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Transposition of the great arteries
Paula Martins, Eduardo Castela
Orphanet Journal of Rare Diseases , 2008, DOI: 10.1186/1750-1172-3-27
Abstract: The diagnosis is confirmed by echocardiography, which also provides the morphological details required for future surgical management. Prenatal diagnosis by foetal echocardiography is possible and desirable, as it may improve the early neonatal management and reduce morbidity and mortality. Differential diagnosis includes other causes of central neonatal cyanosis. Palliative treatment with prostaglandin E1 and balloon atrial septostomy are usually required soon after birth. Surgical correction is performed at a later stage. Usually, the Jatene arterial switch operation is the procedure of choice. Whenever this operation is not feasible, adequate alternative surgical approach should be implemented. With the advent of newer and improved surgical techniques and post operative intensive care, the long-term survival is approximately 90% at 15 years of age. However, the exercise performance, cognitive function and quality of life may be impaired.Transposition of the great arteries; physiologically uncorrected transposition; complete transposition; atrioventricular concordance with ventriculoarterial discordance. The European paediatric cardiac code for this disease is 01.05.01.The transposition of the great arteries was first described by Mathew Baillie in 1797, in the second edition of the book "The Morbid Anatomy of Some of the Most Important Parts of the Human Body". However, the term transposition was only applied in 1814, by Farre, meaning that aorta and pulmonary trunk were placed (positio) across (trans) the ventricular septum.In fact, this congenital cardiac malformation is characterised by atrioventricular concordance and ventriculoarterial discordance. In other words, the morphological right atrium is connected to the morphological right ventricle which gives rise entirely to or most of the aorta; the morphological left atrium is connected to the morphological left ventricle from where the pulmonary trunk emerges [1].The term congenitally corrected transposition
Transposition of Great Arteries: New Insights into the Pathogenesis  [PDF]
Marta Unolt,Carolina Putotto,Lucia M. Silvestri,Dario Marino,Alessia Scarabotti,Valerio Massaccesi,Angela Caiaro,Paolo Versacci,Bruno Marino
Frontiers in Pediatrics , 2013, DOI: 10.3389/fped.2013.00011
Abstract: Transposition of great arteries (TGA) is one of the most common and severe congenital heart diseases (CHD). It is also one of the most mysterious CHD because it has no precedent in phylogenetic and ontogenetic development, it does not represent an alternative physiological model of blood circulation and its etiology and morphogenesis are still largely unknown. However, recent epidemiologic, experimental, and genetic data suggest new insights into the pathogenesis. TGA is very rarely associated with the most frequent genetic syndromes, such as Turner, Noonan, Williams or Marfan syndromes, and in Down syndrome, it is virtually absent. The only genetic syndrome with a strong relation with TGA is Heterotaxy. In lateralization defects TGA is frequently associated with asplenia syndrome. Moreover, TGA is rather frequent in cases of isolated dextrocardia with situs solitus, showing link with defect of visceral situs. Nowadays, the most reliable method to induce TGA consists in treating pregnant mice with retinoic acid or with retinoic acid inhibitors. Following such treatment not only cases of TGA with d-ventricular loop have been registered, but also some cases of congenitally corrected transposition of great arteries (CCTGA). In another experiment, the embryos of mice treated with retinoic acid in day 6.5 presented Heterotaxy, suggesting a relationship among these morphologically different CHD. In humans, some families, beside TGA cases, present first-degree relatives with CCTGA. This data suggest that monogenic inheritance with a variable phenotypic expression could explain the familial aggregation of TGA and CCTGA. In some of these families we previously found multiple mutations in laterality genes including Nodal and ZIC3, confirming a pathogenetic relation between TGA and Heterotaxy. These overall data suggest to include TGA in the pathogenetic group of laterality defects instead of conotruncal abnormalities due to ectomesenchymal tissue migration.
Echocardiographic diagnosis of transposition of the great arteries associated with anomalous pulmonary venous connection
Lopes, Lilian Maria;Tavares, Gláucia Maria Penha;Mailho, Fred Luiz;Almeida, Vicente de Paulo Cavalcante de;Mangione, José Armando;
Arquivos Brasileiros de Cardiologia , 2001, DOI: 10.1590/S0066-782X2001000700007
Abstract: we report 2 cases of transposition of the great arteries associated with anomalous pulmonary venous connection emphasizing the clinical findings, the diagnosis, and the evolution of the association. one of the patients had the anomalous pulmonary venous connection in its total infradiaphragmatic form, in the portal system, and the other patient had a partial form, in which an anomalous connection of the left superior lobar vein with the innominate vein existed. at the time of hospital admission, the patients had cyanosis and respiratory distress with clinical findings suggesting transposition of the great arteries. the diagnosis in 1 of the cases, in which the anomalous connection was partial, was established only with echocardiography, without invasive procedures that would represent risk for the patient; in the other case, in which the anomalous connection was total, the malformation was only evidenced with catheterization. the patients underwent surgery for anatomical correction of the heart disease. only 1 patient had a good outcome.
Echocardiographic diagnosis of transposition of the great arteries associated with anomalous pulmonary venous connection
Lopes Lilian Maria,Tavares Gláucia Maria Penha,Mailho Fred Luiz,Almeida Vicente de Paulo Cavalcante de
Arquivos Brasileiros de Cardiologia , 2001,
Abstract: We report 2 cases of transposition of the great arteries associated with anomalous pulmonary venous connection emphasizing the clinical findings, the diagnosis, and the evolution of the association. One of the patients had the anomalous pulmonary venous connection in its total infradiaphragmatic form, in the portal system, and the other patient had a partial form, in which an anomalous connection of the left superior lobar vein with the innominate vein existed. At the time of hospital admission, the patients had cyanosis and respiratory distress with clinical findings suggesting transposition of the great arteries. The diagnosis in 1 of the cases, in which the anomalous connection was partial, was established only with echocardiography, without invasive procedures that would represent risk for the patient; in the other case, in which the anomalous connection was total, the malformation was only evidenced with catheterization. The patients underwent surgery for anatomical correction of the heart disease. Only 1 patient had a good outcome.
Ventricular assist device outflow graft in congenitally corrected transposition of great arteries - a surgical challenge  [cached]
Mohite Prashant N,Popov Aron F,Garcia Diana,Hards Rachel
Journal of Cardiothoracic Surgery , 2012, DOI: 10.1186/1749-8090-7-93
Abstract: Congenitally corrected transposition of the great arteries is a complex congenital cardiac anomaly with a wide spectrum of morphologic features and clinical profiles. Most patients are diagnosed late in their life, undergoes surgical repairs, eventually leading to systemic ventricular failure needing heart transplant or mechanical circulatory assistance. As, aorta is located anterior to and left of the PA (Transposition of great arteries), the outflow graft of ventricular assist device traverse across pulmonary artery to reach aorta which poses challenge during further surgical explorations.
Transposición corregida de grandes vasos Corrected transposition of the great arteries
José Rafael Escalona Aguilera,Alberto Ramírez Ramos,Marcia López García
Revista Cubana de Pediatr?-a , 2012,
Abstract: Se presenta una paciente de sexo femenino, de 9 a os de edad, que acude a su médico de familia y se le auscultó un soplo al examen físico y sintomatología que se comportaba como una comunicación interventricular. Luego de los estudios correspondientes, se permitió hacer el diagnóstico de transposición corregida de grandes vasos. La paciente tenía asociada una anomalía de Ebstein, comunicación interauricular, una comunicación interventricular, y una persistencia del conducto arterioso, todo lo cual agravaba su cuadro clínico. Primero es intervenida quirúrgicamente, y se cierra el ducto; de manera espontánea, cierra la comunicación interauricular, y la anomalía de Ebstein parchea la comunicación interventricular. La ni a desarrolla una insuficiencia cardiaca e hipertensión pulmonar, a consecuencia de las anomalías asociadas. El caso es de interés, ya que son pocos los publicados en la literatura internacional con esta cardiopatía congénita asociada a otras anomalías estructurales del corazón, que modifican su historia natural. This paper presented a 9 years-old female patient, who went to the family's doctor and was physically examined to detect heart murmur and symptoms of ventricular septal defect. After the corresponding studies, the diagnosis was the corrected transposition of the great arteries. The patient also suffered Ebstein anomaly, atrial septal defect, ventricular septal defect and persistence of arteriose duct, all of which worsened her clinical picture. First, she was operated on and the duct was closed. Later the atrial septal defect closed spontaneously, and the Ebstein anomaly patched the ventricular septal defect. The girl developed heart failure and pulmonary hypertension, as a result of associated anomalies. The case is interesting since few cases like this one, associated with other structural heart anomalies that change its natural course, are presented in the international literature.
Follow-up of our patients with transposition of the great arteries and arterial switch operation; comparison of simple and complex transposition cases  [cached]
Osman Akdeniz,Canan Ayabakan,Uygar Y?rüker,Kür?ad Tokel
Anadolu Kardiyoloji Dergisi , 2011,
Abstract: Objective: 1. Follow-up data of patients with simple transposition of great arteries (TGA) and TGA with ventricular septal defect (VSD), who had arterial switch operation (ASO) are compared. 2. Factors affecting mortality and morbidity after ASO are described.Methods: Seventy-six patients, who had an ASO between April 2007 and August 2010 were studied retrospectively. The patients with intact ventricular septum (IVS) (n=36) were in Group1, and those with VSD (n=40) in Group 2. The pre and postoperative clinical and echocardiographic variables and intensive care unit (ICU) outcomes were compared among groups using Mann-Whitney U, Pearson correlation and logistic regression tests.Results: The mean age at operation was 44.1 days, weight was 3.6±0.98 kg. Patients were followed for 15.5±11.21 months. The aortic cross-clamp (AoCC) and cardiopulmonary bypass (CPB) times were higher in patients with VSD (p=0.001, p=0.004). Patients in Group 1 had longer inotropic agent infusion (p=0.001). Length of stay in ICU was similar in two groups (p>0.05). There was no correlation between the length of stay in ICU and age, weight, CPB time, AoCC time. Aortic regurgitation was more frequent in Group 2 (p=0.02). During follow-up, 12 patients died (15.7%), and 8 patients had a revision operation (10.5%) (diaphragmatic plication in 4, pulmonary artery reconstruction in 1, recoarctation operation in 3 patients). Mortality was similar in groups (p>0.05).Conclusion: Arterial switch operation provides anatomical correction in TGA. Appropriate timing and good perioperative planning facilitates low morbidity and mortality in patients with VSD as in patients with simple TGA.
Corrected transposition of the great arteries with several associated anomalies in a 68-year-old patient
Jacob, José Luiz Balthazar;
Arquivos Brasileiros de Cardiologia , 2001, DOI: 10.1590/S0066-782X2001001000005
Abstract: few patients with corrected transposition of the great arteries survive past 50 years of age because of the association with congenital defects, development of total atrioventricular block, and right ventricular dysfunction. we report the case of a male patient with dextrocardia in situs solitus and corrected transposition of the great arteries associated with a wide atrial septal defect and severe pulmonary valvar and subvalvar stenoses. the patient also developed a large aneurysm on the pulmonary artery, total atrioventricular block diagnosed 8 years earlier, symptoms of dysfunction of the systemic ventricle in the previous 2 years, insufficiency of the left atrioventricular valve, and aortic regurgitation. despite all these associated anomalies, the patient developed class iii cardiac decompensation only at the age of 68 years, which makes this case a rarity. the patient was clinically treated, and was discharged from the hospital in good condition.
Corrected transposition of the great arteries with several associated anomalies in a 68-year-old patient
Jacob José Luiz Balthazar
Arquivos Brasileiros de Cardiologia , 2001,
Abstract: Few patients with corrected transposition of the great arteries survive past 50 years of age because of the association with congenital defects, development of total atrioventricular block, and right ventricular dysfunction. We report the case of a male patient with dextrocardia in situs solitus and corrected transposition of the great arteries associated with a wide atrial septal defect and severe pulmonary valvar and subvalvar stenoses. The patient also developed a large aneurysm on the pulmonary artery, total atrioventricular block diagnosed 8 years earlier, symptoms of dysfunction of the systemic ventricle in the previous 2 years, insufficiency of the left atrioventricular valve, and aortic regurgitation. Despite all these associated anomalies, the patient developed class III cardiac decompensation only at the age of 68 years, which makes this case a rarity. The patient was clinically treated, and was discharged from the hospital in good condition.
The Difficulties in Determining Time of the Operation in the Total Corrected Transposition of the Great Arteries with Severe Left AV Valve Regurgitation: Case Report
Erden ? et al.
Konuralp Tip Dergisi , 2010,
Abstract: Congenitally corrected transposition of the great arteries (L-TGA) is characterized by discordance between the atria and ventricles, as well as between the ventricles and the great arteries. As a result, the morphologic right ventricle lies to the left of the morphologic left ventricle and becomes the systemic ventricle. The morphologic right ventricle gives rise to the aorta, which is anterior of the pulmonary artery. As a result of discordance at both the atrioventricular (AV) and the great vessel level, physiologic blood flow remains normal. It was shown that the decreased RV functions are related with increased mortality rates in long term follow up. Most of these patients have RV failure due to abnormal pressure load of systemic RV, imbalance between O2 supply and myocardial demand and development of systemic AV valvular regurgitation in most of the patients. Therefore, the most important and difficult point in the follow up of these patients is evaluation of RV functions and degree of systemic AV valve regurgitation. In this review, we discussed the difficulties in the diagnosis and treatment of these patients in the light of a typical TGA case.
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