture and previous conduit tearing. Both drawbacks were successfully sort out under cardiopulmonary by-pass.Conclusion: Surgeon and cardiologist partnership can succeed where their isolated endeavors are not enough. Hybrid procedures keep on spreading, overcoming initial expectations. As a bridge to biventricular repair or transplant, bilateral banding plus ductal stent sounds interesting. Novel indications can be classified into different groups. Hybrid procedures are not complication-free.

Los aneurismas congénitos del ventrículo izquierdo (VI) constituyen una patología infrecuente. Pueden presentarse en forma asintomática, causar embolización, arritmias malignas, insuficiencia cardíaca, rotura o muerte súbita.

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.

INTRODUCTION AND OBJECTIVES The present report updates the characteristics and results of heart transplantation in Spain, mainly focused in the 2008-2017 period. METHODS We describe the recipient and donor characteristics, surgical procedures, and outcomes of heart transplants performed in 2017. The 2017 data were compared with those obtained from 2008 to 2016. RESULTS A total of 304 cardiac transplants were performed in 2017. Between 1984 and 2017, 8173 procedures were performed, 2689 of them after 2008. Significant temporal trends were observed in recipient characteristics (lower pulmonary vascular resistance, lower use of mechanical ventilation, and a higher percentage of diabetic patients and those with previous cardiac surgery), donor characteristics (older donor age and a higher percentage of female donors and those with a prior cardiac arrest) and procedures (lower ischemia time). In 2017, 27% of patients were transplanted after undergoing mechanical ventricular assistance (P

We report a case of an 18-year-old woman who presented with infective endocarditis (IE), in two conduits percutaneously delivered in the right ventricle outflow tract ("double-barrel endocarditis"). The patient's clinical presentation, echocardiogram findings, infectious agent, clinical management, surgical approach, and follow-up assessment are described. Percutaneous pulmonary valve implantation has emerged as a viable therapy for conduit dysfunction in the right ventricular outflow tract. Although the percutaneous approach has several advantages, this strategy and the valves used are not complication-free. IE after transcatheter valve deployment has evoked the growing concern, as there is a higher incidence in these patients compared with patients with surgically repaired pulmonary valves. As a result, this type of surgical treatment is especially important.

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.

The arterial switch operation is currently the gold standard technique for repair of transposition of the great arteries. Some atypical coronary patterns such as intramural, interarterial, and a unique posterior button are associated with more complexity and surgical risk. We report a successful Aubert operation for transposition of the great arteries associated with a single and interarterial coronary artery arising from a posterior sinus.

Objectives We sought to determine the indications, type, and outcomes of reoperations on the aortic root after repair of tetralogy of Fallot (TOF). Methods Eleven centers belonging to the European Congenital Heart Surgeons Association contributed to the data collection process. We included 36 patients who underwent surgical procedures on the aortic root, including surgery on the aortic valve and ascending aorta, between January 1975 and December 2017. Original diagnoses included TOF-pulmonary stenosis (n = 18) and TOF-pulmonary atresia (n = 18). The main indications for reoperation were aortic insufficiency (n = 19, 53%), aortic insufficiency and dilatation of the ascending aorta (n = 10, 28%), aortic root dilatation (n = 4, 11%), and ascending aorta dilatation (n = 3, 8%). Results The median age at reoperation was 30.4 years (interquartile range 20.3-45.3 years), and mechanical aortic valve replacement was the most common procedure performed. Five patients died early after reoperation (14%), and larger ascending aorta diameters were associated with early mortality (P = .04). The median age at the last follow-up was 41.4 years (interquartile range 24.5-51.6 years). Late death occurred in five patients (5/31, 16%). Most survivors (15/26, 58%) were asymptomatic at the last clinical examination (New York Heart Association, NYHA class I). The remaining patients were NYHA class II (n = 7) and III (n = 3). The most common symptoms were fatigue (n = 5), dyspnea (n = 4), and exercise intolerance (n = 3). Conclusions Reoperations on the aortic root are infrequent but may become necessary late after TOF repair. The main indications for reoperation are aortic insufficiency, either isolated or associated with a dilatation of the ascending aorta. The surgical risk at reoperation was high and the presence of ascending aorta dilation is related to higher mortality.

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

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.

Resumen Los diferentes abordajes en cirugia de minima invasion han reducido la morbilidad y mejorado los resultados esteticos en cirugia cardiaca en pacientes adultos en los anos recientes. Sin embargo, en pacientes pediatricos, su empleo ha sido mucho mas limitado. En este contexto, los accesos por mini-incision alternativos a la esternotomia media convencional han ido cobrando relevancia, pero un paso mas alla hacia la introduccion de la cirugia videoasistida aun parece lejano. El pequeno tamano de estos pacientes con cardiopatias congenitas con defectos intracardiacos representa el principal factor limitante. Presentamos la primera experiencia inicial en Espana para entrenamiento en procedimientos videoasistidos con modelos animales y su posterior aplicacion al ambito de la cirugia cardiaca pediatrica. Discutimos los resultados preliminares de este programa, asi como las ventajas, inconvenientes y areas de mejora de la videoasistencia en este contexto, y enfatizamos la necesidad de una estrecha colaboracion entre cirujanos pediatricos expertos en toracoscopia junto con cirujanos cardiacos pediatricos para poder seguir avanzando en este complejo escenario.

This report describes a simple way to deal with time-consuming adhesions and cannula handling in patients with a paracorporeal assist device who are undergoing heart transplantation. By connecting the extrathoracic lines to the heart-lung machine, chest reentry becomes a straightforward issue.

Pulmonary valve repair in an adolescent through upper ministernotomy is reported. Several tips to enhance proper display of the valve in the surgical field are depicted.