Fifth "Jatene Lecture on Surgical Innovation": Innovation in Congenital Heart Surgery: Contributions From South America.

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.

Living to Tell the Tale: A First-Person Chronicle of the Development of the Fontan-Kreutzer Procedure.

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.

Reemplazo percutáneo de válvulas cardíacas derechas con técnica "válvula en válvula" en cardiopatías congénitas: experiencia inicial en Argentina

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.

Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Experiences of parents of children with hypoplastic left heart syndrome during their treatment: a qualitative study.

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.

Technical Aspects and Common Pitfalls of Norwood With Modified Blalock Taussig Shunt.

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.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

CABG With Internal Thoracic Artery in Children With Congenital Heart Defects: A Good Option When It Is the Only One.

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.

Lymphatic Decompression Concomitant With Fontan/Kreutzer Procedure: Early Experience.

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.

A propósito de un caso pediátrico de malformación pulmonar / About a pediatric case of malformation pulmonary

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

History of the World Society for Pediatric and Congenital Heart Surgery: The First Decade.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service.

Percutaneous Transhepatic Fontan-Kreutzer Completion of Hepatic Vein Inclusion.

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.

The Lymphatic System: The Achilles Heel of the Fontan-Kreutzer Circulation.

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.

Augmentation of Bridging Leaflets in Repair of Atrioventricular Canal Defects.

Left atrioventricular (AV) valve regurgitation is the most common complication after a atrioventricular canal defect (AVCD) repair. Despite what appears to be a less complex repair, patients with partial and transitional AV canal have higher reoperation rates for left AV valve regurgitation and left ventricular outflow tract (LVOT) obstruction. Retraction of bridging leaflets with secondary attachments to the septal crest commonly produces increased tension and flattening of the medial left AV valve leaflet and LVOT obstruction after cleft closure. We describe a novel technique of detachment and patch augmentation of bridging leaflets to avoid these complications.

Free Diced Cartilage: A New Application of Diced Cartilage Grafts in Primary and Secondary Rhinoplasty.

BACKGROUND: Irregularities or deformities of the nasal dorsum after hump reduction account for a significant number of revision rhinoplasties. The authors therefore developed a technique of meticulously dicing and exactly placing free diced cartilage grafts, harvested from septum, rib, or ear cartilage. The cartilage paste is used for smoothening, augmentation, or camouflaging of the nasal dorsum in primary or revision rhinoplasties. METHODS: A retrospective analysis of multisurgeon consecutive open approach rhinoplasties from January to December of 2014 was conducted at a single center. The authors compared the outcome of three different techniques to augment or cover the nasal dorsum after an observation period of 7 months. In group I, 325 patients with free diced cartilage grafts as the only onlay were included. In group II, consisting of 73 patients, the dorsal onlay was either fascia alone or in combination with free diced cartilage grafts. Forty-eight patients in group III received a dorsal augmentation with the classic diced cartilage in fascia technique. RESULTS: Four hundred forty-six patients undergoing primary and secondary rhinoplasties in which one of the above-mentioned diced cartilage techniques was used were included in the study. The authors found revision rates for dorsal irregularities within the 7-month postoperative observation period of 5.2, 8.2, and 25 percent for groups I, II, and III, respectively. CONCLUSION: The authors' findings strongly support their clinical experience that the free diced cartilage graft technique presents an effective and easily reproducible method for camouflage and augmentation in aesthetic and reconstructive rhinoplasty.