Avaliação da Circulação Coronariana após a Cirurgia de Jatene / Evaluation of Coronary Circulation after Arterial Switch Operation

Resumo Fundamento A avaliação da artéria coronária após a cirurgia de Jatene ainda é um desafio clínico. Objetivo Correlacionar alterações anatômicas identificadas por tomografia computadorizada cardíaca (TCC) com alterações fisiológicas detectadas na avaliação clínica para diagnosticar obstrução coronária no pós-operatório tardio de pacientes submetidos à cirurgia de Jatene. Métodos Este estudo incluiu 61 pacientes consecutivos com idade média de 9,4 anos que foram submetidos à cirurgia de Jatene. Os pacientes realizaram ecocardiografia, eletrocardiografia, teste cardiopulmonar do exercício, e tomografia computadorizada cardíaca para avaliação da capacidade funcional e anatomia da artéria coronária. Resultados A tomografia computadorizada cardíaca revelou que somente 3,3% dos pacientes apresentaram estenose da artéria coronária. Esses pacientes eram assintomáticos, e não foram detectados sinais de isquemia miocárdicas pelos exames realizados. Conclusão A incidência de anormalidades da artéria coronária é de 3,3% no seguimento tardio de nossa coorte de pacientes submetidos à cirurgia de Jatene. Não existe uma diretriz clara sobre o porquê, quando, e como esses pacientes deveriam ser rastreados, ou o que propor quando pacientes assintomáticos forem diagnosticados com obstrução coronária.

Abstract Background Coronary artery evaluation remains after arterial switch operation a clinical challenge. Objective This study aims to correlate anatomical changes diagnosed by cardiac computed tomography (CCT) with physiological alterations on clinical evaluation to diagnose coronary obstruction in late ASO patients. Methods This study included 61 consecutive patients with mean age of 9.4 years who underwent ASO. The patients were submitted to echocardiography, electrocardiography, cardiopulmonary exercise test, and cardiac computed tomography to evaluate functional capacity and coronary artery anatomy. Results Cardiac computed tomography revealed that only 3.3% of the patients had coronary stenosis. These patients were asymptomatic, and no signs of myocardial ischemia were detected by the tests. Conclusion The incidence of coronary abnormalities in late ASO patients was 3.3% in our cohort. There is no clear guideline as to why, when, and how these patients should be screened or what to propose when a coronary obstruction is diagnosed in asymptomatic patients.

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.

Late arterial switch surgery under ecmo support in a patient with transposition of the great arteries with intact ventricular septum: a case report

Abstract A 30-month-old male patient with transposition of the great arteries with intact ventricular septum (TGA/IVS) is presented. Arterial switch operation (ASO) was performed in the light of echocardiographic and angiographic findings. The patient remained under extracorporeal membrane oxygenation support for seven days postoperatively, and his cardiac functions returned to normal at the postoperative 10th day. He was discharged at the postoperative 20th day. The present case, which presents one of the most advanced ages at operation for TGA/IVS among previously reported cases, is used to discuss late ASO in this study.

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).

Malformações congênitas: um relato de reabilitação fisioterapêutica em paciente com transposição das grandes artérias / Congenital abnormalities: a physical therapy rehabilitation report in patient with transposition of great vessels

Objetivo: descrever uma abordagem fisioterapêutica na reabilitação neuropsicomotora e respiratória pediátrica em paciente com transposição das grandes artérias. Relato de caso: paciente R. S. C. R, sexo masculino, nascido de parto cesáreo, com diagnóstico clínico de malformações congênitas, portador de Transposição das Grandes Artérias ­ TGA, Comunicação Interatrial ­ CIA e Comunicação Interventricular Ampla ­ CIV. Avaliado aos 09 meses de idade com diagnóstico fisioterapêutico de atraso no desenvolvimento neuropsicomotor. Conclusão: esse relato de caso evidenciou efetiva abordagem fisioterapêutica na reabilitação neuropsicomotora e respiratória de uma criança com TGA + CIA + CIV em acompanhamento clínico, resultando em melhora neuropsicomotora identificada pelo melhor controle cervical e maior tempo na posição sentada, e melhora do desconforto respiratório passando de moderado para leve

Experiencia inicial en la cirugía Jatene, en pacientes con transposición clásica de grandes arterias, en el Hospital Nacional de Niños Benjamín Bloom, de junio de 2015 a junio de 2016, serie de casos / Initial experience in Jatene surgery, in patients with classic transposition of the great arteries, at the Benjamín Bloom National Children's Hospital, from June 2015 to June 2016, case series

La transposición clásica de las grandes arterias (TGA) o llamada también discordancia ventrículo arterial, es una anomalía cardiaca congénita en la que la aorta emerge del ventrículo derecho (VD) y la arteria pulmonar del ventrículo izquierdo (VI). Es el segundo defecto cardiaco congénito más común con una prevalencia a nivel mundial de 0.24/1000 nacidos vivos. Antes de la era quirúrgica, la Transposición de grandes arterias, (TGA), representaba la quinta parte de las muertes cardiacas en la niñez. En junio de 2015, fue realizada en El Salvador la primera cirugía Jatene; en el Hospital Nacional de Niños Benjamín Bloom (HNNBB), siendo este el principal centro de referencia nacional e institución pública de tercer nivel de atención que cuenta con un programa cardiovascular, tanto clínico como quirúrgico, desde hace 20 años y que brinda atención cardiaca a la niñez con defectos congénitos y adquiridos de El Salvador; por lo que a través del presente estudio damos a conocer la experiencia inicial obtenida

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.

Procedimento de Lecompte para a correção de transposição das grandes artérias, associada à comunicação interventricular e obstrução de via de saída do ventrículo esquerdo / Lecompte procedure for correction of the transposition of the great arteries associated with ventricular septal defect and left ventricle outflow tract obstruction

OBJETIVO: Avaliar o procedimento de Lecompte para a correção da transposição das grandes artérias associada à comunicação interventricular e obstrução da via de saída do ventrículo esquerdo (TGA, CIV e OVSVE) e apresentar os resultados no período pós-operatório intermediário e tardio. MÉTODO: Entre fevereiro de 1994 e julho de 2005, sete pacientes, com idade de 2 a 8 anos (mediana -M-: 3,0), portadores de TGA, CIV e OVSVE, foram submetidos a tratamento cirúrgico corretivo. Em seis casos, foi utilizado o procedimento de Lecompte. Esta técnica consiste na abordagem por ventriculotomia direita, ressecção ampla do septo conal e construção de um túnel ventricular conectando o ventrículo esquerdo à aorta; o caso restante apresentava obstrução da prótese valvulada implantada entre o ventrículo direito e a artéria pulmonar (VD-AP) e falência do VD e foi submetido à conversão no procedimento de Lecompte. RESULTADOS: Os tempos de CEC variaram entre 105 e 194 min (M: 130) e os tempos de anoxia entre 65 e 90 min (M: 78). Houve um óbito no pós-operatório imediato devido a coagulopatia, seguido de insuficiência ventricular direita. Os seis pacientes sobreviventes receberam alta hospitalar no período de 5 a 30 dias (M: 11) e permaneceram em acompanhamento entre 12 a 144 meses (M: 73,6). CONCLUSÃO: O procedimento de Lecompte teve como vantagens: 1 - Indicação cirúrgica em pacientes com menor faixa etária; 2 - Baixa morbi-mortalidade; 3 - Expectativa de acompanhamento a longo prazo, sem reoperação; 4 - Possibilidade de converter o procedimento de Rastelli em Lecompte.

OBJECTIVE: To evaluate the Lecompte procedure used for the correction of transposition of the great arteries (TGA) associated with ventricular septal defect (VSD) and left ventricle outflow tract obstruction (LVOTO) and to present the intermediate and long-term results of the surgery. METHODS: Between February 1994 and July 2005, seven patients with ages between 2 and 8 years (median: 3.0) suffering from TGA, VSD and LVOTO underwent corrective surgery. In six cases, the Lecompte procedure was performed. This technique consists in right ventriculotomy, extensive resection of the conal septum and construction of a ventricular tunnel connecting the left ventricle to the aorta (LV-Ao). The remaining case presented with obstruction of a valvular prosthesis implanted between the right ventricle and the pulmonary artery (RV-PA) and RV failure. This case was converted to the Lecompte procedure. RESULTS: The cardiopulmonary bypass time varied from 105 to 194 minutes (Median: 130) and the aortic clamping time varied from 65 to 90 minutes (Median: 78). There was one death in the immediate post-operative period due to coagulopathy followed by RV failure. Six patients were released from hospital between the 5th to 30th postoperative days (Median: 11) and the follow up period was from 12 to 144 months (Median: 73.6). CONCLUSIONS: The Lecompte procedure presents the following advantages: 1. Surgical indication for infants, 2. Low morbidity and mortality rates, 3. Free from reoperation over the long term. 4. Possibility of conversion of the Rastelli procedure into the Lecompte procedure.