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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.

A prospective, observational single-center study was carried out. Pediatric patients undergoing congenital heart defect surgery were evaluated before, during, and after surgery. At each time point, sublingual microcirculation and clinical parameters were assessed, along with analytical variables. Twenty-four patients were included. All microcirculatory parameters worsened during cardiopulmonary bypass and returned to baseline values after surgery (p ≤ 0.001). In the intraoperative evaluation, body temperature correlated with perfused small vessel density (p = 0.014), proportion of perfused small vessels (p < 0.001), small vessel microvascular flow index (p = 0.003), and small vessel heterogeneity index (p < 0.002). Patients with cyanotic disease exhibited higher small vessel density (p < 0.008) and higher density of perfused small vessels (p < 0.022) at baseline, and a lower microvascular flow index (p = 0.022) and higher heterogeneity (p = 0.026) in the intraoperative phase. Children with congenital heart disease exhibited decreased vascular density and microvascular blood flow and increased heterogeneity during cardiopulmonary bypass. All these parameters returned to baseline values after surgery.

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.

Continuous incisional lidocaine infusion has been proposed as an adjunctive therapy in the management of postoperative pain in adult patients. The aim of this study was to determine the efficacy and safety of a continuous subcutaneous lidocaine infusion in pediatric patients following open heart surgery. All patients receiving a subcutaneous lidocaine infusion in median sternotomy incisions after open heart surgery during 2 consecutive years were included in the study. A historical cohort of patients was used as a control group. Demographic variables (age, size, and surgical procedure), variables related to sedation and analgesia (COMFORT and analgesia scales, drug doses, and duration), and complications were registered. 106 patients in the lidocaine infusion group and 79 patients in the control group were included. Incisional analgesia was effective for the treatment of pain as it reduced the dose and duration of intravenous fentanyl (odds ratio (OR) 6.26, confidence interval (CI) 95%: 2.48-15.97, p = 0.001 ; OR 4.30, CI 95%: 2.09-8.84, p = 0.001 , respectively). The reduction in fentanyl use was more important in children over two years of age. Adverse effects were seen in three children (2.8%): they all had decreased level of consciousness, and one of them presented seizures as well. Two of these three patients had lidocaine levels over 2 mcg/ml. A continuous lidocaine incisional infusion is effective for the treatment of pain after open heart surgery. This procedure reduced intravenous analgesic drug requirements in pediatric patients undergoing a median sternotomy incision. Although the incidence of secondary effects is low, monitoring of neurologic status and lidocaine blood levels are recommended in all patients.

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.

Background The use of intraoperative fluorescence images with indocyanine green (ICG) has recently been described as an aid in decision-making during surgical procedures in adults.We present our first experiences with different laparoscopic procedures performed in children using ICG fluorescence images. Material and Method We have used ICG fluorescence imaging technique in varicocele ligation, two nephrectomies, cholecystectomy, and one case of aortocoronary fistula closure. All procedures were performed through a minimally invasive approach. A high definition camera equipped with a visible infrared light source and gray-scale vision technology was used.After injection of ICG before or during the laparoscopic procedure, precise identification of vascular anatomy and bile duct architecture were easily identified. Fluorescence helped to assess blood flow from the spermatic vessels, define the variability of renal vascularization, and determine the precise location of the aortocoronary fistula. Biliary excretion of the ICG allowed the definition of the biliary tract. Conclusion Fluorescein-assisted images allowed a clear definition of the anatomy and safe surgical maneuvers during surgical procedures. The ICG imaging system seems to be simple and safe. Larger and more specific studies are needed to confirm its applicability, expand its indications, and address its advantages and disadvantages.

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

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

Regulatory T cells (Tregs), which are characterized by the expression of the transcription factor forkhead box P3 (FOXP3), are the main immune cells that induce tolerance and are regulators of immune homeostasis. Natural Treg cells (nTregs), described as CD4+CD25+FOXP3+, are generated in the thymus via activation and cytokine signaling. Transforming growth factor beta type 1 (TGF-β1) is pivotal to the generation of the nTreg lineage, its maintenance in the thymus, and to generating induced Treg cells (iTregs) in the periphery or in vitro arising from conventional T cells (Tconvs). Here, we tested whether TGF-β1 treatment, associated with interleukin-2 (IL-2) and CD3/CD28 stimulation, could generate functional Treg-like cells from human thymocytes in vitro, as it does from Tconvs. Additionally, we genetically manipulated the cells for ectopic FOXP3 expression, along with the TGF-β1 treatment. We demonstrated that TGF-β1 and ectopic FOXP3, combined with IL-2 and through CD3/CD28 activation, transformed human thymocytes into cells that expressed high levels of Treg-associated markers. However, these cells also presented a lack of homogeneous suppressive function and an unstable proinflammatory cytokine profile. Therefore, thymocyte-derived cells, activated with the same stimuli as Tconvs, were not an appropriate alternative for inducing cells with a Treg-like phenotype and function.

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.

A prospective, observational single-center study was carried out. Pediatric patients undergoing congenital heart defect surgery were evaluated before, during, and after surgery. At each time point, sublingual microcirculation and clinical parameters were assessed, along with analytical variables. Twenty-four patients were included. All microcirculatory parameters worsened during cardiopulmonary bypass and returned to baseline values after surgery (p ≤ 0.001). In the intraoperative evaluation, body temperature correlated with perfused small vessel density (p = 0.014), proportion of perfused small vessels (p0.001), small vessel microvascular flow index (p = 0.003), and small vessel heterogeneity index (p0.002). Patients with cyanotic disease exhibited higher small vessel density (p0.008) and higher density of perfused small vessels (p0.022) at baseline, and a lower microvascular flow index (p = 0.022) and higher heterogeneity (p = 0.026) in the intraoperative phase. Children with congenital heart disease exhibited decreased vascular density and microvascular blood flow and increased heterogeneity during cardiopulmonary bypass. All these parameters returned to baseline values after surgery.

Background The integration of 3D printing technology in hospitals is evolving toward production models such as point-of-care manufacturing. This study aims to present the results of the integration of 3D printing technology in a manufacturing university hospital. Methods Observational, descriptive, retrospective, and monocentric study of 907 instances of 3D printing from November 2015 to March 2020. Variables such as product type, utility, time, or manufacturing materials were analyzed. Results Orthopedic Surgery and Traumatology, Oral and Maxillofacial Surgery, and Gynecology and Obstetrics are the medical specialties that have manufactured the largest number of processes. Working and printing time, as well as the amount of printing material, is different for different types of products and input data. The most common printing material was polylactic acid, although biocompatible resin was introduced to produce surgical guides. In addition, the hospital has worked on the co-design of custom-made implants with manufacturing companies and has also participated in tissue bio-printing projects. Conclusions The integration of 3D printing in a university hospital allows identifying the conceptual evolution to “point-of-care manufacturing.”

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.

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.

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.

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.

Modified techniques for orthotopic heart transplantation are mandatory when complex congenital anomalies are associated in adult patients. An unusual case of a heterotaxy syndrome and dilated cardiomyopathy following mitral ring annuloplasty is presented in a 62-year-old male. Orthotopic cardiac transplantation was performed by using a modified operative strategy: selective peripheral and central venous cannulation according to the thoraco-abdominal venous challenges, biatrial technique, and preservation of venous drainage via the native coronary sinus. We discuss the anatomical features of heterotaxy in adult patients and surgical approaches when heart transplantation is needed.

A need persists for Fontan conversion that provides alternative approaches for the individual anatomical challenges occurring in these unusual and complex adult patients. The pulmonary arteries present unique variations and the surgical technique needs to be intraoperatively addressed. We describe a technique for Fontan conversion for performing the distal anastomosis of the extracardiac conduit, allowing adequate matching to the pulmonary arteries and preserving an optimal flow into the Fontan circuit. doi: 10.1111/jocs.12564 (J Card Surg 2015;30:605–607)