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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.

espanolIntroduccion: La cardiopatia isquemica es una de las principales causas de morbimortalidad en los paises desarrollados dificultando la vuelta al trabajo de estos pacientes. Pretendemos estudiar la evolucion de la incapacidad Temporal y Permanente en trabajadores espanoles que causan baja por cardiopatia isquemica, asi como analizar las variables que influyen en la reincorporacion laboral. Material y metodos: Estudio observacional retrospectivo de cohortes con pacientes menores 65 anos que sufrieron un infarto agudo de miocardio (IAM) entre Enero de 2000 y mayo de 2011. Se recogieron datos clinicos, demograficos, socioeconomicos y laborales para analizar su relacion con la reincorporacion laboral. Resultados: Se seleccionaron un total de 5067 pacientes, el 94% varones, y con una edad media de 54,3 anos. Un total de 3.679 pacientes (72,6%) se reincorporaron a su actividad laboral tras el infarto, mientras que 1261 (24,9%) obtuvieron la incapacidad laboral permanente. La mayores tasas de incapacidad laboral se observan en los pacientes > 50 anos, en los que han sufrido un mayor dano cardiaco (FEVI 50%) y el procedimiento de revascularizacion empleado (percutaneo/cirugia) asi como las variables socioeconomicas referentes al tipo de trabajo (autonomo/cuenta ajena), ocupacion profesional (carga fisica/carga intelectual) y la edad del trabajador EnglishBackground: Ischemic cardiopathy is one of the main causes of morbidity and mortality in developed countries. It affects the patients quality of life and interferes with their return to work, thus generating a significant economic cost. The aim of our study is to assess labor conditions of patients who suffered an acute myocardial infarction (AMI), highlighting the factors that could have an impact on their return-to-work time. Materials and methods: Observational retrospective study with patients under 65 years old who suffered an AMI between January 2000 and May 2011. Data concerning clinical, social, demographic and labor circumstances was collected to better understand its relation with the return to work. Results: A total of 5.067 patients with Coronary Heart Disease (hereinafter referred to as CHD) were selected. 94% of the samples were men, under 54.3 years old. 3679 out of 5067 patients (72. 6%) returned to work after a heart infarct whereas 1261 (24.9%) did not return, obtaining the permanent incapacity to work. Among the clinical and patient factors, the main determinants were age (mostly 50% / EF >50%) and revascularization procedures (percutaneous coronary interventions -PCI- and coronary artery bypass graft surgery, CABG). Regarding the economic and job-related factors three variables were found to be the strongest predictors of returning to work: job demands (physically demanding jobs vs. intellectually demanding jobs), incomes and job conditions (self-employment vs. employees). There were no significant differences on the returning-to-work rate between men or women or between sick leave due to an acute coronary syndrome or due to an angina. We observed that the return-to-work activity increased gradually from 2000-2011 (from rates of 60% in 2000-2002 to over 80% in 2010). This increase is present in all occupational activities as well as in all age ranges. Conclusion: In our study we observed a gradual increase of patients returning to work in recent years. The variables used to better predict the return-to-work time in a period of sick leave after a CHD episode are categorized as follows: clinic viable like the extent of myocardial damage (LVEF 50%) and the revascularization procedures (Percutaneous coronary intervention (PCI) or coronary artery bypass graft surgery, CABG), socioeconomic variables related to the kind of job (self-employment vs. employees) and lastly labor conditions like job demands (physical-demanding jobs vs. intellectual-demanding jobs) or age.

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).

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)

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.

Complex cases undergo step surgical and percutaneous procedures, including stent deployment. Concerns arise on stent removal at latest surgery. Our initial experience is presented. Forty-six stents in 35 patients were partially or totally removed at surgery. Univentricular heart was diagnosed in 20 patients. Stents were previously deployed in: ductus (6), right ventricle outflow tract (12), atrial septal defect (4), right pulmonary artery (4), left pulmonary artery (16), inferior vena cava (2), superior vena cava (1) and ascending aorta (1). Surgical procedures performed: 9 transplants, 6 Fontan, 4 Glenn, 1 comprehensive repair (Norwood + Glenn), 1 Glenn takedown, 8 conduit replacement, 2 Fallot, 2 Rastelli, 1 ventricular septal defect closure and 1 iatrogenic aortopulmonary window. Five ductal stents were clipped. Eleven stents in right ventricle, four ones in atrial septal defect, two in right pulmonary artery, seven in the left pulmonary artery and two in inferior vena cava were completely removed. Two stents in right pulmonary artery, one in superior vena cava, one in ascending aorta and nine in the left pulmonary artery were partially retrieved. Handling the stents in ductus, right ventricle and atrial septal defect was straightforward. On the contrary, stent removal in the ductus (comprehensive case), pulmonary branches, both vena cavae or aorta required short periods of deep hypothermia with circulatory arrest. Surgery over stents is increasing in complex, step procedures. Univentricular hearts are most prevalent. Congenital transplant surgery faces new challenges. Stent removal at the time of surgery may require deep hypothermic circulatory arrest.

We present the first reported case of truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage into a retro-aortic innominate vein in a neonate in whom successful palliation was performed using a two-staged procedure. An early repair after presentation along with a re-routing of anomalous pulmonary venous drainage at the time of the second stage should be undertaken to optimize the patient's haemodynamic status prior to a Fontan completion.

FIGURE 1. A, Cardiac catheterization. Melody valve (Medtronic Inc, Minneapolis, Minn) and stent (descending aorta) are shown. Amplatzer device (yellow arrow) into the APW. B, Displacement of the Amplatzer device (yellow arrow) after implantation of the left PA stent. Ao, Aorta; LPA, left pulmonary artery; PA, pulmonary artery. CLINICAL SUMMARY A 2-month-old baby with a diagnosis of dextrotransposition of the great arteries underwent the ASO (LeCompte maneuver). At the age of 10 years, a covered stent was placed on the main pulmonary artery (PA) for supravalvular pulmonary stenosis, but secondary pulmonary regurgitation developed. The patient was referred for transcatheter pulmonary valve implantation at the age of 11 years. A 22-mm, stent-mounted valved bovine jugular vein graft (Melody valve; Medtronic Inc, Minneapolis, Minn) was implanted, but the patient became hemodynamically unstable (pulmonary-to-systemic ratio 1.96). Transesophageal echocardiography (TEE) showed a traumatic APW in the uppermost portion of the ascending aorta. Hemodynamic stability was achieved after closure using a 10-mm Amplatzer Muscular VSD Occluder (AGA Medical Corp, Golden Valley, Minn) (Figure 1, A), but he remained symptomatic in the subsequent weeks. TEE showed residual left-to-right shunt in the proximal margin, and a covered stent was implanted on the left PA to deal with the residual shunt, but the Amplatzer was dislocated (Figure 1, B) and the patient was referred for emergency surgery. Arterial cannulation was performed through the left femoral artery, and the inferior venae cavae and right atrium were cannulated separately. The patient was cooled to 18 C

Cardiac myxoma is a benign primitive tumor of endocardial origin that occurs with an incidence of 40-50% of all detected primary cardiac neoplasms. The vast majority of them are manifest as cavitary gelatinous masses and they may have a smooth or rough surface or thrombus adherent. The tumor is constituted by primitive connective tissue cells form rings, cords and nests that are often associated with capillaries and they exist in a myxoid stroma that is composed of variable amounts of proteoglycans, elastin and collagen.

True aneurysm formation in arterialized autologous veins is an unusual complication. A saccular aneurysmal degeneration of 53 mm (maximal diameter) of a saphenous vein graft inserted for repair of a popliteal aneurysm, four years after implantation, is reported. The patient (with prior history of abdominal aortic aneurysm) had been initially treated through a posterior approach. A new saphenous vein bypass grafting (medial approach) was performed. Histological examination revealed myointimal fibrosis, medial degeneration and inflammation. In spite of the widespread use of the autologous saphenous vein as an arterial substitute, this complication is extremely rare and its etiology remains unclear. Atherosclerosis is considered to be the main cause of aneurysm formation in vein grafts, but current data suggest that additional etiopathogenic factors should be further investigated. We note the rarity of this finding and review the literature for true aneurysm formation within vein grafts used for bypass procedures.

An optimal preoperative evaluation is fundamental to the assessment of all patients undergoing cardiac surgery. We report the case of a male patient who underwent elective coronary artery bypass surgery, and intraoperative transesophageal echocardiography (TEE) revealed an unexpected left atrial cavernous hemangioma. The tumor was resected via a transatrial approach, and a definitive diagnosis was made after histological examination. This uncommon intra-atrial tumor (which accounts for approximately 2.8% of all benign cardiac neoplasms) was detected at the time of the operation as no basic echocardiography study had been performed on the patient during the process of diagnosing his coronary heart disease because of a suboptimal cardiological work-up. Most cardiac masses are discovered incidentally by imaging techniques; in this patient, transthoracic echocardiography could have aided in the preoperative study and enabled the cardiac surgeon to plan and perform the adequate surgical procedure beforehand. Intraoperative TEE should be used routinely in all patients undergoing cardiac surgery: the clinical information obtained in certain cases might have a direct impact on surgical decision-making and might therefore positively influence patient's outcome.

Right-sided lesions occur in 5-25% of patients after arterial switch operation (ASO) and percutaneous or surgical treatments are available (although they are not complication-free).

Orthotopic heart transplantation after univentricular palliation presents a difficult challenge due to the complex anatomy and prior surgeries.

A need persists for Fontan conversion (classical Fontan to total cavopulmonary connection (TCPC)) that provides alternative approaches for the individual anatomical challenges occurred in these unusual and complex congenital grown-up patients.

Case Reports; Journal Article; About 2% of all paragangliomas are located in the chest, and a few have been described to be found in the heart. Primary cardiac paragangliomas are extremely uncommon tumors and surgical experience with this neoplasm is limited. Treatment strategies described in the literature have included simple excision, excision with reconstruction, autotransplantation after excision of the tumor and even orthotopic cardiac transplantation, depending on the extent of disease. A primary retrocardiac paraganglioma catecholamine-productive was identified in an asymptomatic 49-year old female associated to familial pheochromocytoma-paraganglioma syndrome caused by germline mutation of the gen which codifies for the subunit B of succinate dehydrogenase enzyme (SDHB). The neoplasm was surgically excised from the posterior surface of the left atrium via median sternotomy using cardiopulmonary bypass. Direct ligation of feeding vessels of the tumor along with left atrial reinforcement using a pericardial patch was performed. The post-operative course was uneventful, with normalization of catecholamine secretion and no recurrence at three-month follow-up. We review the current literature about this exceptional cardiac tumor, pathophysiological conditions and options for surgical management. Yes

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.

ResumenLa atresia pulmonar es una cardiopatía congénita compleja caracterizada por la falta de continuidad entre el ventrículo derecho y la arteria pulmonar. Cuando se asocia a comunicación interventricular y arterias colaterales aortopulmonares mayores supone un reto quirúrgico que puede precisar diferentes vías de abordaje en distintos tiempos quirúrgicos, como el caso presentado.AbstractPulmonary atresia is a complex congenital heart disease characterized by the lack of continuity between the right ventricle and pulmonary artery. Pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries remain as a surgical challenge and may require staged surgical approaches, as in the case presented.