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.

Tetralogy of Fallot Repair in Developing Countries: International Quality Improvement Collaborative.

BACKGROUND: Isolated reports from low- and middle-income countries (LMICs) for surgical results in tetralogy of Fallot (TOF) are available. The International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) seeks to improve surgical results promoting reductions in infection and mortality in LMICs. METHODS: All cases of TOF in the IQIC database performed between 2010 and 2014 at 32 centers in 20 LMICs were included. Excluded from the analysis were TOF with any associated lesions. A logistic regression analysis was performed to identify risk factors for in-hospital mortality after surgery for TOF. RESULTS: A total of 2,164 patients were identified. There were 1,839 initial primary repairs, 200 with initial systemic-to-pulmonary artery shunt, and 125 underwent secondary repair after initial palliation. Overall mortality was 3.6% (78 of 2,164), initial primary repair was 3.3% (60 of 1,839), initial systemic-to-pulmonary artery shunt was 8.0% (16 of 200), and secondary repair was 1.6% (2 of 125; p = 0.003). Major infections occurred in 5.9% (128 of 2,164) of the entire cohort. Risk factors for death after the initial primary repair were oxygen saturation less than 90% and weight/body mass index for age below the fifth percentile (p < 0.001). The initial primary repair occurred after age 1 year in 54% (991 of 1,839). Older age at initial primary repair was not a risk factor for death (p = 0.21). CONCLUSIONS: TOF patients are often operated on after age 1 year in LMICs. Unlike in developed countries, older age is not a risk factor for death. Nutritional and hypoxemic status were associated with higher mortality and infection. This information fills a critical knowledge gap for surgery in LMIC.

Morbilidad en cirugía de cardiopatìa congénita en un hospital público en Argentina / Morbidity in congenital heart surgery in a public hospital in Argentina

Objetivo: Describir las complicaciones asociadas a cirugía cardíaca, compararlas con una población de referencia e identificar factores de riesgo de mortalidad. Pacientes y métodos: Estudio retrospectivo, descriptivo. Se incluyeron todos los pacientes operados en 2013-2015 en el Hospital Garrahan. Se registró edad, peso, procedimiento, ventilación mecánica, días de internación, morbilidad y evolución. Se consideró morbilidad la insuficiencia renal con diálisis, déficit neurológico, marcapaso permanente, asistencia circulatoria, parálisis frénica o de cuerdas vocales, reoperación, infección de herida, quilotórax y traqueotomía. Se realizó un análisis estadístico descriptivo y por categorías de riesgo utilizando la escala de morbilidad de la Sociedad de Cirugía Torácica (Surgical Thoracic Society, STS). Resultados: 1536 pacientes, mediana de 12 meses (rango intercuartílico -RIC- 25-75: 3-60), peso de 8 kg (RIC25-75: de 4,4 a 17,5), con mortalidad de 5%. Se registraron 361 eventos en 183 pacientes. La reoperación no planificada fue el más frecuente (7,2%); los restantes ocurrieron en ≤ 3% de los pacientes. En comparación con los pacientes sin complicaciones, los pacientes con eventos tuvieron más días de ventilación mecánica: 9,95 (RIC25-75: 7,65-12,24) vs. 1,8 (RIC25- 75: 1,46-2,14), p< 0,00001; mayor internación: 28,8 (RIC25-75: 25,1-32,5) vs. 8,5 (RIC25-75: 7,99,2), p <0,0001; y mayor mortalidad: 19,6% vs. 3,1%(RR 4,58;IC95%: de 3,4 a 6,0), p <0,0001. La asistencia circulatoria e insuficiencia renal se asociaron con mayor mortalidad. Conclusiones: La reoperación no planificada fue el evento más frecuente. Los pacientes con complicaciones tuvieron más días de ventilación mecánica, de internación y mayor mortalidad. La asistencia circulatoria y la insuficiencia renal se asociaron con mayor mortalidad.

Objective: To describe the complications associated with heart surgery, compare them to a reference population, and identify mortality risk factors. Patients and methods: Retrospective and descriptive study. All patients who underwent surgery at Hospital Garrahan in the 2013-2015 period were included. Age, weight, procedure, mechanical ventilation, length of stay in days, morbidity, and course were recorded. Renal failure requiring dialysis, neurological deficit, permanent pacemaker, circulatory support, phrenic nerve or vocal cord palsy, reoperation, wound infection, chylothorax, and tracheotomy were considered morbidities. A descriptive, statistical analysis by risk category was done using the Society of Thoracic Surgeons (STS) morbidity score. Results: 1536 patients, median age: 12 months (interquartile range --#91;IQR--#93; 25-75: 3-60), weight: 8 kg (IQR 25-75: 4.4 to 17.5), mortality: 5%. A total of 361 events were recorded in 183 patients. An unplanned reoperation was the most common event (7.2%); the rest occurred in < 3% of patients. Compared to patients without complications, patients who had events required more days on mechanical ventilation: 9.95 (IQR 25-75: 7.6512.24) versus 1.8 (IQR 2575: 1.46-2.14), p< 0.00001; a longer length of stay: 28.8 (IQR 25-75: 25.1-32.5) versus 8.5 (IQR 25-75: 7.9-9.2), p< 0.0001; and had a higher mortality: 19.6% versus 3.1% (RR: 4.58, 95% CI: 3.4 to 6.0), p< 0.0001. Circulatory support and renal failure were associated with a higher mortality. Conclusions: An unplanned reoperation was the most common event. Patients with complications required more days on mechanical ventilation and a longer length of stay and had a higher mortality. Circulatory support and renal failure were associated with a higher mortality.

Morbidity in congenital heart surgery in a public hospital in Argentina. / Morbilidad en cirugía de cardiopatìa congénita en un hospital público en Argentina.

OBJECTIVE: To describe the complications associated with heart surgery, compare them to a reference population, and identify mortality risk factors. PATIENTS AND METHODS: Retrospective and descriptive study. All patients who underwent surgery at Hospital Garrahan in the 2013-2015 period were included. Age, weight, procedure, mechanical ventilation, length of stay in days, morbidity, and course were recorded. Renal failure requiring dialysis, neurological deficit, permanent pacemaker, circulatory support, phrenic nerve or vocal cord palsy, reoperation, wound infection, chylothorax, and tracheotomy were considered morbidities. A descriptive, statistical analysis by risk category was done using the Society of Thoracic Surgeons (STS) morbidity score. RESULTS: 1536 patients, median age: 12 months (interquartile range [IQR] 25-75: 3-60), weight: 8 kg (IQR 25-75: 4.4 to 17.5), mortality: 5%. A total of 361 events were recorded in 183 patients. An unplanned reoperation was the most common event (7.2%); the rest occurred in < 3% of patients. Compared to patients without complications, patients who had events required more days on mechanical ventilation: 9.95 (IQR 25-75: 7.6512.24) versus 1.8 (IQR 2575: 1.46-2.14), p< 0.00001; a longer length of stay: 28.8 (IQR 25-75: 25.1-32.5) versus 8.5 (IQR 25-75: 7.9-9.2), p< 0.0001; and had a higher mortality: 19.6% versus 3.1% (RR: 4.58, 95% CI: 3.4 to 6.0), p< 0.0001. Circulatory support and renal failure were associated with a higher mortality. CONCLUSIONS: An unplanned reoperation was the most common event. Patients with complications required more days on mechanical ventilation and a longer length of stay and had a higher mortality. Circulatory support and renal failure were associated with a higher mortality.

OBJETIVO: Describir las complicaciones asociadas a cirugía cardíaca, compararlas con una población de referencia e identificar factores de riesgo de mortalidad. PACIENTES Y MÉTODOS: Estudio retrospectivo, descriptivo. Se incluyeron todos los pacientes operados en 2013-2015 en el Hospital Garrahan. Se registró edad, peso, procedimiento, ventilación mecánica, días de internación, morbilidad y evolución. Se consideró morbilidad la insuficiencia renal con diálisis, déficit neurológico, marcapaso permanente, asistencia circulatoria, parálisis frénica o de cuerdas vocales, reoperación, infección de herida, quilotórax y traqueotomía. Se realizó un análisis estadístico descriptivo y por categorías de riesgo utilizando la escala de morbilidad de la Sociedad de Cirugía Torácica (Surgical Thoracic Society, STS). RESULTADOS: 1536 pacientes, mediana de 12 meses (rango intercuartílico -RIC- 25-75: 3-60), peso de 8 kg (RIC25-75: de 4,4 a 17,5), con mortalidad de 5%. Se registraron 361 eventos en 183 pacientes. La reoperación no planificada fue el más frecuente (7,2%); los restantes ocurrieron en ≤ 3% de los pacientes. En comparación con los pacientes sin complicaciones, los pacientes con eventos tuvieron más días de ventilación mecánica: 9,95 (RIC25-75: 7,65-12,24) vs. 1,8 (RIC25- 75: 1,46-2,14), p< 0,00001; mayor internación: 28,8 (RIC25-75: 25,1-32,5) vs. 8,5 (RIC25-75: 7,99,2), p <0,0001; y mayor mortalidad: 19,6% vs. 3,1%(RR 4,58;IC95%: de 3,4 a 6,0), p <0,0001. La asistencia circulatoria e insuficiencia renal se asociaron con mayor mortalidad. CONCLUSIONES: La reoperación no planificada fue el evento más frecuente. Los pacientes con complicaciones tuvieron más días de ventilación mecánica, de internación y mayor mortalidad. La asistencia circulatoria y la insuficiencia renal se asociaron con mayor mortalidad.

Global perspective on training and staffing for paediatric cardiac critical care.

This manuscript provides a global perspective on physician and nursing education and training in paediatric cardiac critical care, including available resources and delivery of care models with representatives from several regions of the world including Africa, Israel, Asia, Australasia, Europe, South America, and the United States of America.

Series de Pediatría Garrahan. El niño con cardiopatía congénita

A 27 años del nacimiento del Hospital Garrahan, hemos sentido la necesidad de compartir con el equipo de salud las estrategias de atención del niño y su familia utilizadas en nuestro Hospital, colaborar con su actualización permanente y poner énfasis en el trabajo interdisciplinario. En este tomo desarrollamos el tema del niño con cardiopatía congénita. Se trata de la malformación congénita grave más frecuente, que se presenta en aproximadamente 5 a 8 cada mil nacidos vivos. En Argentina, nacen alrededor de 700.000 niños cada año, es decir alrededor de 4.500 con esta patología. La supervivencia actual con calidad de vida futura normal es alta para la mayoría de estos niños. A esto contribuyen las mejoras en el diagnóstico prenatal y neonatal temprano, el traslado seguro, la estabilización preoperatoria y el tratamiento por equipos cardiovasculares interdisciplinarios. El rol del pediatra es fundamental en el diagnóstico y seguimiento de estos pacientes. En este volumen, el pediatra encontrará aspectos epidemiológicos y patogénicos de algunas de las enfermedades más prevalentes, así como claves para el diagnóstico oportuno, con énfasis en la semiología y los estudios complementarios accesibles, indicaciones de derivación, prevención de riesgos y asesoramiento general para pacientes y padres.

Asistencia circulatoria mecánica en pediatría: Experiencia en el Hospital de Pediatría Dr. Juan P. Garrahan. Argentina / Mechanical circulatory support in pediatrics: Experience at the Hospital de Pediatría Dr. Juan P. Garrahan. Argentina

Introducción: La asistencia circulatoria mecánica permite aportar oxígeno a los tejidos en pacientes con enfermedades cardiacas y/o respiratorias reversibles refractarios a tratamientos convencionales. Objetivo: Mostrar los resultados iniciales de asistencia circulatoria mecánica en niños con cardiopatía en nuestra institución. Método: Estudio de cohorte descriptivo retrospectivo entre marzo de 2006 y marzo de 2012. Datos demográficos (edad, sexo, peso, diagnóstico cardiológico), de la cirugía (técnica, tiempo de bomba y de pinzamiento aórtico) y de la asistencia circulatoria mecánica (tipo, indicación, duración, complicaciones y evolución). Resultados: Fueron asistidos 33 pacientes (1.3% del total de cirugías), con oxigenación por membrana extracorpórea en 32 casos y un caso con dispositivo de asistencia ventricular. Mediana de edad 7.4 meses (un día-18 años) y peso 6 kg (2.3-75). Las malformaciones cardiacas que se asistieron con mayor frecuencia son las trasposiciones de grandes arterias asociadas a otras anomalías y las trasposiciones corregidas (inversión ventricular-doble discordancia). El motivo de ingreso más frecuente fue disfunción biventricular poscardiotomía. En 28 pacientes la asistencia circulatoria mecánica fue postoperatoria y en 4 preoperatoria. Un paciente presentó miocarditis. Mediana de asistencia 3 días (1-10). Complicaciones más frecuentes: infección el 21% y sangrado el 21%. Decanulación electiva en un 94% de los casos. Supervivencia con alta hospitalaria: 52%. Conclusiones: La asistencia circulatoria mecánica en nuestra institución es una herramienta segura con procedimiento estandarizado. Se utiliza en un reducido número de casos, con supervivencia similar a informes internacionales. Es un procedimiento complejo, plenamente justificado que permitió la recuperación en más de la mitad de los pacientes que de otro modo hubieran fallecido.

Introduction: Mechanical circulatory support provides oxygen to the tissues in patients with cardiac and/or respiratory reversible disease refractory to conventional treatments. Objective: The aim of this study is to show our initial results of mechanical circulatory support in children with heart disease. Method: Retrospective cohort between March 2006 and March 2012. Demographic data (age, sex, weight, cardiac diagnosis), surgery (technique, pump, aortic cross clamping time) and mechanical circulatory support (type of assistance, indication, duration, complications and outcome) were collected. Results: Thirty-three patients were supported (1.3% of all surgeries), extracorporeal membrane oxygenation 32 cases and one ventricular assist device. The median age 7.4 months (one day-18 years) and weight 6 kg (2.3-75). The most frequent cardiac malformations supported were the transpositions of the great arteries associated with other anomalies and the corrected transpositions (ventricular inversion or double discordance). The most common reason for admission was post-cardiotomy biventricular dysfunction. Twenty-eight patients were supported in the postoperative period, 4 in the preoperative period and in one with myocarditis. Median days of support were 3 days (1-10). The most common complications were infection (21%), bleeding (21%). Elective decannulation was achieved in 94% of cases. Hospital discharge survival: 52%. Conclusions: The mechanical circulatory support in our institution is a safe and standard procedure. We have been using it in a small number of cases with a similar survival to that reported internationally. This complex procedure is widely justified because it allows for the recovery of more than half of the patients who otherwise would have died.

[Mechanical circulatory support in pediatrics. Experience at the Dr. Juan P. Garrahan Pediatric Hospital. Argentina]. / Asistencia circulatoria mecánica en pediatría. Experiencia en el Hospital de Pediatría Dr. Juan P. Garrahan. Argentina.

INTRODUCTION: Mechanical circulatory support provides oxygen to the tissues in patients with cardiac and/or respiratory reversible disease refractory to conventional treatments. OBJECTIVE: The aim of this study is to show our initial results of mechanical circulatory support in children with heart disease. METHOD: Retrospective cohort between March 2006 and March 2012. Demographic data (age, sex, weight, cardiac diagnosis), surgery (technique, pump, aortic cross clamping time) and mechanical circulatory support (type of assistance, indication, duration, complications and outcome) were collected. RESULTS: Thirty-three patients were supported (1.3% of all surgeries), extracorporeal membrane oxygenation 32 cases and one ventricular assist device. The median age 7.4 months (one day-18 years) and weight 6kg (2.3-75). The most frequent cardiac malformations supported were the transpositions of the great arteries associated with other anomalies and the corrected transpositions (ventricular inversion or double discordance). The most common reason for admission was post-cardiotomy biventricular dysfunction. Twenty-eight patients were supported in the postoperative period, 4 in the preoperative period and in one with myocarditis. Median days of support were 3 days (1-10). The most common complications were infection (21%), bleeding (21%). Elective decannulation was achieved in 94% of cases. Hospital discharge survival: 52%. CONCLUSIONS: The mechanical circulatory support in our institution is a safe and standard procedure. We have been using it in a small number of cases with a similar survival to that reported internationally. This complex procedure is widely justified because it allows for the recovery of more than half of the patients who otherwise would have died.

[Congenital heart disease: surgical results in a public hospital in Argentina]. / Cardiopatías congénitas: resultados quirúrgicos en un hospital público en Argentina.

OBJECTIVE: To describe the results of congenital heart surgery at the Hospital de Pediatría J. P.Garrahan. METHODS: Between 2004 and 2009, 2942 patients, median age 2.5 years (1 d to 22.5 y), median weight 11.5 kg (1.6 kg to 96 kg), and 84% with cardiopulmonary bypass, were analyzed. Adjusted mortality risk analysis using RACHS and Aristotle score was performed. Newborn surgery and one stage repair in Fallot and ventricular septal defect with coarctation were analyzed as subgroupes. RESULTS: Mortality was 5.5%. Required mechanical ventilation 45%, 5% peritoneal dialysis, 12% delayed sternal closure and 8.4% reoperation. Twenty five percent with previous surgery, and 10% in poor clinical conditions. RACHS-1 categories morality distribution were 0.4% in one, 2.4% in two, 7.1% in three, 14% in four y 34 % in 5 and 6. One stage repair was performed in 84% of Fallots and 90% of ventricular septal defects with coarctation, with 3.2% and 10% mortality rate respectively. Two hundred and five newborns were operated with cardiopulmonary bypass with a mortality rate of 15% in the last year. CONCLUSIONS: Almost every congenital heart disease can be repaired without previous palliation, with satisfactory results in our institution. Poor clinical conditions significantly increased morbidity and mortality.

Cardiopatías congénitas: resultados quirúrgicos en un hospital público en Argentina / Congenital heart disease: surgical results in a public hospital in Argentina

Objetivo: Describir los resultados en cirugía cardiovascular del Hospital de Pediatría J. P. Garrahan. Métodos: Se analizaron 2942 pacientes entre 2004 y 2009; de los cuales, 84% con circulación extracorpórea, mediana de edad 2.5 años, rango de un día a 22.5 años y peso 11.5 kg (1.6 kg a 96 kg); con cirugías previas 25% y 10% malas condiciones preoperatorias. Se analizó la mortalidad por RACHS-1 y Aristóteles, la cirugía neonatal, la cirugía en un tiempo de Fallot y de comunicación interventricular con coartación de aorta. Resultados: La mortalidad fue 5.5%. Requirió ventilación mecánica 45%, 5% diálisis peritoneal, 12% cierre esternal diferido y 8.4% reoperación. De los Fallot 84% y 90% de las comunicaciones interventriculares con coartación se repararon en un tiempo, con 3.2% y 10% de mortalidad respectivamente. Se operaron 205 neonatos con bomba y con 15% de mortalidad en el último año. Las malas condiciones previas aumentaron la morbilidad, (Odds ratio 3.63 IC 95%, 2.27 -4.81) y la mortalidad (Odds ratio 6.47 IC 95%, 4.36 - 9.60). La mortalidad por RACHS fue 0.4% en categoría uno, 2.4% en la dos, 7.1% en la tres, 14% en la cuatro y 34% en la cinco y seis. Conclusiones: En nuestra institución la mayoría de las cardiopatías se resuelven en un tiempo con resultados satisfactorios. Las malas condiciones preoperatorias aumentan significativamente la morbimortalidad.

Objective: To describe the results of congenital heart surgery at the Hospital de Pediatría J. P.Garrahan. Methods: Between 2004 and 2009, 2942 patients, median age 2.5 years (1 d to 22.5 y), median weight 11.5 kg (1.6 kg to 96 kg), and 84% with cardiopulmonary bypass, were analyzed. Adjusted mortality risk analysis using RACHS and Aristotle score was performed. Newborn surgery and one stage repair in Fallot and ventricular septal defect with coarctation were analyzed as subgroupes. Results: Mortality was 5.5%. Required mechanical ventilation 45%, 5% peritoneal dialysis, 12% delayed sternal closure and 8.4% reoperation. Twenty five percent with previous surgery, and 10% in poor clinical conditions. RACHS-1 categories morality distribution were 0.4% in one, 2.4% in two, 7.1% in three, 14% in four y 34 % in 5 and 6. One stage repair was performed in 84% of Fallots and 90% of ventricular septal defects with coarctation, with 3.2% and 10% mortality rate respectively. Two hundred and five newborns were operated with cardiopulmonary bypass with a mortality rate of 15% in the last year. Conclusions: Almost every congenital heart disease can be repaired without previous palliation, with satisfactory results in our institution. Poor clinical conditions significantly increased morbidity and mortality.

[Functional univentricular heart: immediate and long term results, in the different stages of sequential correction]. / Corazón univentricular funcional: resultados inmediatos y alejados, en las distintas etapas de corrección secuencial.

INTRODUCTION: The functionally univentricular heart represents an heterogeneous group of anomalies that have in common the feature that only one of the chambers within the ventricular mass is capable of supporting independently either the pulmonary or systemic circulation. OBJECTIVE: Evaluation from the neonatal period of the immediate and long term surgical results of the sequential total cavo-pulmonary connection in the various anatomical forms of the functional univentricular hearts. METHODS: From May 1998 to May 2009, 84 patients have been followed since the neonatal period and in a prospective retro-prospective way (bidirectional), in which 181 sequential surgical procedures were performed in three stages: Neonatal, Bidirectional Glenn and Total cava pulmonary connection. We analyzed the following variables: age, performed surgical procedures, risk stratification (RACHS), surgical and inter stage mortality and complications in the follow up. The categorical variables are reported as percentages with their confident interval (CI) 95%, the quantitative variables as median ratio. A Kaplan Meyer survival curve was performed. RESULTS: A total of 181 procedures were screened. Stage 1: 68 patients, median age 5.5 days (r = 2-60) mortality 14.7% (CI95: 6.3%-23%). Stage 2: 70 patients, median age 4 month (r= 3-12), mortality 1.4% (CI95 0%-4.1%) Stage 3: 43 patients, median age 3 years (r = 2-6), mortality 9,3% (CI95%: 0.7%-18%). The interstage mortality was 11.9% (CI95: 5.1%- 8.8%). The total mortality was 29.7% (CI95 19.3% -38.7%). The mortality by RACHS: RACHS 2:1.6% (CI95: 0%-4.7%); RACHS 3: 5,5% (CI95: 0.3%-10.7%); RACHS 4: 0% and RACHS 6: 23.8% (CI95: 10.9%- 35.1%).Survival index at 10 years 69% CONCLUSIONS: The sequential surgical approach from the neonatal period has changed the natural history of this entity with reasonable survival rate and mortality according to RACHS. The medium term follow up is encouraging and may improve in the future.

Corazón univentricular funcional: resultados inmediatos y alejados, en las distintas etapas de corrección secuencial / Functional univentricular heart: immediate and long term results, in the different stages of sequential correction

Introducción: El corazón univentricular funcional abarca un heterogéneo grupo de entidades anatómicas que tienen en común la característica de que uno de los ventrículos es incapaz de mantener independientemente la circulación sistémica o pulmonar. Objetivo: Evaluar, desde el periodo neonatal, los resultados quirúrgicos inmediatos y a largo plazo del enfoque terapéutico secuencial del corazón univentricular funcional. Método: Desde mayo de 1998 a mayo de 2009, se incluyeron 84 pacientes seguidos en forma prospectiva retrospectiva (bidireccional) desde el periodo neonatal. El total de procedimientos quirúrgicos fue de 181. Se realizó un enfoque terapéutico secuencial en tres estadios: neonatal, Glenn bidireccional, y Cavo-pulmonar total. Las variables analizadas fueron: edad, procedimientos realizados, estratificación de riesgo según RACHS, mortalidad por estadio e interestadío y complicaciones en el seguimiento. Las variables cuantitativas se presentan como mediana (rango); las variables categóricas como porcentajes, con sus respectivos intervalos de confianza (IC) 95%. Se realizó curva de supervivencia de Kaplan-Meier. Resultados: Del total de 181 procedimientos se operaron: Estadio 1: 68 pacientes, edad mediana: 5.5 días (r = dos a 60), mortalidad: 14.7% (IC 95: 6.3% a 23%). Estadio 2: 70 pacientes, edad mediana: cuatro meses (r = tres a 12), mortalidad: 1.4% (IC 95: 0% a 4.1%). Estadio 3: 43 pacientes, edad mediana: tres años (r = 2-6), mortalidad: 9.3% (IC 95: 0.7%-18%). Mortalidad interestadios: 11.9% (IC95: 5.1% a 18.9%). Mortalidad total: 29.76% (IC 95: 19.3% a 38.7%). La mortalidad según RACHS fue: RACHS 2: 1.6°% (IC 95°%: 0°% a 4.7°%) RACHS 3: 5.5°% (IC 95 0.3°% a 10,7°%), RACHS 4: 0% y RACHS 6: 23.8% (IC 95%: 10.9% a 35,1%). Índice de sobrevida a 10 años: 69%. Conclusiones: El enfoque quirúrgico secuencial, desde el período neonatal, ha cambiado la historia natural de esta entidad, con índices de sobrevida aceptables y mortalidad acorde a la estratificación de riesgo (RACHS). El seguimiento a mediano plazo es alentador y mejorable en el futuro.

Introduction: The functionally univentricular heart represents an heterogeneous group of anomalies that have in common the feature that only one of the chambers within the ventricular mass is capable of supporting independently either the pulmonary or systemic circulation. Objective: Evaluation from the neonatal period of the immediate and long term surgical results of the sequential total cavo-pulmonary connection in the various anatomical forms of the functional univentricular hearts. Methods: From May 1998 to May 2009, 84 patients have been followed since the neonatal period and in a prospective retro-prospective way (bidirectional), in which 181 sequential surgical procedures were performed in three stages: Neonatal, Bidirectional Glenn and Total cava pulmonary connection. We analyzed the following variables: age, performed surgical procedures, risk stratification (RACHS), surgical and inter stage mortality and complications in the follow up. The categorical variables are reported as percentages with their confident interval (CI) 95%, the quantitative variables as median ratio. A Kaplan Meyer survival curve was performed. Results: A total of 181 procedures were screened. Stage 1: 68 patients, median age 5.5 days (r = 2-60) mortality 14.7% (CI95: 6.3%-23%). Stage 2: 70 patients, median age 4 month (r= 3-12), mortality 1.4% (CI95 0%-4.1%) Stage 3: 43 patients, median age 3 years (r = 2-6), mortality 9,3°% (CI95%: 0.7%-18%). The interstage mortality was 11.9°% (CI95: 5.1%-18.8%). The total mortality was 29.7% (CI95 19.3% -38.7%). The mortality by RACHS: RACHS 2:1.6% (CI95: 0%-4.7%); RACHS 3: 5,5% (CI95: 0.3%-10.7%); RACHS 4: 0% and RACHS 6: 23.8% (CI95: 10.9%-35.1%).Survival index at 10 years 69% Conclusions: The sequential surgical approach from the neonatal period has changed the natural history of this entity with reasonable survival rate and mortality according to RACHS. The medium term follow up is encouraging and may improve in the future.

[Cardiac surgical repair in newborns: five years' experience in neonatal open surgery]. / Cirugía cardíaca reparadora en recién nacidos: experiencia de 5 años en cirugía neonatal con circulación extracorpórea.

INTRODUCTION: Four hundred newborns die every year in our country suffering from congenital heart disease. Definitive surgical repair, whenever possible, is nowadays the optimal therapeutic strategy. Our goal is to describe mortality and morbidity in neonatal surgery with cardiopulmonary bypass in a tertiary public hospital in Argentina. PATIENTS AND METHODS: Descriptive, retrospective study. Every patient, younger than 45 days, with cardiac surgery requiring cardiopulmonary bypass, at the Garrahan Hospital between 2004 and 2008 was included. Demographic, surgical and postoperative data were collected. Adjusted mortality risk analysis, and descriptive statistics from the most frequent diagnosis were performed. Results are expressed as median and rank or percentage. RESULTS: 200 newborns were operated, 62% males. Median age was 21 days (r 1- 45) and median weight 3.1 kg (r 1.6-6.2). Total anomalous pulmonary venous return, transposition of great arteries an hypoplastic left heart syndrome diagnoses accounted for 75% of the procedures. Median length of stay was 12 days (r 0-191), and 6 days of mechanical ventilation (r 0-180). Eighteen percent of the patients required peritoneal dialysis. Whole series mortality was 19% and fell to 14% in 2008. Unstable preoperative condition and postoperative complications increased mortality, OR= 2.23 (1.02-4.89) and OR= 10 (3.6-33.4), respectively. CONCLUSIONS: Our postoperative mortality is similar to those reported in foreign countries databases. Patients with unstable preoperative condition and post-operative complications had higher mortality.

Cirugía cardíaca reparadora en recién nacidos: experiencia de 5 años en cirugía neonatal con circulación extracorpórea / Cardiac surgical repair in newborns: five years experience in neonatal open surgery

Introducción. En nuestro país fallecen anualmente 400 niños con cardiopatía congénita, en el 1er mes de vida. La cirugía constituye la estrategia actual para su tratamiento. El objetivo es describir la mortalidad hospitalaria y las complicaciones de la cirugía cardíaca neonatal con circulación extracorpórea en un hospital público de alta complejidad. Pacientes y métodos. Estudio descriptivo retrospectivo. Se registraron datos demográficos, de la cirugía y del postoperatorio de los pacientes con circulación extracorpórea, menores de 45 días, operados en el Hospital Garrahan, entre 2004-2008. Se realizó un análisis por patologías y de mortalidad ajustado por riesgo. Los resultados se expresan en mediana e intervalo, o en porcentaje, según corresponda. Resultados. Se operaron 200 recién nacidos, 62 por ciento varones. La edad fue 21 (1- 45) días y el peso 3,1 (1,6-6,2) kg. Anomalía total de retorno venoso pulmonar, transposición de grandes arterias y síndrome de corazón izquierdo hipoplásico concentraronel 75 por ciento de las cirugías. El tiempo de internación fue 12 (0-191) días, con 6 (0-180) días de ventilación mecánica, y 18 por ciento de los pacientes requirieron diálisis. La mortalidad global fue 19 por ciento y 14 por ciento en el último año analizado. Los pacientes en mala condición preoperatoria, así como los que presentaron complicaciones, exhibieron una mortalidad mayor (OR= 2,23 [1,02-4,89] y OR= 10 [3,6-33,4], respectivamente). Conclusiones. La mortalidad postoperatoria es similar a la comunicada en bases de datos del exterior. Los pacientes con mala condición preoperatoria y aquellos con complicaciones presentaron mayor mortalidad en nuestra serie.

Introduction. Four hundred newborns die every year in our country suffering from congenital heart disease. Definitive surgical repair, whenever possible, is nowadays the optimal therapeutic strategy. Our goal is to describe mortality and morbidity in neonatal surgery with cardiopulmonary bypass in a tertiary public hospital in Argentina. Patients and methods. Descriptive, retrospective study. Every patient, younger than 45 days, with cardiac surgery requiring cardiopulmonary bypass, at the Garrahan Hospital between 2004 and 2008 was included. Demographic, surgical and postoperative data were collected. Adjusted mortality risk analysis, and descriptive statistics from the most frequent diagnosis were performed. Results are expressed as median and rank or percentage. Results. 200 newborns were operated, 62 percent males. Median age was 21 days (r 1- 45) and median weight 3.1 kg (r 1.6-6.2). Total anomalous pulmonary venous return, transposition of great arteries and hypoplastic left heart syndrome diagnoses accounted for 75 percent of the procedures. Median length of stay was 12 days (r 0-191), and 6 days of mechanical ventilation (r 0-180). Eighteen percent of the patients required peritoneal dialysis. Whole series mortality was 19 percent and fell to 14 pecent in 2008. Unstable preoperative condition and postoperative complications increased mortality, OR= 2.23 (1.02-4.89) and OR= 10 (3.6-33.4), respectively. Conclusions. Our postoperative mortality is similar to those reported in foreign countries databases. Patients with unstable preoperative condition and post-operative complications had higher mortality.

Cirugía cardíaca reparadora en recién nacidos: experiencia de 5 años en cirugía neonatal con circulación extracorpórea / Cardiac surgical repair in newborns: five years experience in neonatal open surgery

Introducción. En nuestro país fallecen anualmente 400 niños con cardiopatía congénita, en el 1er mes de vida. La cirugía constituye la estrategia actual para su tratamiento. El objetivo es describir la mortalidad hospitalaria y las complicaciones de la cirugía cardíaca neonatal con circulación extracorpórea en un hospital público de alta complejidad. Pacientes y métodos. Estudio descriptivo retrospectivo. Se registraron datos demográficos, de la cirugía y del postoperatorio de los pacientes con circulación extracorpórea, menores de 45 días, operados en el Hospital Garrahan, entre 2004-2008. Se realizó un análisis por patologías y de mortalidad ajustado por riesgo. Los resultados se expresan en mediana e intervalo, o en porcentaje, según corresponda. Resultados. Se operaron 200 recién nacidos, 62 por ciento varones. La edad fue 21 (1- 45) días y el peso 3,1 (1,6-6,2) kg. Anomalía total de retorno venoso pulmonar, transposición de grandes arterias y síndrome de corazón izquierdo hipoplásico concentraronel 75 por ciento de las cirugías. El tiempo de internación fue 12 (0-191) días, con 6 (0-180) días de ventilación mecánica, y 18 por ciento de los pacientes requirieron diálisis. La mortalidad global fue 19 por ciento y 14 por ciento en el último año analizado. Los pacientes en mala condición preoperatoria, así como los que presentaron complicaciones, exhibieron una mortalidad mayor (OR= 2,23 [1,02-4,89] y OR= 10 [3,6-33,4], respectivamente). Conclusiones. La mortalidad postoperatoria es similar a la comunicada en bases de datos del exterior. Los pacientes con mala condición preoperatoria y aquellos con complicaciones presentaron mayor mortalidad en nuestra serie.(AU)

Introduction. Four hundred newborns die every year in our country suffering from congenital heart disease. Definitive surgical repair, whenever possible, is nowadays the optimal therapeutic strategy. Our goal is to describe mortality and morbidity in neonatal surgery with cardiopulmonary bypass in a tertiary public hospital in Argentina. Patients and methods. Descriptive, retrospective study. Every patient, younger than 45 days, with cardiac surgery requiring cardiopulmonary bypass, at the Garrahan Hospital between 2004 and 2008 was included. Demographic, surgical and postoperative data were collected. Adjusted mortality risk analysis, and descriptive statistics from the most frequent diagnosis were performed. Results are expressed as median and rank or percentage. Results. 200 newborns were operated, 62 percent males. Median age was 21 days (r 1- 45) and median weight 3.1 kg (r 1.6-6.2). Total anomalous pulmonary venous return, transposition of great arteries and hypoplastic left heart syndrome diagnoses accounted for 75 percent of the procedures. Median length of stay was 12 days (r 0-191), and 6 days of mechanical ventilation (r 0-180). Eighteen percent of the patients required peritoneal dialysis. Whole series mortality was 19 percent and fell to 14 pecent in 2008. Unstable preoperative condition and postoperative complications increased mortality, OR= 2.23 (1.02-4.89) and OR= 10 (3.6-33.4), respectively. Conclusions. Our postoperative mortality is similar to those reported in foreign countries databases. Patients with unstable preoperative condition and post-operative complications had higher mortality.(AU)

[Levosimendan, a new inotropic drug: experience in children with acute heart failure]. / Levosimendán, un nuevo agente inotrópico: experiencia en niños con fallo cardíaco agudo.

INTRODUCTION: Low cardiac output syndrome occurs frequently in pediatric patients after cardiac surgery. Catecholamines are used as inotropic drugs to treat this threatening condition, but may cause undesirable and potentially harmful side effects. This study was performed to evaluate the efficacy and safety of levosimendan (LEVO) in pediatric patients with low cardiac output syndrome. PATIENTS AND METHODS: Open prospective, quasi-experimental cohort. LEVO was given as compassionate treatment in patients with refractory post-surgical low cardiac output syndrome. Every patient received an IV infusion of LEVO at 6 microg/kg during a fifteen minutes period, followed by a 24 h IV infusion at 0.1 microg/kg/min. Clinical improvement of cardiac output was the primary end point of the study. Two independent observers performed clinical evaluation, bidimensional echocardiogram, hemodynamic and laboratory tests were performed pre and after LEVO infusion. RESULTS: LEVO was infused in 18 opportunities (fourteen children). The response was considered successful in 9/18 interventions (50%; p= 0.004). Both inotropic score (12.1 vs. 6,1, p= 0.01) and A-VDO(2)2 (26.78 +/- 11.5% vs. 20.81 +/- 7.72%, p= 0.029) showed reduction, while SvO2 improved (69.5 +/- 11.4% vs. 76 +/- 9.29%, p= 0.03). No adverse effects were noticed. Four patients died, none of them related to LEVO administration. CONCLUSIONS: LEVO improved cardiac output in 50% of the interventions with post-surgical LCOS and no adverse effect was observed.

Levosimendán, un nuevo agente inotrópico: experiencia en niños con fallo cardíaco agudo / Levosimendan, a new inotropic drug: experience in children with acute heart failure

Introducción. El síndrome de bajo gasto cardíaco ocurre frecuentemente en el posoperatorio de pacientes con cirugía cardíaca. Las catecolaminas, utilizadas como inotrópicos, pueden provocar efectos adversos potencialmente deletéreos. El levosimendán es un nuevo fármaco inotrópico no adrenérgico con posibles beneficios en esta poblaciónde pacientes. Este estudio evaluó la seguridad y eficacia del levosimendán en niños con bajo gasto posoperatorio.Población, material y métodos. Estudio prospectivo, abierto, no controlado, realizado en la Unidad de Recuperación Cardiovascular de un hospital pediátrico de alta complejidad. Se administrólevosimendán, como uso compasivo, a todos los pacientes con bajo gasto posoperatorio refractario al tratamiento convencional. Se administró una dosisde carga de 6 μg/kg EV, seguido de 0,1 μg/kg/min por 24 h. La variable primaria predeterminada fue el impacto clínico del levosimendán sobre el gastocardíaco. Dos observadores independientes realizaron la evaluación clínica del gasto cardíaco. Se analizó la función cardíaca por ecocardiografía yvariables clínicas, bioquímicas y hemodinámicas antes de la infusión y después de ella. Resultados. Se administró levosimendán a 14 pacientesen 18 oportunidades La mediana de edadfue de 27,5 meses (r: 0,16-197) y el peso de 11,1 kg (r: 2,98-48). En 9/18 intervenciones (50%; p= 0,004) seobservó una mejoría en el gasto cardiaco. El puntaje de inotrópicos disminuyó (12,1 contra 6,1; p= 0,01),la SvO2 mejoró (69,5 ± 11,4% contra 76 ± 9,29%, p= 0,03) y la A-VDO2 disminuyó (26,78 ± 11,5% contra 20,81 ± 7,72%, p= 0,029). No se identificaron efectos adversos. Cuatro pacientes fallecieron, ninguno en relación con la administración del fármaco. Conclusiones. En el 50% de las intervenciones se observó mejoría en el gasto cardíaco. No se detectaron efectos adversos atribuibles al fármaco.

Introduction. Low cardiac output syndrome occurs frequently in pediatric patients after cardiac surgery. Catecholamines are used as inotropic drugs to treat this threatening condition, but may cause undesirable and potentially harmfull side effects. Thisstudy was performed to evaluate the efficacy and safety of levosimendan (LEVO) in pediatric patients with low cardiac output syndrome. Patients and methods. Open prospective, cuasi experimental, cohort. LEVO was given as compassionate treatment in patients with refractory possurgical low cardiac output syndrome. Every patient received an IV infusion of LEVO at 6 μg/kg during a fifteen minutes period, followed by a 24 h IV infusion at 0.1 μg/kg/min. Clinical improvement of cardiac output was the primary end point of the study. Two independent observers performedclinical evaluation, bidimensional echocardiogram, hemodinamic and laboratory tests were performed pre and after LEVO infusión. Results. LEVO was infused in 18 oportunities (fourteenchildren). The response was considered successful in 9/18 interventions (50%; p= 0.004). Both inotropic score (12.1 vs. 6,1, p= 0.01) and A-VDO22 (26.78 ± 11.5% vs. 20.81 ± 7.72%, p= 0.029) showed reduction, while SvO2 improved (69.5 ± 11.4% vs. 76 ± 9.29%, p= 0.03). No adverse effects were noticed.Four patients died, none of them related to LEVO administration. Conclusions. LEVO improved cardiac output in 50% of the interventions with pos-surgical LCOS and no adverse effect was observed.

Levosimendán, un nuevo agente inotrópico: experiencia en niños con fallo cardíaco agudo / Levosimendan, a new inotropic drug: experience in children with acute heart failure

Introducción. El síndrome de bajo gasto cardíaco ocurre frecuentemente en el posoperatorio de pacientes con cirugía cardíaca. Las catecolaminas, utilizadas como inotrópicos, pueden provocar efectos adversos potencialmente deletéreos. El levosimendán es un nuevo fármaco inotrópico no adrenérgico con posibles beneficios en esta poblaciónde pacientes. Este estudio evaluó la seguridad y eficacia del levosimendán en niños con bajo gasto posoperatorio.Población, material y métodos. Estudio prospectivo, abierto, no controlado, realizado en la Unidad de Recuperación Cardiovascular de un hospital pediátrico de alta complejidad. Se administrólevosimendán, como uso compasivo, a todos los pacientes con bajo gasto posoperatorio refractario al tratamiento convencional. Se administró una dosisde carga de 6 μg/kg EV, seguido de 0,1 μg/kg/min por 24 h. La variable primaria predeterminada fue el impacto clínico del levosimendán sobre el gastocardíaco. Dos observadores independientes realizaron la evaluación clínica del gasto cardíaco. Se analizó la función cardíaca por ecocardiografía yvariables clínicas, bioquímicas y hemodinámicas antes de la infusión y después de ella. Resultados. Se administró levosimendán a 14 pacientesen 18 oportunidades La mediana de edadfue de 27,5 meses (r: 0,16-197) y el peso de 11,1 kg (r: 2,98-48). En 9/18 intervenciones (50%; p= 0,004) seobservó una mejoría en el gasto cardiaco. El puntaje de inotrópicos disminuyó (12,1 contra 6,1; p= 0,01),la SvO2 mejoró (69,5 ± 11,4% contra 76 ± 9,29%, p= 0,03) y la A-VDO2 disminuyó (26,78 ± 11,5% contra 20,81 ± 7,72%, p= 0,029). No se identificaron efectos adversos. Cuatro pacientes fallecieron, ninguno en relación con la administración del fármaco. Conclusiones. En el 50% de las intervenciones se observó mejoría en el gasto cardíaco. No se detectaron efectos adversos atribuibles al fármaco.(AU)

Introduction. Low cardiac output syndrome occurs frequently in pediatric patients after cardiac surgery. Catecholamines are used as inotropic drugs to treat this threatening condition, but may cause undesirable and potentially harmfull side effects. Thisstudy was performed to evaluate the efficacy and safety of levosimendan (LEVO) in pediatric patients with low cardiac output syndrome. Patients and methods. Open prospective, cuasi experimental, cohort. LEVO was given as compassionate treatment in patients with refractory possurgical low cardiac output syndrome. Every patient received an IV infusion of LEVO at 6 μg/kg during a fifteen minutes period, followed by a 24 h IV infusion at 0.1 μg/kg/min. Clinical improvement of cardiac output was the primary end point of the study. Two independent observers performedclinical evaluation, bidimensional echocardiogram, hemodinamic and laboratory tests were performed pre and after LEVO infusión. Results. LEVO was infused in 18 oportunities (fourteenchildren). The response was considered successful in 9/18 interventions (50%; p= 0.004). Both inotropic score (12.1 vs. 6,1, p= 0.01) and A-VDO22 (26.78 ± 11.5% vs. 20.81 ± 7.72%, p= 0.029) showed reduction, while SvO2 improved (69.5 ± 11.4% vs. 76 ± 9.29%, p= 0.03). No adverse effects were noticed.Four patients died, none of them related to LEVO administration. Conclusions. LEVO improved cardiac output in 50% of the interventions with pos-surgical LCOS and no adverse effect was observed.(AU)

A case of pediatric tumefactive demyelinating lesion misdiagnosed and treated as glioblastoma.

Because of their clinical and neuroradiological features, tumefactive demyelinating lesions, or giant plaques, are easily mistaken for tumors, with a consequent risk of gross errors in the choice of treatment. This article reports a 10-year-old girl who underwent surgery for a left parietal lesion misinterpreted as a glioblastoma which subsequently proved to be a case of giant plaque.