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We present the fifth "Jatene Lecture on Surgical Innovation" on Innovation in Congenital Heart Surgery, given at the Eighth Scientific Meeting of the World Society for Pediatric and Congenital Heart Surgery and Eighth World Congress of Pediatric Cardiology and Cardiac Surgery in Washington DC in 2023. We highlight what surgical innovation is and how innovation was accomplished in cardiac surgery and particularly in congenital heart surgery. A brief history of the development of congenital heart surgery across the world is summarized and we finally illustrate the South American contributions to congenital heart surgery, acknowledging the great innovations of Adib Jatene and Guillermo Kreutzer to our field.

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The current surgical approach for patients with a single ventricle is the culmination of decades of dedicated research and groundbreaking innovation. From 1971 to the present day, a series of incremental advancements have significantly extended the life expectancy of these patients. Since the very beginning, Dr Guillermo Kreutzer and his team have pioneered different techniques with the ultimate goal of improving outcomes for these individuals. This is, narrated by him, the story of how it all began.

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INTRODUCCIÓN: El reemplazo quirúrgico con colocación de bioprótesis es una estrategia utilizada frecuentemente para tratar valvulopatías en cardiopatías congénitas. OBJETIVO: Presentar la experiencia inicial en Argentina con fractura/remodelación del anillo valvular en bioprótesis y posterior reemplazo percutáneo "válvula en válvula" en pacientes con cardiopatías congénitas. MATERIAL y métodos: Estudio descriptivo y observacional. Se incluyeron todos los pacientes tratados con reemplazo percutáneo debido a disfunción de prótesis valvular biológica. RESULTADOS: Desde agosto de 2021 hasta mayo de 2023, 5 pacientes (3 de sexo femenino) con disfunción de bioprótesis derechas recibieron tratamiento percutáneo como alternativa a la realización de un nuevo reemplazo quirúrgico. La edad media fue 21,2 ± 9,2 años, el peso medio fue 56,2 ± 22,2 kg. Fueron implantadas cinco válvulas balón expandibles: 3 en posición pulmonar y 2 en posición tricúspide y en 4 casos, con fractura previa del anillo valvular. En todos los pacientes se restauró la función valvular, sin complicaciones. En el seguimiento, en un sólo paciente se objetivó insuficiencia valvular moderada derecha CONCLUSIÓN: El reemplazo percutáneo "válvula en válvula" en bioprótesis disfuncionantes derechas es una estrategia atractiva y segura en casos seleccionados, que restaura la competencia y elimina las obstrucciones. Esta estrategia es una opción razonable como alternativa a la realización de un nuevo recambio valvular quirúrgico.

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AIM: To investigate the opinion and personal experience of parents of children born with Hypoplastic Left Heart Syndrome and what advice they would give to other parents who have to decide between treatment options. METHODS: We conducted a qualitative, descriptive and retrospective study by means of a survey directed to parents of children born with Hypoplastic Left Heart Syndrome in a tertiary hospital in Buenos Aires (Argentina). Their answers and data regarding medical procedures were analysed. RESULTS: Parents of thirteen out of sixteen patients with Hypoplastic Left Heart Syndrome were surveyed. Norwood surgery had been performed in all the patients, many had received other procedures, and five had died. In relation to the decision-making process, sixty-one percent of parents would recommend other parents to remain at peace after having done everything possible and 54% would suggest to not feel guilt despite the final result. None of the parents would recommend rejecting surgical treatment and choosing comfort care. CONCLUSION: The majority of parents of children with Hypoplastic Left Heart Syndrome would recommend continuing with the therapeutic effort in order to feel at peace and reduce feelings of guilt.

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The Stage 1 Norwood procedure is the first of 3 stages in the surgical palliation of hypoplastic left heart syndrome and certain other single ventricle lesions with systemic outflow obstruction. In this article, we address some technical aspects and common pitfalls of the Norwood procedure with systemic to pulmonary shunt for HLHS palliation. We report our results with the Norwood with Blalock Taussig shunt in a cohort of 44 patients over a 7-year period in 2 institutions in Argentina. The results of the Norwood procedure have improved significantly through the understanding and refinement of the surgical techniques. Procedures must be technically perfect since residual lesions are poorly tolerated. Norwood with a modified Blalock Taussig shunt can be performed with low mortality and may provide excellent long-term outcomes.

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OBJECTIVE: Coronary complications may present during or after repair of congenital heart defects. We report coronary artery bypass grafting (CABG) by internal thoracic artery (ITA) grafts to either coronary artery in children with congenital anomalies. METHODS: Four cases who underwent CABG with ITA grafts from March 2016 to March 2020 were retrospectively reviewed. RESULTS: At the time of operation, patient's ages and weight were 7 and 20 months old and 14 and 15 years old and 6.5, 10, 40, and 45 kg, respectively. Diagnosis were anomalous origin of the left coronary artery from the pulmonary artery with leftward lateral ostial origin (n = 1), neopulmonary annulus hypoplasia post arterial switch with contiguous right coronary artery (RCA) arising from the left facing sinus (n = 1), RCA stenosis after the Ross procedure (n = 1), and right coronary ostial obstruction after aortic valve replacement in truncus arteriosus (n = 1). Procedures included left ITA to left coronary ostium (n = 1), right ventricular outflow tract (RVOT) enlargement with pulmonary valve replacement with left ITA to RCA (n = 1), RVOT enlargement with pulmonary valve replacement with right ITA to RCA (n = 1), and aortic valve re-replacement, pulmonary valve replacement, and right ITA to RCA (n = 1). At last follow-up, all four patients were asymptomatic, with normal ventricular function, and all grafts were patent. CONCLUSIONS: The use of CABG in children is valuable alternative when dealing with complex coronary anatomy not suitable for classic repairs. In children, graft patency is required to be longer than 50 years; therefore, use of arterial grafts seems mandatory.

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OBJECTIVE: To present a strategy for identifying patients at risk of lymphatic failure in the setting of planned Fontan/Kreutzer completion, allowing a tailored surgical approach. METHODS: Since January 2017, clinical evaluation before performance of the Fontan/Kreutzer procedure included T2-weighted magnetic resonance imaging (MRI) lymphangiography. Thoracic lymphatic abnormalities were categorized using a scale of I to IV according to progression of severity. Patients with severe lymphatic abnormalities (types III and IV) underwent Fontan/Kreutzer with lymphatic decompression via connection of the left jugular-subclavian junction containing the thoracic duct to the systemic atrium (group A). RESULTS: Thirteen patients were enrolled. Magnetic resonance imaging showed type I abnormalities in four cases (30.7%), II in four (30.7%), III in two (15.3%), and IV in three (23.3%). Patients in types III and IV underwent a Fontan/Kreutzer with lymphatic decompression (group A, n = 5), while patients in types I and II underwent a fenestrated extracardiac Fontan/Kreutzer procedure without lymphatic decompression (group B, n = 8). Preoperatively, there were no differences in age, weight, ventricular dominance (right vs left), superior vena cava pressure, incidence of chylothorax after previous superior cavopulmonary anastomosis (Glenn), or need for concomitant procedures at Fontan/Kreutzer completion. There were no differences in procedural times between the groups, nor were there differences in mortalities and Fontan/Kreutzer takedowns. There were no statistically significant differences in early and late morbidity between the two groups with the exception of total volume of effusions output postoperatively. At median follow-up of 18 months (range, 4-28 months), all patients in group A are in New York Heart Association class 1 with no differences between groups in arterial oxygen saturation. CONCLUSIONS: Lymphatic decompression during Fontan/Kreutzer procedure was successfully performed in patients identified by MRI as predisposed to lymphatic failure. A larger cohort of patients and longer follow-up are required to determine the efficacy of this approach in preventing early- and long-term Fontan/Kreutzer failure.

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Las malformaciones congénitas del pulmón y de la vía aérea (MCPA) son un grupo heterogéneo de alteraciones del desarrollo pulmonar. Se producen en distintas etapas de la embriogénesis, afectando al parénquima, la irrigación arterial, el drenaje venoso o una combinación de ellos. La incidencia estimada es de 1 cada 10.000 a 35.000 embarazos. Existen muchas clasificaciones, la más utilizada es la de Langston basada en la fisiopatología, que incluye a los secuestros pulmonares (SP)1, 2. Los SP son MCPA poco frecuentes; afectan predominantemente a varones. Tienen origen mixto: bronquial y arterial; se los define como una zona de tejido pulmonar embrionario no funcionante y displásico, la mayoría de los cuales se localizan en los lóbulos inferiores del pulmón. La clínica es variable. Desde el punto de vista anatómico se los clasifica en 1) secuestros intralobares (SI) y secuestros extralobares (SE); en ambos la vascularización se hace a través de una arteria sistémica anómala. Los SI se localizan en un lóbulo pulmonar, y están cubiertos por pleura visceral; su drenaje venoso se realiza a través de las venas pulmonares. Raramente se asocian con otras anomalías congénitas. Integran el listado de los secuestros típicos. Los SE tienen pleura independiente y su drenaje venoso es hacia la vena cava o ácigos. Clínicamente suelen caracterizarse por neumopatías a repetición de difícil resolución e igual ubicación. Más del 60% se asocian a otras malformaciones. El plan diagnóstico de los SP incluye técnicas de imágenes pre y posnatal. El tratamiento varía dependiendo del momento del diagnóstico, la presentación clínica, complicaciones y malformaciones asociadas. En la mayoría de los casos se recomienda la resección quirúrgica. El estudio anatomopatológico de la pieza resecada confirma el diagnóstico2- 4. Presentamos un lactante con SE, asociado a otras malformaciones

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We report the case of an 11-year-old girl with heterotaxy syndrome, dextrocardia, and azygos continuation of an interrupted inferior vena cava who had developed pulmonary arteriovenous fistulas after a Kawashima procedure consisting of bilateral superior cavopulmonary anastomoses. She presented with profound cyanosis, fatigue, and failure to thrive. An operative procedure to direct hepatic vein effluent to the pulmonary circulation was performed with placement of an extracardiac conduit between the hepatic veins and the left pulmonary artery. Persistence of cyanosis led to investigation, which led to the discovery of an unintentionally excluded right hepatic vein. A percutaneous transhepatic catheter intervention was performed in which a vascular plug was implanted to occlude the "missed" right hepatic vein, redirecting the flow through intrahepatic venovenous channels to the conduit. Clinical condition and arterial oxygen saturation were substantially improved one year after the two-step hepatic vein inclusion procedure.

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In spite of excellent long term survival the Fontan Kreutzer procedure commonly presents late failure due to end-organ damage. Several advances have been described to refine single ventricle management and surgical techniques. However, very little research has been dedicated to the lymphatic circulation in the precarious Fontan hemodynamic state. The lymphatic circulation is clearly affected since there is increased lymph production, which requires to be drained at a similar or higher pressure than it is produced, commonly resulting in chronic lymphedema. Chronic lymphedema induces fibrosis and end-organ failure even in normal circulation. Diverting lymph drainage to the low-pressured systemic atrium in Fontan may represent a valid alternative for the treatment of devastating complications as protein-losing enteropathy and plastic bronchitis and may prevent or decrease the development of end-organ fibrosis or failure.

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Introducción: La angioplastia con implante de stent se considera el tratamiento de elección para la mayoría de los casos de estenosis congénita o adquirida posquirúrgica de ramas de arterias pulmonares en pacientes portadores de cardiopatías con-génitas. Sin embargo, aún persisten dudas sobre la efectividad clínica y la incidencia de complicaciones de esta técnica que necesitan ser esclarecidas. Material y métodos: Estudio multicéntrico descriptivo, retrospectivo, observacional. Los procedimientos se realizaron desde enero de 2005 hasta abril de 2015. Un total 25 pacientes fueron sometidos a angioplastia con implante de stent en ramas de arterias pulmonares, los cuales fueron seguidos evolutivamente en su estado clínico y por diferentes métodos de imágenes. Se definió buena efectividad clínica al mejoramiento clínico sintomático y/o anatómico persistente del paciente intervenido. Resultados: La cohorte de 25 pacientes tenía una edad promedio de 9,48 años (2 meses-34 años), un peso promedio de 27,54 kg (3-104) y el 44% era de sexo femenino. Las cardiopatías congénitas de base intervenidas fueron en su mayoría tetralogía de Fallot y sus variantes, seguida por corazones con fisiología univentricular poscirugías de Glenn bidireccional y/o de Fontan-Kreutzer. La presión sistólica del ventrículo derecho y de la arteria pulmonar disminuyeron significativamente posintervención (de 68,35 mm Hg a 45,8 mm Hg y de 47,4 mm Hg a 32,08 mm Hg, respectivamente) (p < 0,0001). El diámetro mínimo de la lesión a tratar se incrementó significativamente posprocedimiento (de 3,98 mm a 9,82 mm; p < 0,0001). La incidencia de complicaciones fue del 8% (2 pacientes) y se registró buena efectividad clínica en 22 pacientes (88%). Conclusiones: La angioplastia con colocación de stent en las ramas de arterias pulmonares resultó una técnica segura y eficaz, aunque desafiante, con una marcada mejoría clínica y anatómica de las lesiones tratadas. Representa una alternativa a la cirugía, con disminución del riesgo de reintervenciones en pacientes con cardiopatías congénitas.

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We describe the use of the so-called "sutureless" technique for repair of stenosis of the left pulmonary artery after bilateral banding in the management of two patients with hypoplastic left heart syndrome who had undergone initial palliation with a hybrid procedure. After bilateral pulmonary artery debanding, arterioplasty with autologous pericardial patch and sutureless technique was carried out to decrease the risk of restenosis. Both patients survived the operation, and the exit pulmonary angiography revealed adequate flow across the arterioplasty sites. Patients are alive and well at 17 and 24 months of age, respectively.

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We report a challenging case of a 32-year-old patient in New York Heart Association (NYHA) class IV with pulmonary atresia, ventricular septal defect, a left aortopulmonary collateral artery, a right modified Blalock-Taussing shunt, and a gigantic aneurysm of the ascending aorta with severe aortic valve insufficiency. A combined Rastelli and Bentall procedure was performed by a joint adult and pediatric cardiovascular surgery team. One year after the operation, the patient is in NYHA class I, working full time.

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The first successful total right heart bypass via atriopulmonary anastomosis (APA) were reported in 1971 for patients with tricuspid atresia. At the Children's Hospital of Buenos Aires, the cohort of such procedures started in July, when the first fenestrated right heart by pass was performed, with the interposition of a homograft between the right atrial appendage and the main pulmonary artery. In the second patient, instead of placing a homograft, the APA was achieved with the patient's own pulmonary root harvested from the outflow tract of the right ventricle. These techniques were soon replaced in 1978 with the development of the direct valveless posterior APA. Since the very beginning the principle was that the right atrium only functions as a pathway rather than a pump (reason why no inferior vena cava valves were ever used), and the diastolic properties of the systemic ventricle regulate the only real "pump" of this system. The late hemodynamic problems inherent of the APA diminished with modern surgical techniques like the lateral tunnel (LT) or the extracardiac conduit (EC). In spite of the improvement in prognosis and quality of life that the modern techniques have brought for univentricular hearts (UH), with the passing of time, deterioration of this system is frequently seen, due to chronic low cardiac output, elevated central venous pressure making heart transplantation the final stage of treatment. Progressive increase in pulmonary vascular resistances and ventricular dysfunction result in a decline in quality of life and survival. However, the timing of this occurrence is variable, and many survivors enjoy today a satisfactory clinical status. The challenge is to develop a better solution for UH, but in the mean time the Fontan Kreutzer palliation represents the best and only surgical option. It is undoubtedly one of the triumphs of cardiac surgery in congenital heart disease.

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We report to simultaneous use of extracorporeal life support (ECLS), plasmapheresis, hemodiafiltration and exogenous surfactant for the treatment of pulmonary hemorrhage, arterial hypertension, and renal failure secondary to nodose polyarteritis (NPA) in a 23-month-old patient. NPA is an autoimmune disease that affects small -and medium- caliber muscular arteries. Hypoxemia refractory to treatment with conventional and high frequency oscillatory ventilation was supported by ECMO while awaiting control of the autoimmune disease through the use of plasmapheresis and immunosuppressive treatment. Although the combination of ECLS with plasmapheresis is rare, it has been described during the management of cases of organ transplant, intoxication, cardiac failure, and sepsis. There are only two previous reports describing the use of this combined therapy for the treatment of pulmonary hemorrhage secondary to autoimmune vasculitis in childhood.

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Se comunica el caso de un paciente de 23 meses de edad, con hemorragia pulmonar, hipoxemia refractaria, hipertensión arterial y falla renal secundarias a poliarteritis nodosa (PN) que ha requerido asistencia simultánea con soporte cardiopulmonar extracorpóreo (ECMO), plasmaféresis, hemodiafiltración y surfactante exógeno. La PN es una vasculitis autoinmunitaria que afecta los vasos arteriales de pequeño y mediano calibres. La hipoxemia, refractaria al tratamiento con ventilación mecánica convencional y de alta frecuencia oscilatoria, fue sostenida con ECMO mientras se controló la enfermedad autoinmunitaria mediante plasmaféresis y tratamiento inmunosupresor. La combinación de ECMO con plasmaféresis es infrecuente, pero ha sido descripta en relación al trasplante de órganos, intoxicaciones, fallo cardíaco y sepsis. Existen dos casos descriptos en la bibliografía sobre el uso combinado de estas terapéuticas para el tratamiento de hemorragia pulmonar secundaria a vasculitis autoinmunitaria en niños.

We report to simultaneous use of extracorporeal life support (ECLS), plasmapheresis, hemodiafiltration and exogenous surfactant for the treatment of pulmonary hemorrhage, arterial hypertension, and renal failure secondary to nodose polyarteritis (NPA) in a 23-month-old patient. NPA is an autoimmune disease that affects small -and medium- caliber muscular arteries. Hypoxemia refractory to treatment with conventional and high frequency oscillatory ventilation was supported by ECMO while awaiting control of the autoimmune disease through the use of plasmapheresis and immunosuppressive treatment. Although the combination of ECLS with plasmapheresis is rare, it has been described during the management of cases of organ transplant, intoxication, cardiac failure, and sepsis. There are only two previous reports describing the use of this combined therapy for the treatment of pulmonary hemorrhage secondary to autoimmune vasculitis in childhood.

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