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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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Subcostal view showing discordant ventriculoarterial connections together with the presence of parallel, rather than crossing, great arteries arising form the ventricles.
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Figure 2: Subcostal view showing discordant ventriculoarterial connections together with the presence of parallel, rather than crossing, great arteries arising form the ventricles.

Mentions: The definitive diagnosis relies however on echocardiography. This imaging modality provides accurate morphological and functional assessment of the heart, being able to show the specific features of the transposition of the great arteries. In the four-chamber view, one assesses atrioventricular concordance, but the ventriculoarterial discordance is better observed using other incidences. In the five-chamber, parasternal long-axis or even subcostal view, the vessel arising from the morphologically left ventricle has a posterior course and bifurcates immediately, being identified as the pulmonary trunk (Fig. 1). The morphologically right ventricle is related to a vessel that gives out the coronary, head and neck arteries, thus the aorta. The proximal portions of the two arteries run parallel to each other, rather than in the usual cross pattern, giving it a typical and easily recognisable appearance in the parasternal long-axis and subcostal views (Fig. 2). In the short-axis view, the pulmonary trunk is usually in a central position, with the aorta being placed anteriorly and to the right (Fig. 3).


Transposition of the great arteries.

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

Subcostal view showing discordant ventriculoarterial connections together with the presence of parallel, rather than crossing, great arteries arising form the ventricles.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Subcostal view showing discordant ventriculoarterial connections together with the presence of parallel, rather than crossing, great arteries arising form the ventricles.
Mentions: The definitive diagnosis relies however on echocardiography. This imaging modality provides accurate morphological and functional assessment of the heart, being able to show the specific features of the transposition of the great arteries. In the four-chamber view, one assesses atrioventricular concordance, but the ventriculoarterial discordance is better observed using other incidences. In the five-chamber, parasternal long-axis or even subcostal view, the vessel arising from the morphologically left ventricle has a posterior course and bifurcates immediately, being identified as the pulmonary trunk (Fig. 1). The morphologically right ventricle is related to a vessel that gives out the coronary, head and neck arteries, thus the aorta. The proximal portions of the two arteries run parallel to each other, rather than in the usual cross pattern, giving it a typical and easily recognisable appearance in the parasternal long-axis and subcostal views (Fig. 2). In the short-axis view, the pulmonary trunk is usually in a central position, with the aorta being placed anteriorly and to the right (Fig. 3).

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