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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.

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

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.

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

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.

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.

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.

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.