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Although Down syndrome (DS) is considered a risk factor for hemodynamic instabilities (mainly pulmonary hypertension-PH) following surgery for congenital cardiac communications, many DS patients do surprising well postoperatively. We prospectively analyzed perioperative factors for a possible correlation with post-cardiopulmonary bypass (CPB) inflammatory reaction and postoperative PH in pediatric subjects. Sixty patients were enrolled (age 3 to 35 months), 39 of them with DS. Clinical and echocardiographic parameters (anatomical and hemodynamic) were computed preoperatively. Pulmonary and systemic mean arterial pressures (PAP and SAP) were assessed invasively intra and postoperatively. Immediate postoperative PAP/SAP ratio (PAP/SAPIPO) and the behavior of pressure curves were selected as primary outcome. Serum levels of 36 inflammatory proteins were measured by chemiluminescence preoperatively and 4 h post CPB. Of all factors analyzed, peripheral oxygen saturation (O2Sat, bedside assessment) was the only preoperative predictor of PAP/SAPIPO at multivariate analysis (p = 0.007). Respective values in non-DS, DS/O2Sat ≥ 95% and DS/O2Sat < 95% subgroups were 0.34 (0.017), 0.40 (0.027) and 0.45 (0.026), mean (SE), p = 0.004. The difference between non-DS and DS groups regarding postoperative PAP curves (upward shift in DS patients, p = 0.015) became nonsignificant (p = 0.114) after adjustment for preoperative O2Sat. Post-CPB levels of at least 5 cytokines were higher in patients with O2Sat < 95% versus those at or above this level, even within the DS group (p < 0.05). Thus, a baseline O2Sat < 95% representing pathophysiological phenomena in the airways and the distal lung, rather than DS in a broad sense, seems to be associated with post-CPB inflammation and postoperative PH in these patients.

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OBJECTIVE: The aim of this study was compare the palpation technique and ultrasound-guidance for femoral artery catheterization in pediatric patients undergoing surgery for congenital heart disease. MATERIALS AND METHODS: This prospective and randomized controlled study included American Society of Anesthesiologists III-IV 40 children who underwent congenital heart surgery. The patients were divided into two groups; ultrasound-guided catheterization group and palpation-guided catheterization group. Demographic and clinical characteristics of the patients, access time, success rate, number of attempts, first-attempt success, number of trials, and failed cannulations were recorded. RESULTS: The diameter of the femoral artery was significantly shorter, access time and numbers of trials were significantly lower, and first-attempt success rate was significantly higher in the US group. The complication rate was significantly higher in the P group. The number of failed catheterization was higher in the P group. Total cost required for the procedure was significantly lower in the US group. CONCLUSION: We found that ultrasound-guided arterial catheterization increases the success rate and the number of successful catheterizations, while reducing the overall procedure time, incidence of complications, and cost. Therefore, we believe that the use of ultrasound guidance in arterial catheterization in pediatric cardiac surgery would be a better choice.

OBJETIVO: El objetivo de este estudio fue comparar la técnica de palpación y ecoguiado para el cateterismo de la arteria femoral en pacientes pediátricos operados de cardiopatías congénitas. MATERIALES Y MÉTODOS: Este estudio prospectivo, aleatorizado y controlado incluyó a 40 niños ASA III-IV que se sometieron a cirugía cardíaca congénita. Los pacientes se dividieron en 2 grupos; Grupo de cateterismo guiado por ecografía y grupo de cateterismo guiado por palpación. RESULTADOS: El diámetro de la arteria femoral fue significativamente más corto, el tiempo de acceso y el número de intentos fueron significativamente menores y la tasa de éxito del primer intento fue significativamente mayor en grupo estadounidense. La tasa de complicaciones fue significativamente mayor en el grupo P. El número de cateterismos fallidos fue mayor en el grupo P. El costo total requerido para el procedimiento fue significativamente menor en el grupo de EE. CONCLUSIONES: Encontramos que el cateterismo arterial guiado por ultrasonido aumenta la tasa de éxito y el número de cateterismos exitosos, al tiempo que reduce el tiempo total del procedimiento, la incidencia de complicaciones y el costo. Por tanto, creemos que el uso de guía ecográfica en cateterismo arterial en cirugía cardiaca pediátrica sería una mejor opción.

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BACKGROUND: Pulmonary vascular abnormalities pose a risk for severe life-threatening hemodynamic disturbances following surgical repair of congenital cardiac communications (CCCs). In the distal lung, small airways and vessels share a common microenvironment, where biological crosstalks take place. Because respiratory cells infected by viruses express a number of molecules with potential impact on airway and vascular remodeling, we decided to test the hypothesis that CCC patients carrying viral genomes in the airways might be at a higher risk for pulmonary (and systemic) hemodynamic disturbances postoperatively. METHODS: Sixty patients were prospectively enrolled (age 11 [7-16] months, median with interquartile range). Preoperative pulmonary/systemic mean arterial pressure ratio (PAP/SAP) was 0.78 (0.63-0.88). The presence or absence of genetic material for respiratory viruses in nasopharyngeal and tracheal aspirates was investigated preoperatively in the absence of respiratory symptoms using real-time polymerase chain reaction (kit for detection of 19 pathogens). Post-cardiopulmonary bypass (CPB) inflammatory reaction was analyzed by measuring serum levels of 36 inflammatory proteins (immunoblotting) 4 h after its termination. Postoperative hemodynamics was assessed using continuous recording of PAP and SAP with calculation of PAP/SAP ratio. RESULTS: Viral genomes were detected in nasopharynx and the trachea in 64% and 38% of patients, respectively. Rhinovirus was the most prevalent agent. The presence of viral genomes in the trachea was associated with an upward shift of postoperative PAP curve (p = 0.011) with a PAP/SAP of 0.44 (0.36-0.50) in patients who were positive versus 0.34 (0.30-0.45) in those who were negative (p = 0.008). The presence or absence of viral genomes in nasopharynx did not help predict postoperative hemodynamics. Postoperative PAP/SAP was positively correlated with post-CPB levels of interleukin-1 receptor antagonist (p = 0.026), macrophage migration inhibitory factor (p = 0.019) and monocyte chemoattractant protein-1 (p = 0.031), particularly in patients with virus-positive tracheal aspirates. CONCLUSIONS: Patients with CCCs carrying respiratory viral genomes in lower airways are at a higher risk for postoperative pulmonary hypertension, thus deserving special attention and care. Preoperative exposure to respiratory viruses and post-CPB inflammatory reaction seem to play a combined role in determining the postoperative behavior of the pulmonary circulation.

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Resumen: Objetivo: determinar las características y desenlaces clínicos de pacientes menores de 18 años tratados con oxigenación con membrana extracorpórea (ECMO) posterior a cirugía para la reparación o paliación de cardiopatías congénitas en un centro de referencia del suroccidente colombiano entre 2015 y 2020. Método: estudio descriptivo longitudinal con recolección retrospectiva de la información. Resultados: entre enero de 2015 y diciembre de 2020, 77 pacientes requirieron ECMO posterior a cirugía cardiaca pediátrica, con una mediana de edad de < 1 mes, mediana de peso de 4 kg, tiempo de circulación extracorpórea de 202 minutos y tiempo de pinzamiento aórtico de 95 minutos. La cardiopatía congénita más ingresada a ECMO fue el síndrome de corazón izquierdo hipoplásico (19.4%) en su posquirúrgico de Norwood (18.1%). La mediana del tiempo de ECMO fue de 111 horas. La indicación más frecuente fue la disfunción ventricular (80.5%). El 80.5% presentó complicaciones y la reintervención por sangrado fue la más frecuente (46.7%). La sobrevida a la ECMO fue del 48% y la sobrevida general al momento del alta fue de 25.97%. La edad < 1 mes (p = 0.030), el ácido láctico preECMO ≥ 5 mmol/l (p = 0.014) y el tiempo de normalización del lactato ≥ 24 horas (0.021), se asociaron a un mayor riesgo de mortalidad al egreso hospitalario. Conclusiones: la ECMO proporciona un soporte adecuado posterior a la cirugía cardiaca pediátrica. La edad < 1 mes, el ácido láctico preECMO ≥ 5 mmol/L y el tiempo de normalización del lactato ≥ 24 horas fueron las variables que se asociaron con un mal resultado y mortalidad hospitalaria.

Abstract: Objective: to determine the characteristics and clinical outcomes of patients under 18 years of age treated with extracorporeal membrane oxygenation (ECMO) after surgery for the repair or palliation of congenital heart disease. Method: longitudinal descriptive study with retrospective data collection. Results: between January 2015 and December 2020, 77 patients required ECMO after pediatric cardiac surgery with a median age of < 1 month, median weight of 4 kg, ECC time 202 minutes (IQR 125-272 minutes), aortic cross-clamp time 95 minutes. 76.6% were supported with ECMO prior to their return to the ICU. The congenital heart disease most frequently admitted to ECMO was hypoplastic left heart syndrome (19.4%) in the Norwood postoperative period (18.1%). The median time of ECMO was 111 hours. The most frequent indication was ventricular dysfunction (80.5%). 80.5% presented complications and reoperation for bleeding was the most frequent of these (46.7%). ECMO survival was 48% and overall survival at discharge was 25.97%. Age < 1 month (p = 0.030), pre-ECMO lactic acid ≥ 5 mmol/l (p = 0.014), OR 0.260 and time of normalization of lactate ≥ 24 hours (0.021), OR 0.24 were associated with a higher risk of mortality at hospital discharge. Conclusions: ECMO provides adequate support after surgery for the repair or palliation of congenital heart disease. Age < 1-month, pre-ECMO lactic acid ≥ 5 mmol/l, and lactate normalization time ≥ 24 hours were the variables that were associated with poor outcome and hospital mortality.

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BACKGROUND: The indications for pediatric coronary revascularization are diverse. There are a large proportion of patients with sequelae of severe inflammatory diseases such as Kawasaki disease, and other less common causes. METHODS: Retrospective review of ten pediatric patients undergoing coronary artery bypass surgery from January 2004 to December 2016. RESULTS: Ten children and adolescents ranging in age from 2 to 17 (median, 6) years at operation were followed up for as long as 13 years with a median follow-up of 2 years. The surgical indications include ischemia symptoms and/or coronary stenosis angiographically documented. Diagnoses include Kawasaki disease, anomalous origin of the left coronary artery from the pulmonary artery, and iatrogenic lesion of the right coronary artery. All the surgical procedures were performed with cardiopulmonary bypass with crystalloid cardioplegic arrest. The number of distal anastomoses was 1.6 per patient, and the left internal thoracic artery was used in one patient, the right internal thoracic artery in four patients, bilateral internal thoracic artery in four patients, and bilateral internal thoracic artery plus left radial artery in one patient, most frequently for right coronary artery revascularization. The patients underwent noninvasive diagnostic study during follow-up to evaluate their coronary status. The ten patients had no symptoms, and there was no mortality. CONCLUSIONS: Although survival was excellent after pediatric coronary bypass in our center, we need to continue the follow-up. Coronary revascularization by means of arterial grafting is a safe and reliable surgical modality for coronary disease in children.

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The development of the membrane oxygenator for pediatric cardiopulmonary bypass has been an incorporation of ideology and technological advancements with contributions by many investigators throughout the past two centuries. With the pursuit of this technological achievement, the ability to care for mankind in the areas of cardiac surgery has been made possible. Heart disease can affect anyone within the general population, but one such segment that it can affect from inception includes children. Currently, congenital heart defects are the most common birth defects nationally and worldwide. A large meta-analysis study from 1930 to 2010 was conducted in review of published medical literature totaling 114 papers with a study population of 24,091,867 live births, and divulged a staggering incidence of congenital heart disease involving 164,396 subjects with diverse cardiac illnesses. The prevalence of these diseases increased from 0.6 per 1,000 live births from 1930-1934 to 9.1 per 1,000 live births after 1995. These data reveal an emphasis on a growing public health issue regarding congenital heart disease. This discovery displays a need for heightened awareness in the scientific and medical industrial community to accelerate investigative research on emerging cardiovascular devices in an effort to confront congenital anomalies. One such device that has evolved over the past several decades is the pediatric membrane oxygenator. The pediatric membrane oxygenator, in conjunction with the heart lung machine, assists in the repair of most congenital cardiac defects. Numerous children born with congenital heart disease with or without congestive heart failure have experienced improved clinical outcomes in quality of life, survival, and mortality as a result of the inclusion of this technology during their cardiac surgical procedure. The purpose of this review is to report a summary of the published medical and scientific literature related to development of the pediatric membrane oxygenator from its conceptual evolutionary stages to artificially supporting whole body perfusion in the modern pediatric cardiac surgical setting.

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South America is a territory of 17,819,100 km(2), where ∼388 million people live in 13 countries. In the region, access to medical assistance (e.g., for treatment of cardiovascular disorders) is relatively easy in metropolitan areas but difficult in remote places such as the Andes and the Amazon. Altitudes up to ∼6,700 m influence the prevalence of congenital heart disease (CHD) and pulmonary arterial hypertension (PAH). In tertiary centers, CHD is now treated earlier in life but remains an important etiology of PAH. In adolescents and adults with PAH assisted at institutions devoted to treatment of cardiovascular disorders, the relative frequency of PAH-CHD (∼50%-60%) is even higher than that of idiopathic PAH. In one big tertiary center in São Paulo, Brazil, the prevalence of advanced PAH in children and adults with CHD is 1.2% and 4.2%, respectively. In young patients with cardiac septal defects (aged up to 2 years), pulmonary vascular abnormalities are a matter of concern in the decision about operability in 4.9% of cases. Access to specific PAH drugs is not uniform in South America, being unrealistic in remote places. In big cities, there are real possibilities for management of complex CHD, neonatal disorders, and even cardiac transplantation. Research activities have been implemented at clinical, translational, and basic levels. However, because of social and economic inequalities and political issues, access to best standards of medical care remains a problem in the region as a whole.

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OBJECTIVE: To analyze the preoperative plasma antigenic concentration and activity of von Willebrand factor and its main cleaving protease ADAMTS-13 in pediatric patients with cyanotic congenital heart disease undergoing surgical treatment and investigate possible correlations with postoperative bleeding. METHODS: Plasma antigenic concentrations (von Willebrand factor:Ag and ADAMTS-13:Ag) were measured using enzyme-linked immunoassays. Collagen-binding assays were developed to measure biological activities (von Willebrand factor:collagen binding and ADAMTS-13 activity). The multimeric structure of von Willebrand factor was analyzed using Western immunoblotting. Demographic, diagnostic, and general and specific laboratory data and surgery-related variables were subjected to univariate, bivariate, and multivariate analysis for the prediction of postoperative bleeding. RESULTS: Forty-eight patients were enrolled, with ages ranging from 9 months to 7.6 years (median 2.5 years). The plasma concentrations of von Willebrand factor:Ag and ADAMTS-13:Ag were decreased by 65 and 82%, respectively, in the patients compared with the controls (p<0.001). An increased density of low-molecular-weight fractions of von Willebrand factor, which are suggestive of proteolytic degradation (p = 0.0081), was associated with decreased ADAMTS-13 activity, which was likely due to ADAMTS-13 consumption (71% of controls, p = 0.0029) and decreased von Willebrand factor:collagen binding (76% of controls, p = 0.0004). Significant postoperative bleeding occurred in 13 patients. The preoperative ADAMTS-13 activity of <64.6% (mean level for the group), preoperative activated partial thromboplastin time, and the need for cardiopulmonary bypass were characterized as independent risk factors for postoperative bleeding, with respective hazard ratios of 22.35 (95% CI 1.69 to 294.79), 1.096 (95% CI 1.016 to 1.183), and 37.43 (95% ...

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The modified Fontan procedure represents the final stage of reconstructive surgery for most patients with functionally univentricular hearts. Although outcomes following Fontan procedures performed at sea level are widely reported, less has been documented and reported concerning outcomes in regions at high altitude. To clarify the main features involved, we present our institutional experience with Fontan operations performed in Mexico city (2,240 m above the sea level), with an emphasis on historical evolution of treatment. A retrospective and observational study was undertaken, which included 98 patients over a period of 18 years, and clinical outcomes in terms of morbidity and mortality were analyzed. A change in operative technique from intra-cardiac nonfenestrated Fontan procedure to extra-cardiac fenestrated technique occurred in 2001. Early mortality rates before and after this change in surgical approach were 26% and 4.7%, respectively. The most common morbidity was the occurrence of pleural effusions (98% of patients), which also appears to be a risk factor for operative mortality. Much remains unknown about the pathophysiology of the Fontan circulation at high altitude, and we need to develop morphological study protocols that include pulmonary biopsy to increase our knowledge and inform our therapeutic actions.

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Until the first quarter of the 20th century, most physicians were more than happy to differentiate congenital heart lesions from rheumatic heart disease, which then was rampant. As early as 1932, Dr Rodolfo Kreutzer, from Buenos Aires, Argentina, was already involved in the study of congenital heart defects. He started off assessing children with a stethoscope and with Einthoven electrocardiography equipment. The cardiac unit at the Buenos Aires Children's Hospital was created in 1936. It established the onset of pediatric cardiology in Argentina and fueled its development in South America. Nearly at the same time, Agustin Castellanos from Cuba also became a pioneer in the assessment of congenital heart disease. He described the clinical applications of intravenous angiocardiography in 1937. Meanwhile in Mexico, Dr Ignacio Chavez founded the National Institute of Cardiology in 1944 in Mexico City. It was the first center in the world to be exclusively devoted to cardiology. From this center, Victor Rubio and Hugo Limon performed the first therapeutic cardiac catheterization in 1953. Meanwhile, Professor Euriclydes Zerbini from Sao Paulo, Brazil, built the largest and most important school of cardiac surgeons in South America. In Santiago, Chile, the Calvo Makenna Hospital was the center where Helmut Jaegger operated on the first infant with extracorporeal circulation in Latin America in 1956. The patient was a 1-month-old baby, with complete transposition of the great arteries, who underwent an Albert procedure. Currently, there are many fully equipped centers all over the region, capable of dealing with most lesions and of providing excellent medical, interventional, and surgical treatment. Outcomes have improved substantially over the last 20 years. These achievements have gone beyond our pioneers' dreams. However, many neonates and young infants die prior to surgery because referral centers are overburdened and have long surgical waiting lists. Clearly, we still have to mastermind and establish sustainable public health policies to overcome these challenges.

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Very little information is available about the epidemiology of congenital heart disease in developing parts of the world, including South America. This article describes the incidence of congenital cardiac disease, the different treatment rates among countries, and future solutions for achieving improved coverage for the children with cardiac diseases in South America. An incidence of congenital cardiac disease of 8 per 1000 live births appears to be a fair approximation for the population of the world and also the population in South America. Nevertheless, a wide variation exists in the observed incidence of congenital cardiac disease in South American countries, which can be partly explained by inequalities in the access to diagnosis, differences in the diagnostic criteria, and true regional variations. It is estimated that 58,718 children are born yearly with congenital heart disease in South America. Brazil, Colombia, and Argentina have the highest number, followed by Peru, Venezuela, Chile, Ecuador, Bolivia, Paraguay, Uruguay, and Guyana. It is also estimated that in South America, 24,081 children per year with a new diagnosis of congenital cardiac disease do not receive any treatment. This paper provides strategies for improving the access to and quality of pediatric cardiac surgery in South America.

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PURPOSE--To analyze the results observed in children who underwent surgical correction of congenital anomalies, in whom the sternotomy was left open to avoid postoperative heart compression. METHODS--Fourteen children with mean age of 11 months, with different kind of congenital malformations who underwent definitive correction were submitted to the technique of delayed sternal closure. The indication was: hemodynamic instability due to sternal approximation (8), uncontrolled bleeding (4) and preventive (2). In all patients a bovine pericardial patch was sutured to the skin edges allowing a good decompression of the heart. RESULTS--Among the 14 children, two died in the immediate postoperative period, before the secondary sternal closure. There were two other deaths in the remaining 12 children who underwent sternal approximation, one due to acute pneumothorax and the other due to low cardiac output. The incision was closed after hemodynamic stabilization was achieved, and happened usually around the 3rd postoperative day. There were no cases of mediastinal infection. CONCLUSION--The technique of delayed sternal closure is an important approach in pediatric cardiac surgery and can be life saving. In our experience a bovine pericardial patch proved to be effective in decompressing the heart and also in protecting the mediastinal cavity

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PURPOSE--To determine the frequency and main features of subsequent cardiovascular surgery in patients operated on for coarctation of the thoracic aorta. METHODS--One hundred and five patients operated on for coarctation of the aorta with a mean follow-up period of 14 years had their notes analysed. The patients were divided in 4 groups according to age at correction of the coarctation. The incidence of recoarctation repair and other cardiovascular operations were noted. RESULTS--Surgical morbidity was important: 33 per cent . Recoarctation occurred in 14 per cent of the cases, mainly in those who had the coarctation resected during the first year of life. Operation for other cardiovascular defects was necessary in 24.5 per cent of the cases. Among these, correction of left-to-right shunts was done in 50 per cent of the patients who had the coarctation resected in the first year of life. Relief of aortic stenosis was the most frequent procedure (73 per cent ), tends to be more frequent the other is the patient at coarctation repair and more than one procedure may be necessary in some cases. CONCLUSION--Reoperation is frequent in the long term of patients operated on for coarctation of the aorta. The elective coarctation should be repaired after the first year of life hoping to avoid recoarctation. Routine follow-up is advisable for all patients aiming to detect residual left-to-right shunts and left ventricular outflow tract obstruction. Family counseling regarding prognosis after coarctation resection is recommended

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Objetivo - Estabelecer rotina para cirurgia cardíaca pediátrica em hospital geral, visando diminuiçäo dos riscos, maior certeza diagnóstica, uma abordagem mais precisa e conseqüente melhoria dos resultados. Métodos - Trezentos e noventa crianças portadoras de cardiopatias congênitas foram submetidas a correçäo cirúrgica em período de 5 anos. Entre os procedimentos habituais enfatizam-se o diagnóstico näo invasivo, a internaçäo conjunta com a mäe, a reduçäo do período de hospitalizaçäo, os cuidados com a coleta e preservaçäo do sangue, bem como a preferência pelo sangue fresco, as técnicas de monitorizaçäo, anestesia, circulaçäo extra-corpórea e proteçäo miocárdica, cuidados com a estética das incisöes em pacientes do sexo feminino e cuidados pós-operatórios. Resultados - Evidenciou-se um alto índice de extubaçäo precoce, com diminuiçäo significativa das complicaçöes pulmonares secundárias à ventilaçäo mecânica prolongada. As complicaçöes pós-operatórias como instabilidade hemodinâmica, arritmias bem como sangramento aumentado, näo foram, freqüentes. Os baixos índices de mortalidade e o curto período de internaçäo hospitalar confirmaram os bons resultados. Conclusäo - A elaboraçäo de rotina para cirurgia cardíaca peiátrica, com eliminaçäo ou minimizaçäo dos riscos inerentes a cada etapa, possibilitou uma melhoria progressiva dos resultados cirúrgicos

Purpose - To establish the routines for pediatric cardiac surgery in a general hospital, with a view to acurate diagnostic, a more precise intervention, a reduction of the risks and consequently an improvement of the results. Methods - Three hundred and ninety surgeries were carried out in children with congenital heart disease. The method use highlighted noninvasive diagnosis, joint lodging, reduced period of hospitalization, care with the collection and preservation of blood, as well as the preference for fresh blood, techniques of monitoring, anesthesia, cardiopulmonary bypass and myocardial protection, concern with aesthetic incision for female patients and postoperative critical care. Results - A high rate of early extubation was observed, with a signifcant reduction of the pulmonary complications resulting from prolonged mechanical ventilation. Low haemodynamic instability, arrhythmia and postoperative bleeding rates were uncommon. The low mortality rate and the average hospitalization period confirmed the good results. Conclusion - With the establishment of routines for pediatric cardiac surgery, we observed a progressive improvement of the results, with low rates of morbidity and mortality in a general hospital

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