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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.

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Transposition of the great arteries (TGA) is a cyanotic congenital heart disease characterized by ventriculoarterial discordance and atrioventricular concordance with the great arteries in a parallel relationship. Prenatal diagnosis of TGA has implications for postnatal outcomes, allowing for planned delivery and perinatal management. Three-dimensional virtual or physical models of fetal TGA allow better understanding of fetal cardiac anomalies by parents and interactive discussion among the multidisciplinary team (obstetricians, pediatricians, maternal-fetal specialists, pediatric cardiologists, and cardiovascular surgeons), as well as continuing medical education.

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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.

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Congenitally corrected transposition of the great arteries is a rare clinical entity, which usually presents during adulthood with associated defects; atrioventricular block, heart failure, systemic valve failure, and arrhythmias usually complicate the clinical course. Even rarer is associated hypertrophic cardiomyopathy, which complicates the disease course and clinical decision-making. Herein, we present a patient with this condition who underwent heart transplantation, with adequate clinical resolution.

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OBJECTIVE: The objective of the study was to compare the use of neonatal conventional brain magnetic resonance imaging (MRI) with that of clinical factors and socioeconomic status (SES) to predict long-term neurodevelopment in children with severe congenital heart disease (CHD). STUDY DESIGN: In this prospective cohort study, perioperative MRIs were acquired in 57 term-born infants with CHD undergoing cardiopulmonary bypass surgery during their first year of life. Total brain volume (TBV) was measured using an automated method. Brain injury severity (BIS) was assessed by an established scoring system. The neurodevelopmental outcome was assessed at 6 years using standardized test batteries. A multiple linear regression model was used for cognitive and motor outcomes with postoperative TBV, perioperative BIS, CHD complexity, length of hospital stay, and SES as covariates. RESULTS: CHD diagnoses included univentricular heart defect (n = 15), transposition of the great arteries (n = 33), and acyanotic CHD (n = 9). Perioperative moderate-to-severe brain injury was detected in 15 (26%) patients. The total IQ was similar to test norms (P = .11), whereas the total motor score (P < .001) was lower. Neither postoperative TBV nor perioperative BIS predicted the total IQ, but SES (P < .001) and longer hospital stay (P = .004) did. No factor predicted the motor outcome. CONCLUSION: Although the predictive value of neonatal conventional MRIs for long-term neurodevelopment is low, duration of hospital stay and SES better predict the outcome in this CHD sample. These findings should be considered in initiating early therapeutic support.

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OBJECTIVE: To identify predictors of impaired executive function in adolescents after surgical repair of critical congenital heart disease (CHD). STUDY DESIGN: We analyzed patient factors, medical and surgical history, and family social class from 3 single-center studies of adolescents with d-transposition of the great arteries (d-TGA), tetralogy of Fallot (TOF), and Fontan repair. Machine learning models were developed using recursive partitioning to predict an executive function composite score based on five subtests (population mean 10, SD 3) of the Delis-Kaplan Executive Function System. RESULTS: The sample included 386 patients (139 d-TGA, 91 TOF, 156 Fontan) of age 15.1 ± 2.1 (mean ± SD) years and an executive function composite score of 8.6 ± 2.4. Family social class emerged as the most important predictive factor. The lowest (worst) mean executive function score (5.3) occurred in patients with low to medium social class (Hollingshead index <56) with one or more neurologic events and a diagnosis of TOF. The highest (best) mean score (9.7) occurred in subjects with high social class (Hollingshead index ≥56) and shorter duration of deep hypothermic circulatory arrest. Other factors predicting lower executive function scores included low birth weight and a greater number of catheterizations. CONCLUSIONS: In regression tree modeling, family social class was the strongest predictor of executive function in adolescents with critical CHD, even in the presence of medical risk factors. Additional predictors included CHD diagnosis, birth weight, neurologic events, and number of procedures. These data highlight the importance of social class in mitigating risks of executive dysfunction in CHD.

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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.

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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.

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Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

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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.

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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.

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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.

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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.

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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.

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BACKGROUND: Transposition of the great arteries is the most common cyanotic cardiac lesion in newborns. Transposition of the great arteries without surgical correction is fatal during the first year of life. Contemporary outcome studies have shown that survival rates after surgery are excellent and most patients live to adulthood. CASE SUMMARY: Woman with complex transposition of the great arteries with atrial and ventricular septal defects and subvalvular and valvular pulmonary stenosis, who has survived until the age of 31 years without surgery. The diagnosis was made by echocardiography and cardiac magnetic resonance. She underwent successful corrective surgical treatment after this age, by means of a Jatene operation. CONCLUSION: In transposition of the great arteries patients, a high index of cases dies in the first month of life. Our case represents a natural history of the complex transposition of the great arteries. Non-invasive imaging studies are very useful for the diagnosis and follow-up of patients with transposition of the great arteries, especially echocardiography and cardiac magnetic resonance. In our case, the multimodality approach and the corrective surgery allowed her to survive.

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