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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.
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.
Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation
Ad JJC Bogers, Stuart J Head, Peter L de Jong, Maarten Witsenburg, Arie Kappetein
Journal of Cardiothoracic Surgery , 2010, DOI: 10.1186/1749-8090-5-74
Abstract: A total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.Excluding the cardiac transplantation patients, mean age at operation was 16 years (sd 15 years, range 1 week - 49 years). Median follow-up was 12 years (sd 10 years, range 7 days - 32 years). Survival obtained from Kaplan-Meier analysis at 20 years after surgery was 63% (CI 53-73%). For the non-Rastelli group these data at 20 years were 62% (CI 48-76%) and for the Rastelli group 67% (CI 51-83%). Freedom of reoperation at 20 years was 32% (CI 19-45%) in the overall group. In the non-Rastelli group the data at 20 years were 47% (CI 11-83%) and for the Rastelli group 21% (CI 0-54%) after almost 19 years.Long term follow up confirms that surgery in CCTGA with the right ventricle as systemic ventricle has a suboptimal survival and limited freedom of reoperation. Death occurred mostly as a result of cardiac failure.Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac anomaly with an incidence of less than 1% of patients with congenital heart disease [1]. Characteristically the right atrium is connected to the morphologically left ventricle, which connects to the pulmonary artery and the left atrium is connected to the morphologically right ventricle, which connects to the aorta, resulting in atrio-ventricular discordance and ventriculo-arterial discordance, or double discordance [2]. In 90% of these patients associated anomalies are present as well, with ventricular septal defect as the most common, followed by pulmonary stenosis and atrial septal defect [1,3-5].The prognosis of patients with CCTGA is variable with some patients showing satisfactory long-term survival [1,5-7]. However, both deteriorating right ventricular function o
Corrected Transposition of the Great Arteries with Ebstein’s Anomaly, Dysplasia of the Mitral Leaflets and Persistence of Left Superior Vena Cava in an Adult  [PDF]
Aureo Campos-Tello, Giancarlo A. Valle, Luis M. Milla, Angel D. Cueva, Sara Ramirez-Flores, Oscar García-Rosadio, Pablo A. Mendoza, Nilda Espinola-Zavaleta
World Journal of Cardiovascular Diseases (WJCD) , 2020, DOI: 10.4236/wjcd.2020.102006
Abstract: Introduction: Congenitally corrected transposition of the great arteries (cc-TGA) is a congenital heart disease rarely described among adult population, especially if it is associated with other abnormalities such as Ebstein’s anomaly and abnormal origin of coronary arteries. Case presentation: Twenty-two-year-old woman admitted to intensive care unit with acute decompensated heart failure. The transthoracic echocardiography demonstrated atrioventricular and ventriculoarterial discordance. The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein’s anomaly. The computed tomography corroborated these findings and additionally showed a left superior vena cava and an abnormal origin of the coronary arteries. The patient is in NYHA functional class II, receiving treatment with diuretic, digitalis, beta-blockers and angiotensin-converting enzyme (ACE) inhibitor. Conclusion: This is a very interesting case of an adult in the third decade of life with cc-TGA associated with abnormalities not described in the literature that had reached adulthood. Noninvasive images play an important role in its diagnosis.
A Case of Congenitally Corrected Transposition of the Great Arteries Discovered on Coronary Computed Tomography  [PDF]
Adam T. Marler,Jennifer N. Slim,Travis Batts,James Watts,Ahmad M. Slim
Case Reports in Vascular Medicine , 2013, DOI: 10.1155/2013/420213
Abstract: Congenitally corrected transposition of the great arteries is a rare condition accounting for less than 1% of all congenital cardiac diseases. The fundamental nature of this condition involves a blend of atrioventricular as well as ventriculoarterial discordance. Congenitally corrected transposition of the great arteries is classically associated with three additional abnormalities, including ventricular septal defect, right ventricular outflow tract obstruction, and tricuspid valve abnormalities. Patients with this anomaly have been shown to exhibit reduced exercise tolerance as well as reduced health-related quality of life when compared to patients with normal cardiovascular anatomy. We present the case of a 33-year-old active duty lieutenant in the United States Air Force referred to the cardiology clinic for evaluation of valvular heart disease with subsequent discovery of congenitally corrected transposition of the great arteries on cardiac gated computed tomography. 1. Case Report A 33-year-old male with a history of secundum atrial septal defect percutaneously ended in May 2008 referred for further evaluation of valvular heart disease. Transthoracic echocardiogram completed for a heart murmur was noted to be technically difficult with suboptimal image quality due to mesocardia. Official report from this examination detailed grossly normal left ventricular wall motion with moderate, posteriorly directed, eccentric mitral regurgitation, mild prolapse of the anterior mitral valve leaflet, mild aortic insufficiency, and an echo bright structure contiguous to the lateral aspect of the tricuspid annulus. The right ventricle and right atrium were not well visualized. At initial visit he reported feeling well overall, but he felt fatigued at 200–400 meters. Exercise stress testing was completed with the patient exercising 12 minutes 26 seconds on a standard Bruce protocol achieving a workload of 14.2 metabolic equivalents (METS) achieving 96% of maximal age-predicted heart rate with exercise. Exercise testing terminated due to fatigue with the patient reporting no chest pain during evaluation. No ischemic changes were noted on electrocardiogram during the study. Patient’s chest X-ray was suggestive of possible RV on the left side of the chest with possible dextrocardia or ccTGA in the differential (Figure 1). Figure 1: X-ray changes anticipated in ccTGA with right ventricular border outlined with red arrow and Amplatzer occluder device outlined with dark arrow. Subsequently, the patient underwent imaging evaluation with cardiac computed tomography (CT)
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.
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
Atrial Fibrillation-Induced Cardiac Shock: First Manifestation of a Congenitally Corrected Transposition of the Great Arteries in a 45-Year-Old Man  [PDF]
M. Graf,M. Zaczkiewicz,J. Torzewski,O. Zimmermann
Case Reports in Cardiology , 2012, DOI: 10.1155/2012/126764
Abstract: Background. The congenitally corrected transposition of the great arteries (L-TGA) is a very rare congenital heart defect, which often remains undetected for several decades of life. Case Presentation. We report on a 45-year-old man without prior history of heart disease, presenting with cardiac shock related to a first episode of tachycardic atrial fibrillation. The diagnostic work-up identified a L-TGA as the underlying cause for acute heart failure. Discussion. L-TGA is a very rare congenital heart defect, which is characterized by an atrioventricular as well as a ventriculoarterial discordance. By this means, the physiological sequence of pulmonary and systemic circulation is still maintained. On the basis of an ongoing strain of the right ventricle, which has to carry the burden of the systemic blood pressure, after more than four decades without symptoms, acute heart failure was triggered by a tachycardic atrial fibrillation. 1. Introduction The congenitally corrected transposition of the great arteries (L-TGA) is a very rare congenital heart defect that represents less than 1% of all congenital heart disorders [1]. In many cases, these patients remain asymptomatic for several decades of life. We report the case of a 45-year-old man, who was admitted to our emergency unit due to haemodynamic instable tachycardic atrial fibrillation. Further diagnostics revealed an—so far unknown—L-TGA. 2. Case Presentation A 45-year-old man was admitted to our emergency unit because of rapidly increasing dyspnoea and pulmonary rales. The patient’s history lacked previous significant illnesses, particularly structural heart or lung diseases and cardiovascular risk factors. The patient described himself up to now as sportive and physically trained, but remarked a general weakness within the last couple of weeks. Since 48?h he sensed palpitations which had now ended up in tachycardia. The electrocardiogram (ECG) showed tachycardic atrial fibrillation with a mean heart rate of 160?bpm. The initial systolic blood pressure amounted not more than 80?mmHg. Under the clinical presentation of acute heart failure, we immediately performed an electrical cardioversion. After two attempts with biphasic application of 150 and 200?Joule of energy, a normofrequent sinus rhythm could be established. Intermitting short episodes of an AV-junctional rhythm were observed. Even now, the patient still showed signs of an ongoing cardiac shock and remained dependent on constant dobutamine supply. Although an ameliorated spontaneous breathing was reported, orthopnoea persisted under the
Palliation for transposition of great arteries
VO Adegboye, SI Omokhodion, O Ogunkunle, AI Brimmo, OA Adepo
Nigerian Journal of Surgical Research , 2003,
Abstract: Background: At the University College Hospital Ibadan we have no facility for total surgical correction of transportation of the great arteries (TGA). This prospective study reviews the palliative procedures we have used in the management of TGA. Method: Patients with the diagnosis of TGA were evaluated for morphological type. The choice of palliative procedure was made in some of the patients with morphological type in mind. No fixed criteria were used for allocating patients to Blalock-Hanlon (B-H), atrial septectomy while pulmonary banding (PB) and Blalock-Taussig (B-T) shunt have definite indications. Results: Fourteen consecutive patients with TGA were palliated. The ages of these patients ranged between 3 to 11 months (6.8 ± 2.4 months), there were 8 males to 6 females (1.3:1). Six patients had B-H atrial septectomy and 2(33.3%) died within 48 hours, 4 patients had B-T shunt and there were no mortality, 4 patients had PB and 2 (50.0%) died within 72 hours. The overall operative mortality was 28.6%. All the 10 survivors had improvement of their clinical features and fall in packed cell volume during the period of follow-up, which lasted 5 to 13 months (mean 9.3 ± 1.2 months). All patients had delayed wound healing. Conclusion: Appropriate and timely palliative surgery has a place in patients with TGA as an interim care. Key Words: Palliative surgery, transposition, great arteries Nigerian Journal of Surgical Research Vol.5(1&2) 2003: 129-133
Hepatitis isquémica en paciente de 81 a os con transposición congénita corregida de grandes vasos Ischemic hepatitis in an 81 year old patient with congenitally corrected transposition of the great arteries  [cached]
R. Labeaga,M. Muniesa,E. Urbiola,F. Jiménez Bermejo
Anales del Sistema Sanitario de Navarra , 2005,
Abstract: La transposición corregida de grandes vasos es una malformación congénita cardiaca extraordinariamente inusual, con muy escasa supervivencia por encima de la sexta década. Todavía más rara es su presencia sin otras anomalías cardiacas asociadas. La hepatitis isquémica es una entidad poco frecuente caracterizada por una elevación de transaminasas y necrosis centrolobulillar debida a una reducción del flujo hepático, generalmente de evolución reversible. Se presenta un caso de hepatitis isquémica de curso fulminante en un paciente de 81 a os con transposición corregida de grandes vasos. Destacan tanto la tórpida evolución de la hepatitis, como la longevidad del paciente. Comentamos los aspectos clínicos y diagnósticos de ambas entidades The congenitally corrected transposition of the great arteries is a rare form of congenital heart disease, with survival beyond the 6th decade of life being rare. Even more unusual is its presentation alone, without any other form of congenital heart disease. Ischemic hepatitis is a rare entity characterized by an elevation of transaminasas and a centrilobular necrosis due to a reduction in hepatic blood flow, generally of reversible evolution. The authors present a case of ischemic hepatitis in an 81-year-old patient with congenitally corrected transposition of the great arteries. The fatal evolution of the episode and the longevity of the patient are both notable. We comment on the clinical and diagnostic aspects of both entities
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