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Transposition of the great arteries.

Martins P, Castela E - Orphanet J Rare Dis (2008)

Bottom Line: Some associated risk factors (gestational diabetes mellitus, maternal exposure to rodenticides and herbicides, maternal use of antiepileptic drugs) have been postulated.Prenatal diagnosis by foetal echocardiography is possible and desirable, as it may improve the early neonatal management and reduce morbidity and mortality.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Serviço de Cardiologia Pediátrica, Hospital Pediátrico de Coimbra, Coimbra, Portugal. paula_mrtns@yahoo.com

ABSTRACT
Transposition of the great arteries (TGA), also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial (VA) discordance. The incidence is estimated at 1 in 3,500-5,000 live births, with a male-to-female ratio 1.5 to 3.2:1. In 50% of cases, the VA discordance is an isolated finding. In 10% of cases, TGA is associated with noncardiac malformations. The association with other cardiac malformations such as ventricular septal defect (VSD) and left ventricular outflow tract obstruction is frequent and dictates timing and clinical presentation, which consists of cyanosis with or without congestive heart failure. The onset and severity depend on anatomical and functional variants that influence the degree of mixing between the two circulations. If no obstructive lesions are present and there is a large VSD, cyanosis may go undetected and only be perceived during episodes of crying or agitation. In these cases, signs of congestive heart failure prevail. The exact aetiology remains unknown. Some associated risk factors (gestational diabetes mellitus, maternal exposure to rodenticides and herbicides, maternal use of antiepileptic drugs) have been postulated. Mutations in growth differentiation factor-1 gene, the thyroid hormone receptor-associated protein-2 gene and the gene encoding the cryptic protein have been shown implicated in discordant VA connections, but they explain only a small minority of TGA cases.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.

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The figure shows the main steps of Rastelli procedure in the setting of a deviated outlet septum (A). A patch is placed to create an interventricular tunnel (B), and an extracardiac conduit is placed between the right ventricle and the pulmonary arteries (C). (adapted from [10]. Martins P, Tran V, Price G, Tsang V and Cook A, Cardiol Young 2008; 18:124–34, with permission from Cambridge University Press).
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Figure 8: The figure shows the main steps of Rastelli procedure in the setting of a deviated outlet septum (A). A patch is placed to create an interventricular tunnel (B), and an extracardiac conduit is placed between the right ventricle and the pulmonary arteries (C). (adapted from [10]. Martins P, Tran V, Price G, Tsang V and Cook A, Cardiol Young 2008; 18:124–34, with permission from Cambridge University Press).

Mentions: In the presence of a ventricular septal defect, the most used options are the REV procedure or its modification and the Rastelli operation. In both, an intraventricular tunnel passing through the septal defect is created to connect the left ventricle to the aorta. In the Rastelli procedure, an extracardiac conduit is placed connecting the right ventricle to the pulmonary artery (Fig. 8). However, in the REV technique, this is accomplished through the LeCompte manouever, which brings the pulmonary trunk forward, allowing its direct implantation in the right ventricle (Fig. 9). Apart from avoiding the use of an extracardiac conduit, the REV procedure has a further advantage in which it involves the resection of the muscular outlet septum, providing better alignment between the aorta and the left ventricle. As expected, late results in terms of reoperation are significantly different in the two procedures. In fact, Rastelli operation was associated with a greater risk of reintervention due to left ventricular outflow tract obstruction, and extracardiac conduit problems such as obstruction, requiring eventual replacement. However, similar early and late mortalities were reported [43,44].


Transposition of the great arteries.

Martins P, Castela E - Orphanet J Rare Dis (2008)

The figure shows the main steps of Rastelli procedure in the setting of a deviated outlet septum (A). A patch is placed to create an interventricular tunnel (B), and an extracardiac conduit is placed between the right ventricle and the pulmonary arteries (C). (adapted from [10]. Martins P, Tran V, Price G, Tsang V and Cook A, Cardiol Young 2008; 18:124–34, with permission from Cambridge University Press).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2577629&req=5

Figure 8: The figure shows the main steps of Rastelli procedure in the setting of a deviated outlet septum (A). A patch is placed to create an interventricular tunnel (B), and an extracardiac conduit is placed between the right ventricle and the pulmonary arteries (C). (adapted from [10]. Martins P, Tran V, Price G, Tsang V and Cook A, Cardiol Young 2008; 18:124–34, with permission from Cambridge University Press).
Mentions: In the presence of a ventricular septal defect, the most used options are the REV procedure or its modification and the Rastelli operation. In both, an intraventricular tunnel passing through the septal defect is created to connect the left ventricle to the aorta. In the Rastelli procedure, an extracardiac conduit is placed connecting the right ventricle to the pulmonary artery (Fig. 8). However, in the REV technique, this is accomplished through the LeCompte manouever, which brings the pulmonary trunk forward, allowing its direct implantation in the right ventricle (Fig. 9). Apart from avoiding the use of an extracardiac conduit, the REV procedure has a further advantage in which it involves the resection of the muscular outlet septum, providing better alignment between the aorta and the left ventricle. As expected, late results in terms of reoperation are significantly different in the two procedures. In fact, Rastelli operation was associated with a greater risk of reintervention due to left ventricular outflow tract obstruction, and extracardiac conduit problems such as obstruction, requiring eventual replacement. However, similar early and late mortalities were reported [43,44].

Bottom Line: Some associated risk factors (gestational diabetes mellitus, maternal exposure to rodenticides and herbicides, maternal use of antiepileptic drugs) have been postulated.Prenatal diagnosis by foetal echocardiography is possible and desirable, as it may improve the early neonatal management and reduce morbidity and mortality.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Serviço de Cardiologia Pediátrica, Hospital Pediátrico de Coimbra, Coimbra, Portugal. paula_mrtns@yahoo.com

ABSTRACT
Transposition of the great arteries (TGA), also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial (VA) discordance. The incidence is estimated at 1 in 3,500-5,000 live births, with a male-to-female ratio 1.5 to 3.2:1. In 50% of cases, the VA discordance is an isolated finding. In 10% of cases, TGA is associated with noncardiac malformations. The association with other cardiac malformations such as ventricular septal defect (VSD) and left ventricular outflow tract obstruction is frequent and dictates timing and clinical presentation, which consists of cyanosis with or without congestive heart failure. The onset and severity depend on anatomical and functional variants that influence the degree of mixing between the two circulations. If no obstructive lesions are present and there is a large VSD, cyanosis may go undetected and only be perceived during episodes of crying or agitation. In these cases, signs of congestive heart failure prevail. The exact aetiology remains unknown. Some associated risk factors (gestational diabetes mellitus, maternal exposure to rodenticides and herbicides, maternal use of antiepileptic drugs) have been postulated. Mutations in growth differentiation factor-1 gene, the thyroid hormone receptor-associated protein-2 gene and the gene encoding the cryptic protein have been shown implicated in discordant VA connections, but they explain only a small minority of TGA cases.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.

Show MeSH
Related in: MedlinePlus