Cannot extubate a newborn patient after an arterial switch operation? check major aortopulmonary collaterals!

Abstract The standard treatment of transposition of the great arteries is the arterial switch operation (ASO). Despite successful surgical correction, patients cannot tolerate extubation after the operation. Major aortopulmonary collaterals (MAPCAs) are one of the rare causes of prolonged mechanical ventilation due to significant hemodynamic effects. We report a 28-day-old newborn with transposition of the great arteries and a ventricular septal defect (VSD) who underwent ASO and VSD closure. After postoperative extubation failed twice, four large MAPCAs were revealed during heart catheterization. After transcatheter closure of these four MAPCAs, the patient was extubated and discharged 27 days after the procedure.

Modified closed coronary transfer is a good alternative to the trap-door method during arterial switch operation: a retrospective propensity-matched comparison

Abstract Objective: To compare the early and long-term results of patients in whom was performed modified closed coronary transfer with the results of patients in whom was performed trap-door transfer techniques by utilizing propensity-matching analysis to provide optimal identical patient matching for the groups. Methods: From August 2015 to December 2017, 127 consecutive patients underwent arterial switch operation due to simple and complex transposition of the great arteries, with or without additional arch and complex coronary pattern, by a single surgical team included into the study. Of these, in 70 patients it was performed modified closed coronary transfer technique and in 57 patients it was performed trap-door style coronary transfer technique. The patients were divided into two groups in terms of coronary transfer method. In the final model, after propensity matching, 47 patients from each group having similar propensity score were included into the study. Results: There was no significant difference between the groups regarding patient characteristics. Cross-clamp time and operation time were significantly lower in the modified technique group compared with the other group (P=0.03 and P=0.05, respectively). When compared the early and late postoperative outcomes, there was no significant difference between the groups. Postoperative echocardiographic findings were mostly similar between the groups. Conclusion: The patients in whom was performed our modified technique demonstrate overall good outcomes and the current technique ensures shorter arterial cross-clamp and operation times. It may be an alternative method to the trap-door technique for the coronary transfer during the arterial switch operation.

Mid- and Longterm Neo-Aortic Valve Regurgitation after Jatene Surgery: Prevalence and Risk Factors / Regurgitação da Valva Neo-Aórtica a Médio e Longo Prazo após Cirurgia de Jatene: Prevalência e Fatores de Risco

Abstract Background: Jatene surgery became the surgical procedure of choice to repair transposition of the great arteries (TGA) in neonates and infants. Late complications, mainly related to the pulmonary outflow tract and coronary arteries, are well known. The behavior of the neo-aortic valve is a cause of concern because of its potential for requiring late reoperation. Objectives: To assess the prevalence and risk factors of neo-aortic valve regurgitation in 127 patients in the late postoperative period of the Jatene surgery. Methods: Of the 328 survivors of the Jatene surgery at the Biocor Institute from October 1997 to June 2015, all patients undergoing postoperative follow-up were contacted via telephone, 127 being eligible for the study. The patients were divided into two groups, simple TGA and complex TGA groups, with follow-up means of 6.4 ± 4.7 years and 9.26 ± 4.22 years, respectively. Echocardiography was performed with adjusted measurements (Z-score) of the neo-aortic annulus, sinus of Valsalva, sinotubular region and ascending aorta, as well as quantification of the neo-aortic valve regurgitation grade. Results: The incidence of mild neo-aortic valve regurgitation was 29% in a follow-up of 7.4 ± 4.7 years. Moderate regurgitation was identified in 24 patients with age mean (± standard-deviation) of 9.81 ± 4.21 years, 19 of whom (79%) in the complex TGA group. Those patients had a higher aortic annulus Z-score. The reoperation rate due to neo-aortic regurgitation associated with aortic dilation was 1.5%, all patients in the complex TGA group. Conclusion: This study shows that, despite the low incidence of reoperation after Jatene surgery due to neo-aorta dilation and neo-aortic valve regurgitation, that is a time-dependent phenomenon, which requires strict vigilance of the patients. In this study, one of the major risk factors for neo-aortic valve regurgitation was the preoperative pulmonary artery diameter (p < 0.001).

Resumo Fundamento: A operação de Jatene tornou-se o procedimento cirúrgico de escolha para o reparo da transposição das grandes artérias (TGA) em neonatos e lactentes. Complicações tardias, principalmente relacionadas à via de saída pulmonar e às artérias coronarianas, já são bem reconhecidas. O comportamento da valva neo-aórtica tem sido motivo de crescente preocupação pelo seu potencial de necessidade de reoperações tardias. Objetivos: Avaliar a prevalência e os fatores de risco associados à regurgitação da valva neo-aórtica em 127 pacientes em pós-operatório tardio de cirurgia de Jatene. Métodos: Dos 328 sobreviventes da cirurgia de Jatene no Biocor Instituto operados de outubro de 1997 a junho de 2015, todos os pacientes em seguimento de pós-operatório foram contatados via ligação telefônica, sendo 127 elegíveis para o estudo. Os pacientes foram divididos em dois grupos, Grupo TGA simples e Grupo TGA complexa, com médias de follow-up de 6,4 ± 4,7 anos e 9,26 ± 4,22 anos, respectivamente. Foi realizada avaliação ecocardiográfica com medidas indexadas (escore Z) do anel da valva neo-aórtica, do seio de Valsalva, da região sinotubular e da aorta ascendente, bem como quantificação do grau de regurgitação da valva neo-aórtica. Resultados: A incidência de leve regurgitação da valva neo-aórtica em nossa casuística foi de 29% em um follow-up de 7,4 ± 4,7 anos. Regurgitação moderada foi observada em 24 pacientes, com idade média (± desvio-padrão) de 9,81 ± 4,21 anos, sendo 19 (79%) no Grupo TGA complexa. Nesses pacientes, observou-se maior escore Z do anel aórtico. A taxa de reoperação por regurgitação da valva neo-aórtica associada a dilatação da aorta foi de 1,5%, sendo todos os casos no Grupo TGA complexa. Conclusão: O estudo demonstra que, embora reoperações após cirurgia de Jatene por dilatação da neo-aorta e regurgitação da valva neo-aórtica tenham incidência baixa, esse é um fenômeno dependente de tempo, requerendo rígida vigilância desses pacientes. Na nossa casuística, um dos principais fatores de risco para regurgitação da valva neo-aórtica foi o diâmetro da artéria pulmonar no pré-operatório (p < 0,001).

The efficacy of thoracic ultrasonography in postoperative newborn patients after cardiac surgery

Abstract Objective: In this study, the efficacy of thoracic ultrasonography during echocardiography was evaluated in newborns. Methods: Sixty newborns who had undergone pediatric cardiac surgery were successively evaluated between March 1, 2015, and September 1, 2015. Patients were evaluated for effusion, pulmonary atelectasis, and pneumothorax by ultrasonography, and results were compared with X-ray findings. Results: Sixty percent (n=42) of the cases were male, the median age was 14 days (2-30 days), and the median body weight was 3.3 kg (2.8-4.5 kg). The median RACHS-1 score was 4 (2-6). Atelectasis was demonstrated in 66% (n=40) of the cases. Five of them were determined solely by X-ray, 10 of them only by ultrasonography, and 25 of them by both ultrasonography and X-ray. Pneumothorax was determined in 20% (n=12) of the cases. Excluding one case determined by both methods, all of the 11 cases were diagnosed by X-ray. Pleural effusion was diagnosed in 26% (n=16) of the cases. Four of the cases were demonstrated solely by ultrasonography, three of them solely by X-ray, and nine of the cases by both methods. Pericardial effusion was demonstrated in 10% (n=6) of the cases. Except for one of the cases determined by both methods, five of the cases were diagnosed by ultrasonography. There was a moderate correlation when all pathologies evaluated together (k=0.51). Conclusion: Thoracic ultrasonography might be a beneficial non-invasive method to evaluate postoperative respiratory problems in newborns who had congenital cardiac surgery.

Transposition of Great Arteries with Intramural Coronary Artery: Experience with a Modified Surgical Technique

Abstract Objective: Transposition of the great arteries is a common congenital heart disease. Arterial switch is the gold standard operation for this complex heart disease. Arterial switch operation in the presence of intramural coronary artery is surgically the most demanding even for the most experienced hands. We are presenting our experience with a modified technique for intramural coronary arteries in arterial switch operation. Methods: This prospective study involves 450 patients undergoing arterial switch operation at our institute from April 2006 to December 2013 (7.6 years). Eighteen patients underwent arterial switch operation with intramural coronary artery. The coronary patterns and technique used are detailed in the text. Results: The overall mortality found in the subgroup of 18 patients having intramural coronary artery was 16% (n=3). Our first patient had an accidental injury to the left coronary artery and died in the operating room. A seven-day old newborn died from intractable ventricular arrhythmia fifteen hours after surgery. Another patient who had multiple ventricular septal defects with type B arch interruption died from residual apical ventricular septal defect and sepsis on the eleventh postoperative day. The remainder of the patients are doing well, showing a median follow-up duration of 1235.34±815.26 days (range 369 - 2730). Conclusion: Transposition of the great arteries with intramural coronary artery is demanding in a subset of patients undergoing arterial switch operation. We believe our technique of coronary button dissection in the presence of intramural coronary arteries using coronary shunt is simple and can be a good addition to the surgeons' armamentarium.

Palliative Senning in the Treatment of Congenital Heart Disease with Severe Pulmonary Hypertension / Senning Paliativo no Tratamento de Cardiopatias Congênitas com Hipertensāo Pulmonar Grave

Background:Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level.Objective:Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure.Methods:Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included.Results:After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced.Conclusion:The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis.

Fundamento:A transposição das grandes artérias (TGA) é a cardiopatia cianogênica mais comum, com incidência variando de 0,2 a 0,4 por 1000 nascidos vivos. Muitos dos pacientes não tratados nos primeiros meses de vida podem evoluir com doença vascular pulmonar grave, e um método terapêutico para o tratamento desses pacientes pode ser realizado por meio de uma cirurgia paliativa para redirecionamento do fluxo em nível atrial.Objetivo:Relatar a experiência institucional com a cirurgia de Senning paliativo em crianças com diagnóstico de TGA e dupla via de saída do ventrículo direito com doença vascular pulmonar severa. Avaliar, também, a evolução clínica precoce e tardia da operação de Senning paliativo.Métodos:Estudo retrospectivo, baseado na avaliação de prontuários no período de 1991 a 2014. Somente pacientes sem indicação para o tratamento cirúrgico definitivo da cardiopatia em razão de elevadas pressões pulmonares.Resultados:Evidenciou-se após um ano de evolução um aumento médio da saturação arterial de oxigênio de 62,1% para 92,5% e uma redução média do hematócrito de 49,4% para 36,3%. A análise da histologia pulmonar foi possível em 16 pacientes. Em oito pacientes foi evidenciada lesão pulmonar grau 3 e 4.Conclusão:A cirurgia de Senning paliativo melhorou a saturação de oxigênio arterial, reduziu a policitemia e proporcionou uma melhora da qualidade de vida em pacientes com TGA com comunicação interventricular, os quais possuíam hipertensão pulmonar severa com prognóstico fechado.

Post-cardiotomy ECMO in pediatric and congenital heart surgery: impact of team training and equipment in the results / ECMO pós-cardiotomia em cirurgia cardíaca pediátrica e congênita: impacto do treinamento da equipe e equipamentos nos resultados

AbstractIntroduction:Post-cardiotomy myocardial dysfunction requiring mechanical circulatory support occurs in about 0.5% of cases. In our environment, the use of extracorporeal membrane oxygenation has been increasing in recent years.Objective:To evaluate the impact of investment in professional training and improvement of equipment in the rate of weaning from extracorporeal membrane oxygenation and survival.Methods:A retrospective study. Fifty-six pediatric and/or congenital heart patients underwent post-cardiotomy extracorporeal membrane oxygenation at our institution between November 1999 and July 2014. We divided this period into two phases: phase I, 36 cases (before the structuring of the extracorporeal membrane oxygenation program) and phase II, 20 cases (after the extracorporeal membrane oxygenation program implementation) with investment in training and equipment). Were considered as primary outcomes: extracorporeal membrane oxygenation weaning and survival to hospital discharge. The results in both phases were compared using Chi-square test. To identify the impact of the different variables we used binary logistic regression analysis.Results:Groups were comparable. In phase I, 9 patients (25%) were weaned from extracorporeal membrane oxygenation, but only 2 (5.5%) were discharged. In phase II, extracorporeal membrane oxygenation was used in 20 patients, weaning was possible in 17 (85%), with 9 (45%) hospital discharges (P<0.01). When the impact of several variables on discharge and weaning of extracorporeal membrane oxygenation was analyzed, we observe that phase II was an independent predictor of better results (P<0.001) and need for left cavities drainage was associated with worse survival (P=0.045).Conclusion:The investment in professional training and improvement of equipment significantly increased extracorporeal membrane oxygenation results.

ResumoIntrodução:Falência ventricular pós-cardiotomia necessitando de suporte circulatório mecânico ocorre em cerca de 0,5% dos casos. Em nosso meio, o uso de ECMO tem aumentado nos últimos anos.Objetivo:Avaliar o impacto do investimento na formação profissional e melhoria dos equipamentos na taxa de desmame de ECMO e na sobrevida.Métodos:Estudo retrospectivo. Cinquenta e seis pacientes cardíacos pediátricos e/ou portadores de cardiopatias congênitas foram submetidos ao implante de ECMO pós-cardiotomia em nossa instituição entre novembro de 1999 e julho de 2014. Nós dividimos este período em duas fases: fase I, 36 casos (antes da estruturação do programa de ECMO) e fase II, 20 casos (após a instalação do programa ECMO com investimento em formação e equipamento). Foram considerados como desfechos primários: o desmame de ECMO e sobrevida até a alta hospitalar. Os resultados em ambas as fases foram comparados pelo teste Chi-quadrado. Para identificar o impacto das diferentes variáveis, ​ foi usada análise de regressão logística binária.Resultados:Na fase I, 9 pacientes (25%) foram desmamados da ECMO, mas apenas 2 (5,5%) tiveram alta. Na fase II, ECMO foi usado em 20 pacientes, o desmame foi possível em 17 deles (85%), com 9 (45%) altas hospitalares. Quando analisamos o impacto das diversas variáveis ​​sobre a sobrevida e desmame de ECMO, observa-se que a fase II foi um preditor independente de melhores resultados (P<0,001) e a necessidade de drenagem das cavidades esquerdas foi associada com pior sobrevida (P=0,045).Conclusão:O investimento na formação profissional e aperfeiçoamento de equipamentos melhorou significativamente os resultados de ECMO em nossa instituição.

Bandagem ajustável do tronco pulmonar: IX: atividade da G6PD do miocárdio de cabras adultas submetido ao treinamento ventricular / Reversible pulmonary trunk banding: IX. G6PD activity of adult goat myocardium submitted to ventricular retraining

OBJETIVO: O aumento da atividade miocárdica da Glicose 6-Fosfato Desidrogenase tem sido demonstrado na insuficiência cardíaca. Este estudo avalia a atividade miocárdica da Glicose 6-Fosfato Desidrogenase no treinamento do ventrículo subpulmonar de cabras adultas. MÉTODOS: Foram utilizadas 18 cabras adultas, divididas em três grupos: convencional (bandagem fixa), sham e intermitente (bandagem ajustável; 12 horas diárias de sobrecarga). A sobrecarga sistólica (70% da pressão sistêmica) foi mantida durante quatro semanas. As avaliações hemodinâmica e ecocardiográfica foram realizadas durante todo o estudo. Depois de cumprido o protocolo, os animais foram mortos para avaliação morfológica e da atividade da Glicose 6-Fosfato Desidrogenase dos ventrículos. RESULTADOS: Apesar de haver sobrecarga sistólica proporcionalmente menor no ventrículo subpulmonar do grupo intermitente (P=0,001), ambos os grupos de estudo apresentaram aumento da massa muscular de magnitude similar. Os grupos intermitente e convencional apresentaram aumento da massa de 55,7% e 36,7% (P<0,05), respectivamente, em comparação ao grupo sham. O conteúdo de água do miocárdio não variou entre os grupos estudados (P=0,27). O ecocardiograma demonstrou maior aumento (37,2%) na espessura do ventrículo subpulmonar do grupo intermitente, em relação aos grupos sham e convencional (P<0,05). Foi observada maior atividade da Glicose 6-Fosfato Desidrogenase na hipertrofia miocárdica do ventrículo subpulmonar do grupo convencional, comparada aos grupos sham e intermitente (P=0,05). CONCLUSÃO: Ambos os grupos de treinamento ventricular desenvolveram hipertrofia ventricular, a despeito do menor tempo de sobrecarga sistólica no grupo intermitente. A maior atividade de Glicose 6-Fosfato Desidrogenase observada no grupo convencional pode refletir um desequilíbrio redox, com maior produção de fosfato de dinucleotídeo de nicotinamida e adenina e glutationa reduzida, um mecanismo importante da fisiopatologia da insuficiência cardíaca.

OBJECTIVE: Increased glucose 6-phosphate dehydrogenase activity has been demonstrated in heart failure. This study sought to assess myocardial glucose 6-phosphate dehydrogenase activity in retraining of the subpulmonary ventricle of adult goats. METHODS: Eighteen adult goats were divided into three groups: traditional (fixed banding), sham, and intermittent (adjustable banding, daily 12-hour systolic overload). Systolic overload (70% of systemic pressure) was maintained during a 4-week period. Right ventricle, pulmonary artery and aortic pressures were measured throughout the study. All animals were submitted to echocardiographic and hemodynamic evaluations throughout the protocol. After the study period, the animals were killed for morphological and glucose 6-phosphate dehydrogenase activity assessment. RESULTS: A 55.7% and 36.7% increase occurred in the intermittent and traditional right ventricle masses, respectively, when compared with the sham group (P<0.05), despite less exposure of intermittent group to systolic overload. No significant changes were observed in myocardial water content in the 3 groups (P=0.27). A 37.2% increase was found in right ventricle wall thickness of intermittent group, compared to sham and traditional groups (P<0.05). Right ventricle glucose 6-phosphate dehydrogenase activity was elevated in the traditional group, when compared to sham and intermittent groups (P=0.05). CONCLUSION: Both study groups have developed similar right ventricle hypertrophy, regardless less systolic overload exposure of intermittent group. Traditional systolic overload for adult subpulmonary ventricle retraining causes upregulation of myocardial glucose 6-phosphate dehydrogenase activity. It may suggest that the undesirable "pathologic systolic overload" is influenced by activation of penthose pathway and cytosolic Nicotinamide adenine dinucleotide phosphate availability. This altered energy substrate metabolism can elevate levels of free radicals by Nicotinamide adenine dinucleotide phosphate oxidase, an important mechanism in the pathophysiology of heart failure.

Taquicardia por reentrada nodal recurrente en paciente con transposición congénita corregida de grandes vasos. Utilización de mapeo no fluoroscópico / Use of non-fluoroscopic mapping in recurrent atrioventricular nodal reentrant tachycardia in a patient with corrected transposition of the great arteries

La complejidad anatómica y la variabilidad en el sistema de conducción en la transposición congénita corregida de los grandes vasos plantean intervenciones con el uso de recursos tecnológicos que faciliten un desenlace favorable. Describimos un caso de ablación de taquicardia por reentrada intranodal en donde el mapeo no fluoroscópico facilitó una intervención compleja.

The anatomy in congenital corrected transposition of the great arteries is complex and the conduction system may experience large degrees of variation. Invasive procedures should be done with the use of the highest possible technological sources to warrant success. We describe here, a patient with recurrent atrioventricular node reentry tachycardia where non-fluoroscopic navigation system helped in a complex ablation.

Tratamiento quirúrgico de la transposición de grandes arterias y factores asociados con la mortalidad / Surgical treatment of great arteries transposition and risk factors for mortality

La transposición de grandes arterias se define como una discordancia en la conexión ventrículo-arterial; su incidencia varía entre 0,02 y 0,05% de todos los nacidos vivos, correspondiendo a 5 - 8% de todas las cardiopatías congénitas. El tratamiento de elección es la cirugía de Jatene. Objetivos: Describir las características de los pacientes con transposición de grandes arterias sometidos a tratamiento quirúrgico y explorar factores asociados con mortalidad. Material y métodos: Estudio descriptivo, retrospectivo de pacientes con diagnóstico de transposición de grandes arterias tratados quirúrgicamente con la técnica de Jatene entre enero del 2001 y diciembre del 2010. Se creó una base datos en SPSS v19.0 y se analizaron las posibles variables asociadas a la mortalidad temprana. Resultados: De los 52 pacientes, 77% fueron varones, presentando un peso aproximado de 3 500 g. La edad promedio fue 54,83 días. La mayoría de los casos fueron de transposición con septum interventricular intacto con una distribución normal de las coronarias. La mortalidad hospitalaria global fue 31% y en los tres últimos años se redujo a 16%. La variable edad mayor de 23 días tuvo 6 veces más riesgo de muerte intrahospitalaria. Conclusiones: Los hallazgos observados coinciden con lo descrito internacionalmente. La edad superior a 23 días se asocia a mayor mortalidad la cual se ha logrado reducir hasta 16% en los últimos tres años del estudio. Aún queda implementar un protocolo institucional con todo el arsenal farmacológico y tecnológico que logre reducir la mortalidad a valores internacionales.

Great arteries transposition is a discordant atrioventricular connection whose incidence varies between 0.02% to 0.05% of all newborns and represents 5-8% of all congenital cardiopathies. The tratment of choice for this conditionis the Jatene ìs surgical procedure.Objectives: To describe clinical features of patients with great arteries transposition in whom Jatene ìs procedure was performed and to explore risk factors for mortality among them. Methods: A retrospective and descriptive study was conducted between January 2001 and December 2010. A database in SPSS v19.0 was created. Results: 52 patients were analyzed; 77% were male; mean body weight was 3500 g; mean age was 54.83 days. Most of the cases presented with artery transposition with intact interventricular septum and withnormal coronary arterial distribution. Overall in-hospital mortality was 31%, which was reduced to 16% in the last3-year period. Age above 23 days was associated with six times more mortality. Conclusions: Our findings are in agreement with previous international reports. Age above 23 days was a risk factor for mortality, which was reduc.

Resultados inmediatos y alejados del switch arterial en pacientes condextrotransposición de grandes arterias: experiencia de 20 años / Early and late results of the switch operation for d-transposition of the great vessels. clinical experience in 108 patients operated on between 1992 and 2012

Objetivos: Comunicar los resultados de la operación de switch arterial en pacientes portadores de dextro Transposición de Grandes Arterias (D-TGA) y evaluar su evolución en el tiempo. Pacientes y Método: Estudio retrospectivo de pacientes sometidos a switch arterial entre mayo de 1992 y noviembre de 2012. Se comparó período 1 (1992 a 2002) con período 2 (2003 a 2012). Se definió D-TGA simple aquella sin lesiones asociadas y D-TGA compleja aquella con asociación de comunicación interventricular o coartación aórtica. Resultados: Un total de 108 pacientes componen la serie, 44 en el período 1 y 64 en el período 2, sin diferencias demográficas y anatómicas entre ambos períodos. Setenta tenían D-TGA simple y 38 D-TGA compleja. La mortalidad operatoria en el período 1 fue 33% versus 8,4 por ciento en el período 2 (p< 0.025); hubo tendencia a mayor mortalidad en pacientes con anatomía coronaria compleja. Se observó una disminución del riesgo relativo de mortalidad de 68,7 por ciento en el período 2. El 28,7 por ciento presentaron complicaciones postoperatorias, sin diferencias entre ambos períodos. Un paciente falleció en forma alejada. La mediana de seguimiento fue de 60 meses y la supervivencia de 84,25 por ciento a 10 y 20 años. Se realizaron 16 re intervenciones, principalmente plastías percutáneas de ramas pulmonares. La mayoría de los pacientes se mantenían asintomáticos. La mortalidad operatoria de los últimos 5 años fue 2,6 por ciento. Conclusiones: La mortalidad operatoria ha disminuido significativamente a lo largo de 20 años; persiste un mayor riesgo en ciertos patrones de anatomía coronaria. La supervivencia alejada y libre de reintervenciones es muy favorable.

Aim: to report the results of the arterial Switch operation in patients with D-transposition of the great vessels (D-TGA) and to evaluate their late course. Patients and Methods: A retrospective review of the clinical data on patients consecutively operated on for D-TGA using the switch procedure. Results obtained in patients operated on between 1992 and 2002 (Period 1) were compared to those obtained in patients undergoing their operation between 2013 and 2012. Patients with D-TGA and no complications were compared to those who had interventricular septal defect or aortic coarctation associated their TGA. Results: 44 patients belonged in Period 1 and 64 in Period 2 (total 108). Demographic and anatomical characteristics were similar in both periods. Simple D-TGA was present in 70 patients and complex D-TGA in 38. Operative mortality was higher in Period 1 compared to Period 2 (33 per cent vs. 8.4 percent, p<0.025). A higher, albeit not statiscally significant mortality was observed in patients with complex as opposed to simple D-TGA. A 68.7 per cent relative reduction mortality risk was observed in Period 2. Complications developed in 28.7 per cent of patients, with no difference between periods. Only 1 patient died during late follow up. Median follow up was 60 months and survival rate was 84.3 percent at 10 y 20 years. Sixteen patients required re-intervention, mainly to perform percutaneous plastic procedures on pulmonary artery branches. Most patients had an asymptomatic course during follow up. Surgical mortality for the last 5 years was 2.6 percent. Conclusion: surgical mortality for the arterial switch operation in patients with D-TGA has significantly decreased along a 20 year period. An increased surgical risk persists for patients with certain forms of coronary artery anomalies. Late survival free of re-intervention was the rule in these patients.

Entrecruzamento das vias de entrada ventriculres (Criss-Cross Heart) e transposição anatomicamente corrigida das grandes artérias: relato de caso e revisao de literatura / Criss-Cross heart and corrected transposition of great arteries: case report and literature review

Criss-cross heart é uma rara e complexa anomalia congênita que se caracteriza pela rotação da conexão atrioventricular e entrecruzamento dos fluxos de entrada dos ventrículos. É resultado de uma anomalia de rotação embrionária da massa ventricular no seu eixo longo de forma horária ou anti-horária, sem movimentação concomitante dos átrios. O quadro clínico depende das malformações associadas, dimensões da comunicação interventricular, presença ou não de estenose pulmonar, straddling da valva mitral e dimensões das cavidades ventriculares. A presente revisão abordará os critérios ecocardiográficos de criss-cross heart, definindo as questões técnicas do exame, além de relatar um caso de criss-cross com transposição corrigida das grandes artérias.

Avaliação experimental do metabolismo energético em dois protocolos de sobrecarga sistólica do ventrículo direito / Experimental evaluation of energy metabolism in two right ventricle systolic overload protocols

Objetivo: Alterações do metabolismo energético tem sido identificadas em processos de hipertrofia miocárdica, sendo algumas consideradas benéficas, porém outras estão associadas a insuficiência cardíaca. O objetivo deste trabalho foi comparar a atividade de três enzimas do metabolismo energético em dois protocolos de sobrecarga sistólica do ventrículo direito (VD) num modelo experimental em cabritos. Métodos: 27 cabritos jovens foram divididos em três grupos: Sham (sem sobrecarga), Contínuo (sobrecarga sistólica constante) e Intermitente (4 períodos de 12 horas de sobrecarga sistólica, intercalados com 12 horas de descanso). Durante as 96 horas do protocolo, a sobrecarga sistólica foi ajustada a fim de atingir relação de pressão entre a aorta e o VD de 0,7. Medidas ecocardiográficas e hemodinâmicas foram realizadas antes e após o período de sobrecarga diariamente até o término do protoloco. Após o término do mesmo, os animais foram sacrificados a fim de obter dados morfológicos e a atividade máxima das enzimas Glicose 6 Fosfato Desidrogenase (G6PD), Hexoquinase (HK) e Lactato Desidrogenase (LDH). Resultados: Houve aumento de 92,1% e 46,5% nas massas do VD e septal no grupo Intermitente, respectivamente, quando comparado ao grupo Sham, enquanto que no grupo Contínuo houve incremento de 37,2% somente na massa septal. O VD e Septo dos grupos submetidos à sobrecarga sistólica contínua e intermitente do VD apresentaram um aumento discreto, porém significativo, do conteúdo de água (VD, p=0,0014; Septo, p=0,0004) em relação ao grupo Sham. Ao final do protocolo, foi observado um aumento significativo de 103,8% da espessura do VD no grupo Intermitente, comparado a um aumento de 38,4% do grupo Contínuo. Houve também dilatação ventricular significativa no grupo Contínuo ao longo do protocolo, quando comparado aos outros grupos (p<0,001). Piores índices de desempenho miocárdico ocorreram no grupo Contínuo no momentos 72 e 96 horas, quando comparados ao grupos Sham...

Objective: Altered energy metabolism has been identified in myocardial hypertrophy. Some processes are considered beneficial, whereas others are linked to heart failure. The purpose of this study was to compare the activity of three different energy metabolism enzymes in two different protocols of right ventricle (RV) systolic overload in young goats. Methods: 27 young goats were separated into three groups: Sham (no overload), Continuous (continuous systolic overload) and Intermittent (4 periods of 12-hour systolic overload, alternated with a 12-hour resting period). During a 96-hour protocol, systolic overload was adjusted to achieve a 0.7 RV / aortic pressure ratio. Echocardiographic and hemodynamic evaluations were performed before and after systolic overload every day postoperatively. After the study period, the animals were humanely killed for morphological and Glucose-6-phosphate dehydrogenase (G6PD), hexoquinase (HK) and lactate dehydrogenase (LDH) activity assessment. Results: There was a 92.1% and 46.5% increase in RV and septal masses of Intermittent group, respectively, as compared to Sham group, while Continuous systolic overload resulted in 37.2% increase of only septal mass. There was a small, but significant increase in water content in RV and septum of Intermittent and Continuous groups, as compared to Sham group (RV, p=0.0014; Septum, p=0.0004). At the end of protocol, it was observed a greater increase in RV thickness (103.8%) in Intermittent group, as compared to Continous group (38.4%). There was also a significant right ventricle dilatation in Continuos group along the protocol, as compared to the other groups (p<0.001). A worsening RV myocardial performance index occurred in the continuous group at 72 hours and 96 hours, compared with the sham (P<0.039) and intermittent groups (P<0.001). Compared to Sham, RV to LV G6PD activity ratio was elevated by 130.1% in Continuous group (p= 0,012) and by 39.8% in Intermittent group (p=0.764)...

Extra corporeal membrane oxygenation after pediatric cardiac surgery: A 10 year experience.

Indications for extra corporeal membrane oxygenation (ECMO) after pediatric cardiac surgery have been increasing despite the absence of encouraging survival statistics. Modification of ECMO circuit led to the development of integrated ECMO cardiopulmonary bypass (CPB) circuit at the author's institute, for children undergoing repair of transposition of great arteries among other congenital heart diseases (CHD). In this report, they analyzed the outcome of children with CHD, undergoing surgical repair and administered ECMO support in the last 10 years. The outcome was analyzed with reference to the timing of intervention, use of integrated ECMO-CPB circuit, indication for ECMO support, duration of ECMO run and the underlying CHD. The results reveal a significantly improved survival rate with the use of integrated ECMO-CPB circuit and early time of intervention rather than using ECMO as a last resort in the management. The patients with reactive pulmonary artery hypertension respond favorably to ECMO support. In all scenarios, early intervention is the key to survival.