Modified Senning Procedure for Treatment of Transposition of the Great Arteries with Crisscross Heart.

CLINICAL DATA: A nine-month-old female infant diagnosed with transposition of the great arteries with symptoms of heart failure associated with cyanosis and difficulty in gaining weight was referred to our center with late diagnosis (at nine months of age). CHEST RADIOGRAPHY: Cardiomegaly; attenuated peripheral vascular markings.Electrocardiography: Sinus rhythm with biventricular overload and aberrantly conducted supraventricular extra systoles. ECHOCARDIOGRAPHY: Wide atrial septal defect, ventricular axis torsion with concordant atrioventricular connection and discordant ventriculoarterial connection. COMPUTED TOMOGRAPHY ANGIOGRAPHY: Concordant atrioventricular connection, right ventricle positioned superiorly and left ventricle positioned inferiorly; discordant ventriculoarterial connection with right ventricle connected to the aorta and left ventricle connected to pulmonary artery. DIAGNOSIS: Crisscross heart is a rare congenital heart defect, accounting for 0.1% of congenital heart diseases. It consists of the 90º rotation of ventricles' axis in relation to their normal position; therefore, ventricles are positioned in the superior-inferior direction rather than anterior-posterior. Most cases have associated cardiac anomalies, and in this case, it is associated with transposition of the great arteries. The complexity and rarity of its occurrence make diagnosis and surgical treatment challenging. OPERATION: Modified Senning procedure using the pericardial sac in the construction of a tunnel from pulmonary veins to the right atrium. Cardiopulmonary bypass time of 147 minutes with nine minutes of total circulatory arrest.

Senning Procedure: Conceptualization in the Wet Lab.

Training congenital heart surgeons today is challenging for themselves and their mentors. The situation becomes even more complicated while teaching complex surgical procedures. Senning operation is one of the most ingenious intracardiac techniques. We consider this surgical technique a worthy example to stand out the potential advantage of wet lab training. This article demonstrates the simulation of the Senning procedure in an explanted porcine model.

Novel use of reverse double switch operation in failed left ventricular recruitment pathway.

In spite of great advances in staged left ventricle recruitment strategy, some patients do not achieve biventricular circulation nor are candidates for reversal to single-ventricle palliation. We present a case of a successful reverse double switch operation in a patient with failure of left ventricle recruitment and pulmonary hypertension. This strategy provided a one-and-a-half repair with a sub-pulmonary hypoplastic left ventricle that improved the patient's clinical status, becoming a novel alternative in this particular subset of patients.

Impact of aortic arch curvature in flow haemodynamics in patients with transposition of the great arteries after arterial switch operation.

AIMS: In this study, we will describe a comprehensive haemodynamic analysis and its relationship to the dilation of the aorta in transposition of the great artery (TGA) patients post-arterial switch operation (ASO) and controls using 4D-flow magnetic resonance imaging (MRI) data. METHODS AND RESULTS: Using 4D-flow MRI data of 14 TGA young patients and 8 age-matched normal controls obtained with 1.5 T GE-MR scanner, we evaluate 3D maps of 15 different haemodynamics parameters in six regions; three of them in the aortic root and three of them in the ascending aorta (anterior-left, -right, and posterior for both cases) to find its relationship with the aortic arch curvature and root dilation. Differences between controls and patients were evaluated using Mann-Whitney U test, and the relationship with the curvature was accessed by unpaired t-test. For statistical significance, we consider a P-value of 0.05. The aortic arch curvature was significantly different between patients 46.238 ± 5.581 m-1 and controls 41.066 ± 5.323 m-1. Haemodynamic parameters as wall shear stress circumferential (WSS-C), and eccentricity (ECC), were significantly different between TGA patients and controls in both the root and ascending aorta regions. The distribution of forces along the ascending aorta is highly inhomogeneous in TGA patients. We found that the backward velocity (B-VEL), WSS-C, velocity angle (VEL-A), regurgitation fraction (RF), and ECC are highly correlated with the aortic arch curvature and root dilatation. CONCLUSION: We have identified six potential biomarkers (B-VEL, WSS-C, VEL-A, RF, and ECC), which may be helpful for follow-up evaluation and early prediction of aortic root dilatation in this patient population.

Is slim better than paunchy?

BACKGROUND: Aortopulmonary mismatch (APM) in transposition of the great arteries (TGA) may be accountable for dilatation of neoaortic root and regurgitation of neoaortic valve as late complications of arterial switch operation (ASO). The manuscript from Arcieri et al. highlights an important aspect of the ASO technique: should we approach APM during ASO and what would be the best strategy to do so? DISCUSSION: Techniques to approach APM at the time of ASO have been published sporadically and are very rarely employed by surgeons. Reconstruction of the neoaorta is generally achieved by trimming the suture line between the pulmonary root (neoaortic root) and ascending aorta resulting in an abnormal dilated and bulky neoaortic root already at the time of ASO. CONCLUSION: Reduction of the pulmonary root dimension by ressection of a fragment of the pulmonary artery wall possibly results in a more homogenous neoaorta with consequent better hemodynamics. We believe that approaching APM during ASO will have a compelling positive impact on the late survival of patients with complex TGA.

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

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.

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.

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!

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.

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.

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.

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.

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.

Prática de exercícios em indivíduo com transposição de grandes artérias submetido à cirurgia de Jatene / Exercise practice in an individual with transposition of large arteries who underwent Jatene surgery

INTRODUÇÃO: Historicamente, a cardiopatia congênita traz redução da qualidade de vida da criança portadora da patologia. Com o aprimoramento das formas de correção, estes pacientes apresentam melhor sobrevida, de forma que hoje há mais adultos com cardiopatia congênita. A prática de atividade física vem sendo encorajada nesses pacientes. Relatamos um caso de um paciente portador de cardiopatia congênita cianótica corrigida nos primeiros dias de vida, atualmente praticante de atividade física. Relato de caso: Paciente masculino, 26 anos, com dextro transposição de grandes artérias (DTGA), comunicação interatrial e persistência do canal arterial. Aos 09 dias de vida, submetido a procedimento para correção da cardiopatia, por meio da cirurgia de Jatene (CJ) com manobra de Lecompte. Aos 18 anos manifestou desejo de praticar atividade física, sendo feita avaliação pré-participação. Cintilografia do Miocárdio com ausência de sinais de isquemia; Teste ergométrico (TE) com boa capacidade funcional, Extras sístoles ventriculares isoladas e raras; ecocardiograma transtorácico dentro da normalidade. Atualmente realiza atividade física resistida (musculação 80 min por dia, 6 dias por semana), sem sintomas durante a prática ou o repouso. Em exames atuais observa--se: TE em ritmo atrial multifocal, sem alterações sugestivas de isquemia, com boa capacidade aeróbica (16,2 METs e VO2 56 mL/kg. min); Ressonância miocárdica com câmaras cardíacas de dimensões preservadas, função sistólica biventricular preservada, ausência de áreas de edema, gordura ou fibrose. DISCUSSÃO e CONCLUSÃO: A DTGA corresponde a cerca de 8% de todas as cardiopatias congênitas, com alto índice de letalidade antes do surgimento de correções cirúrgicas, chegando a 90% de mortalidade no primeiro ano de vida. Inicialmente reduzia-se danos da DTGA através de correção atrial. Com o desenvolvimento da CJ (ou switch arterial) houve melhora progressiva do prognóstico e qualidade de vida, principalmente se realizada nos primeiros dias de vida. Devido a isso, muitos pacientes submetidos à CJ praticam exercícios físicos ou atividades competitivas, tornando o acompanhamento um desafio para o cardiologista, visto que não há evidências robustas sobre os impactos de tais atividades a longo prazo. Conclui-se a importância de seguimento com exames periódicos de tais pacientes, com coleta de dados e a necessidade de mais estudos para este cenário.

Impact of pulmonary artery reduction during arterial switch operation: 14 years follow-up.

OBJECTIVES: Pulmonary artery/aorta (PA/Ao) size discrepancy plays an important role in the development of neoaortic root growth and valve regurgitation. Since 2004, we started using PA reduction to manage severe great vessels root mismatch at the time of arterial switch operation. The purpose of this study is to evaluate the impact of this technique in the mid- and long-term follow-up. METHODS: Patients considered to have severe PA/Ao mismatch (>2:1 ratio) underwent resection of a 3- to 4-mm flap of the posterior PA wall. Patients submitted to this technique were followed up with clinical and image examinations. Echocardiographic findings were reviewed, and Z-scores were recorded to evaluate the incidence and progression of neoaortic root dilatation and valve regurgitation. RESULTS: The median (Q1-Q3) follow-up time was 8 years (3-11). Before arterial switch operation, the median (Q1-Q3) Z-score of the PA annulus was 2.90 (2.75-3.75). At the latest follow-up, the median Z-score of the neoaortic annulus was 1.34 (0.95-1.66). The mean difference between the Z-scores of PA annulus and neoaortic annulus was 1.56 (P < 0.0001). The mean value of the sinus of Valsalva was +0.29 ± 1, that of sinotubular junction was +0.71 ± 0.6 and that of ascending Ao was +1.09 ± 0.7. There was no severe dilatation of the neoaortic annulus, neoaortic root or ascending Ao during follow-up. Neoaortic valve regurgitation was none or mild in 93% of patients. CONCLUSIONS: PA reduction proved to be a feasible and low-risk procedure to approach PA/Ao mismatch in arterial switch operation. Mid- and long-term follow-up showed a tendency towards stabilization of the neoaortic root dilatation and satisfactory valve performance after the procedure. Further investigation is required with a larger population and longer-term follow-up.

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.