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Circulating Annexin A5 Levels after Atrial Switch for Transposition of the Great Arteries: Relationship with Ventricular Deformation and Geometry  [PDF]
Clare T. M. Lai, Pak-cheong Chow, Sophia J. Wong, Koon-wing Chan, Yiu-fai Cheung
PLOS ONE , 2012, DOI: 10.1371/journal.pone.0052125
Abstract: Background Inflammatory cytokines, cardiomyocyte apoptosis, and altered collagen turnover may contribute to unfavourable ventricular remodeling. This unfavourable ventricular remodelling is well documented in patients after atrial switch operation for complete transposition of the great arteries. We therefore tested if levels of circulating markers of inflammation, apoptosis, collagen synthesis, and extracellular matrix degradation are altered in patients after atrial switch operation for transposition of the great arteries. Methods and Results Circulating tumour necrosis factor (TNF)-α, annexin A5 (AnxA5), carboxy-terminal propeptide of type I procollagen (PICP), amino-terminal propeptide of type III procollagen (PIIINP), matrix metalloproteinase-1 (MMP-1), and tissue inhibitor of metalloproteinase-1 (TIMP-1) levels were determined in 27 patients aged 25.2±3.1 years and 20 controls. Ventricular myocardial deformation and left ventricular eccentricity index (EI) were determined by speckle tracking and two-dimensional echocardiography, respectively. Compared with controls, patients had significantly higher circulating AnxA5 (p<0.001) and TNF-α (p = 0.018) levels, but similar PICP, PIIINP, MMP-1 and TIMP-1 levels. For the whole cohort, plasma AnxA5 correlated with serum TNF-α (p = 0.002), systemic ventricular global longitudinal strain (GLS) and systolic and early diastolic strain rate (all p<0.001), and subpulmonary ventricular GLS and early diastolic strain rate (both p<0.001). In patients, plasma AnxA5 level correlated positively with subpulmonary ventricular EI (p = 0.027). Multiple linear regression analysis identified systemic ventricular GLS (β = ?0.50, p<0.001) and serum TNF-α (β = 0.29, p = 0.022) as significant correlates of plasma AnxA5. Conclusions Elevated plasma AnxA5 level in patients after atrial switch operation is associated with impaired systemic myocardial deformation, increased subpulmonary ventricular eccentricity, and increased serum TNF-α level.
Noninvasive Assessment of Autonomic Cardiovascular Function in Patients after Arterial Switch Operation for Transposition of the Great Arteries  [PDF]
Joanna Hlebowicz, Maja Rooth, Sandra Lindstedt, Johan Holm, Ulf Thilén
Surgical Science (SS) , 2015, DOI: 10.4236/ss.2015.63020
Abstract:

Background: Children born with transposition of the great arteries (TGA) must undergo corrective surgery for survival, arterial switch being the standard surgical procedure. The sympathetic innervation of the heart may be damaged during the operation. This study was designed to determine whether adults who were born with TGA and who had arterial switch operation (ASO) in infancy exhibit denervation of the heart, measured as heart rate variability (HRV) with electrocardiography (ECG). Methods: Nine patients with transposition of the great arteries (four men and five women; mean age 26 ± 1 years) who underwent the ASO at a mean age of 85 ± 35 days, and nine healthy adults (five men and five women; mean age 26 ± 2) were included in the study. Cardiac autonomic nerve function was determined by the variation in RR intervals during maximal deep breathing, monitored by continuous ECG. The mean values were calculated for each group from six inspirations (I) and expirations (E), and the E:I ratios were calculated. Results: The E:I ratio did not differ between patients with an arterial switch and healthy controls (P= 0.161). Two patients had signs of denervation of the heart up to 30 years after the arterial switch operation. Conclusions: Reinnervation of the heartmay take place in patients who have undergone the ASO in infancy, and these patients would not necessarily suffer from autonomic dysfunction. The HRV, measured by ECG, has the potential to identify arterially switched patients at risk of developing silent myocardial ischemia.

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.
Midterm results after arterial switch operation for transposition of the great arteries: a single centre experience  [cached]
Popov Aron Frederik,Tirilomis Theodor,Giesler Michael,Oguz Coskun Kasim
Journal of Cardiothoracic Surgery , 2012, DOI: 10.1186/1749-8090-7-83
Abstract: Background The arterial switch operation (ASO) has become the surgical approach of choice for d-transposition of the great arteries (d-TGA). There is, however an increased incidence of midterm and longterm adverse sequelae in some survivors. In order to evaluate operative risk and midterm outcome in this population, we reviewed patients who underwent ASO for TGA at our centre. Methods In this retrospective study 52 consecutive patients with TGA who underwent ASO between 04/1991 and 12/1999 were included. To analyze the predictors for mortality and adverse events (coronary stenoses, distortion of the pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation), a multivariate analysis was performed. The follow-up time was ranged from 1–10 years (mean 5 years, cumulative 260 patient-years). Results All over mortality rate was 15.4% and was only observed in the early postoperative period till 1994. The predictors for poor operative survival were low APGAR-score, older age at surgery, and necessity of associated surgical procedures. Late re-operations were necessary in 6 patients (13.6%) and included a pulmonary artery patch enlargement due to supravalvular stenosis (n = 3), coronary revascularisation due to coronary stenosis in a coronary anatomy type E, aortic valve replacement due to neoaortic valve regurgitation (n = 2), and patch-plasty of a pulmonary vein due to obstruction (n = 1). The dilatation of neoaortic root was not observed in the follow up. Conclusions ASO remains the procedure of choice for TGA with acceptable early and late outcome in terms of overall survival and freedom of reoperation. Although ASO is often complex and may be associated with morbidity, most patients survived without major complications even in a small centre.
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
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
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.
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.
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