Materiały źródłowe

• #1 Transposition of the Great Arteries (TGA) – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/transposition-of-the-great-arteries-tga

  Transposition of the great arteries (in this case, dextro-transposition) occurs when the aorta arises directly from the right ventricle and the pulmonary artery arises from the left ventricle, resulting in independent, parallel pulmonary and systemic circulations; oxygenated blood cannot reach the body except through openings connecting the right and left sides of the heart (eg, patent foramen ovale, ventricular septal defect [VSD]). […] Systemic and pulmonary circulations are completely separated in transposition of the great arteries. After returning to the right heart, desaturated systemic venous blood is pumped into the systemic circulation without being oxygenated in the lungs; oxygenated blood entering the left heart goes back to the lungs rather than to the rest of the body. This anomaly is not compatible with life unless desaturated and oxygenated blood can mix through openings at one or more levels (eg, atrial, ventricular, or great artery level).

• #2 Transposition of the Great Arteries: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/153400-overview

  Transposition of the great arteries (TGA) is the most common cyanotic congenital heart lesion that presents in neonates (ie, in the first week of life. It is the second most common cyanotic congenital heart disease overall, after tetralogy of Fallot.). The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle. […] The etiology for transposition of the great arteries is unknown and is presumed to be multifactorial. The embryology likely involves abnormal persistence of the subaortic conus with resorption or underdevelopment of the subpulmonary conus (infundibulum). This abnormality aligns the aorta anterior and superior with the right ventricle during development.

• #3 Transposition of the Great Arteries: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/153400-overview

  The pulmonary and systemic circulations function in parallel, rather than in series. Oxygenated pulmonary venous blood returns to the left atrium and left ventricle but is recirculated to the pulmonary vascular bed via the abnormal pulmonary arterial connection to the left ventricle. Deoxygenated systemic venous blood returns to the right atrium and right ventricle where it is subsequently pumped to the systemic circulation, effectively bypassing the lungs. This parallel circulatory arrangement results in a deficient oxygen supply to the tissues and an excessive right and left ventricular workload. It is incompatible with prolonged survival unless mixing of oxygenated and deoxygenated blood occurs at some anatomic level.

• #4 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5–7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly’s development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations.

• #5 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK538434/

  Transposition of the great arteries (TGA) is a congenital cardiac defect characterized by an embryological discordance of the aorta and pulmonary trunk. In dextro-transposition of the great arteries (d-TGA), the aorta arises from the right ventricle and the pulmonary trunk from the left ventricle, resulting in 2 parallel circuits that are incompatible with life unless there is a shunt, such as a patent ductus arteriosus (PDA) or septal defect, to allow for blood mixing. […] The exact cause of d-TGA remains unclear, but it is believed to result from multiple complex factors. Understanding the pathogenesis is challenging, particularly due to difficulties replicating the condition in animal models. Traditionally, 2 main theories have been proposed to explain the embryological development of d-TGA. The „extracardiac theory” suggests that d-TGA arises from abnormal spiralization of the aortopulmonary septum during heart development, where the conotruncal septum follows a straight path instead of its normal spiral trajectory.

• #6 Transposition of the great arteries | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-3-27

  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 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. […] In the hearts with concordant atrioventricular and discordant ventriculoarterial connections, the systemic and pulmonary circulations run in parallel, rather than in series. As such oxygenated blood flows through a closed circuit that involves the lungs and the left cardiac chambers. On the other hand, systemic blood flow is supplied by another closed circuit that begins and ends in the right cardiac chambers. In this setting, survival is only possible, if there is adequate mixing between the two circulations, be it between the septums or through the arterial duct.

• #7 Transposition of the great vessels – Wikipedia

  https://en.wikipedia.org/wiki/Transposition_of_the_great_vessels

  Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenital heart diseases involving only the primary arteries (pulmonary artery and aorta) belong to a sub-group called transposition of the great arteries (TGA), which is considered the most common congenital heart lesion that presents in neonates. […] Dextro-Transposition of the great arteries (also known as dextro-TGA) is a cyanotic heart defect in which the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. This switch causes deoxygenated blood from the right heart to be pumped immediately through the aorta and circulated throughout the body and the heart itself, bypassing the lungs altogether.

• #8 Transposition of the Great Arteries – Presentation – TeachMePaediatrics

  https://teachmepaediatrics.com/cardiology/congenital-heart-defects/transposition-great-arteries/

  In dextro-TGA the pulmonary and systemic circulation run in parallel, causing oxygenated blood to recirculate only in the pulmonary circulation and deoxygenated systemic blood to bypass the lungs. This results in cyanosis unless there is mixing of oxygenated blood and deoxygenated blood. […] In levo-TGA [also called as CC-TGA] the ventricles have switched places as opposed to the arteries and thus this is acyanotic as deoxygenated blood can return from the systemic circulation and enter the pulmonary circulation to be oxygenated before entering the systemic circulation again. Nevertheless, the right ventricle and tricuspid valve is not accustomed to the higher pressures of the left side of the heart and thus, there is hypertrophy over time, which can result in tricuspid regurgitation and heart failure.

• #9 Transposition of the great vessels – Wikipedia

  https://en.wikipedia.org/wiki/Transposition_of_the_great_vessels

  Levo-Transposition of the great arteries (also known as Levo-TGA, congenitally corrected TGA, double discordance, or ventricular inversion) is a rare, acyanotic heart defect in which the primary arteries are transposed, with the aorta anterior and to the left of the pulmonary artery, and the morphological left and right ventricles with their corresponding atrioventricular valves are also transposed. […] In many cases, TGV is accompanied by other heart defects, the most common type being intracardiac shunts such as atrial septal defect including patent foramen ovale, ventricular septal defect, and patent ductus arteriosus. […] All infants with TGA will need surgery to correct the defect. Life expectancy is only a few months if corrective surgery is not performed. […] For newborns with transposition, prostaglandins can be given to keep the ductus arteriosus open which allows for the mixing of the otherwise isolated pulmonary and systemic circuits. Thus, oxygenated blood that recirculates back to the lungs can mix with blood that circulates throughout the body and can keep the body oxygenated until surgery can be performed. […] The arterial switch operation is a surgery where the pulmonary artery and the aorta are moved to their normal positions. This is the most common surgery done to correct dextro-TGA, and is considered the definitive treatment.

• #10 Transposition of the great arteries | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/transposition-great-arteries/

  Transposition of the great arteries is a reversal of the aorta and pulmonary artery, the two major blood vessels leaving from the heart. There are two types of transpositions, a D-type and an L-type. […] In D-type transposition of the great arteries (D-TGA), the two main arteries (aorta and pulmonary artery) are connected to the wrong chambers of the heart. When the vessels are switched, the aorta is on the wrong side of the heart and gets oxygen-poor blood from the body instead of oxygen-rich blood fresh from the lungs. The oxygen-poor blood continues to recirculate to the body. At the same time, the pulmonary artery keeps recirculating oxygen-rich blood to the lungs instead of sending it out to the body. This heart defect will require surgery. […] In L-type transposition of the great arteries (L-TGA), not only are the two great arteries reversed, but the two ventricles also are in abnormal positions. Oxygen-poor blood travels from the right atrium to the left ventricle, then out to the pulmonary artery and lungs. Oxygen-rich blood travels from the left atrium to the right ventricle then out the aorta to the body. Overall, blood flow is normal and the condition does not require an operation, however there are many heart defects associated with L-TGA that may require intervention. In addition, the conduction system of the heart can be abnormal and the right ventricle has to do the work of the left ventricle, which makes it apt to fail over time.

• #11 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK538434/

  Transposition of the great arteries (TGA) is a congenital cardiac defect characterized by an embryological discordance of the aorta and pulmonary trunk. In dextro-transposition of the great arteries (d-TGA), the aorta arises from the right ventricle and the pulmonary trunk from the left ventricle, resulting in 2 parallel circuits that are incompatible with life unless there is a shunt, such as a patent ductus arteriosus (PDA) or septal defect, to allow for blood mixing. […] The exact cause of d-TGA remains unclear, but it is believed to result from multiple complex factors. Understanding the pathogenesis is challenging, particularly due to difficulties replicating the condition in animal models. Traditionally, 2 main theories have been proposed to explain the embryological development of d-TGA. The „extracardiac theory” suggests that d-TGA arises from abnormal spiralization of the aortopulmonary septum during heart development, where the conotruncal septum follows a straight path instead of its normal spiral trajectory.

• #12 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  The precise etiology of D-TGA has not been fully determined and its pathogenesis is multifactorial and controversial, especially because D-TGA is difficult to reproduce with animal models. The embryological mechanism of D-TGA has traditionally been explained by two main theories. The “extracardiac theory” is based on abnormal spiralization of the aorto-pulmonary septum during cardiac development. D-TGA occurs when the conotruncal septum fails to follow its spiral course and forms in a linear orientation instead. The “infundibular theory” suggests that D-TGA is caused by abnormal resorption or the underdevelopment of the subpulmonary conus, with a persistence of the subaortic conus which results in the aorta being placed above the anterior right ventricle. […] Many factors, both genetic and environmental, influence the formation of D-TGA. Several genes associated with the pathogenesis of D-TGA, located on different chromosomes, have been identified to date. D-TGA is usually considered to have a low risk of familial recurrence; however, multiple mutations in laterality genes, confirming a pathogenetic relation between TGA and heterotaxy, have been found in some families. Other studies have postulated the influence of associated risk factors, such as gestational diabetes mellitus, maternal exposure to pesticides, maternal use of drugs during the first trimester, a respiratory tract infection during pregnancy, or exposure to ionizing radiation, as well as in the case of in vitro fertilization.

• #13 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  The precise etiology of D-TGA has not been fully determined and its pathogenesis is multifactorial and controversial, especially because D-TGA is difficult to reproduce with animal models. The embryological mechanism of D-TGA has traditionally been explained by two main theories. The “extracardiac theory” is based on abnormal spiralization of the aorto-pulmonary septum during cardiac development. D-TGA occurs when the conotruncal septum fails to follow its spiral course and forms in a linear orientation instead. The “infundibular theory” suggests that D-TGA is caused by abnormal resorption or the underdevelopment of the subpulmonary conus, with a persistence of the subaortic conus which results in the aorta being placed above the anterior right ventricle. […] Many factors, both genetic and environmental, influence the formation of D-TGA. Several genes associated with the pathogenesis of D-TGA, located on different chromosomes, have been identified to date. D-TGA is usually considered to have a low risk of familial recurrence; however, multiple mutations in laterality genes, confirming a pathogenetic relation between TGA and heterotaxy, have been found in some families. Other studies have postulated the influence of associated risk factors, such as gestational diabetes mellitus, maternal exposure to pesticides, maternal use of drugs during the first trimester, a respiratory tract infection during pregnancy, or exposure to ionizing radiation, as well as in the case of in vitro fertilization.

• #14 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK538434/

  TGA results from the failure of the aorticopulmonary septum to spiral, which can arise from defects in the maturation of the right superior and left inferior truncus swellings, improper fusion of the conus swellings with the aorticopulmonary septum, or abnormalities in neural crest development or migration. These pathological changes in d-TGA disrupt normal cardiac physiology. Because the 2 circuits run parallel, deoxygenated blood continuously recirculates through the systemic circulation, while oxygenated blood is confined to the pulmonary circuit. […] Understanding the normal formation of the conotruncal septum is essential for grasping the development of d-TGA. In the fifth week of gestation, pairs of opposing ridges emerge in the truncus arteriosus, specifically the right superior and left inferior truncus swellings. The right superior truncus swelling grows distally to the left, while the left inferior truncus swelling grows distally to the right. This interaction results in the twisting of the swellings around one another, setting the stage for the formation of an anatomically normal spiral septum.

• #15 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  The precise etiology of D-TGA has not been fully determined and its pathogenesis is multifactorial and controversial, especially because D-TGA is difficult to reproduce with animal models. The embryological mechanism of D-TGA has traditionally been explained by two main theories. The “extracardiac theory” is based on abnormal spiralization of the aorto-pulmonary septum during cardiac development. D-TGA occurs when the conotruncal septum fails to follow its spiral course and forms in a linear orientation instead. The “infundibular theory” suggests that D-TGA is caused by abnormal resorption or the underdevelopment of the subpulmonary conus, with a persistence of the subaortic conus which results in the aorta being placed above the anterior right ventricle. […] Many factors, both genetic and environmental, influence the formation of D-TGA. Several genes associated with the pathogenesis of D-TGA, located on different chromosomes, have been identified to date. D-TGA is usually considered to have a low risk of familial recurrence; however, multiple mutations in laterality genes, confirming a pathogenetic relation between TGA and heterotaxy, have been found in some families. Other studies have postulated the influence of associated risk factors, such as gestational diabetes mellitus, maternal exposure to pesticides, maternal use of drugs during the first trimester, a respiratory tract infection during pregnancy, or exposure to ionizing radiation, as well as in the case of in vitro fertilization.

• #16 Transposition of the Great Arteries: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/153400-overview

  Transposition of the great arteries (TGA) is the most common cyanotic congenital heart lesion that presents in neonates (ie, in the first week of life. It is the second most common cyanotic congenital heart disease overall, after tetralogy of Fallot.). The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle. […] The etiology for transposition of the great arteries is unknown and is presumed to be multifactorial. The embryology likely involves abnormal persistence of the subaortic conus with resorption or underdevelopment of the subpulmonary conus (infundibulum). This abnormality aligns the aorta anterior and superior with the right ventricle during development.

• #17 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK538434/

  TGA results from the failure of the aorticopulmonary septum to spiral, which can arise from defects in the maturation of the right superior and left inferior truncus swellings, improper fusion of the conus swellings with the aorticopulmonary septum, or abnormalities in neural crest development or migration. These pathological changes in d-TGA disrupt normal cardiac physiology. Because the 2 circuits run parallel, deoxygenated blood continuously recirculates through the systemic circulation, while oxygenated blood is confined to the pulmonary circuit. […] Understanding the normal formation of the conotruncal septum is essential for grasping the development of d-TGA. In the fifth week of gestation, pairs of opposing ridges emerge in the truncus arteriosus, specifically the right superior and left inferior truncus swellings. The right superior truncus swelling grows distally to the left, while the left inferior truncus swelling grows distally to the right. This interaction results in the twisting of the swellings around one another, setting the stage for the formation of an anatomically normal spiral septum.

• #18 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK538434/

  TGA results from the failure of the aorticopulmonary septum to spiral, which can arise from defects in the maturation of the right superior and left inferior truncus swellings, improper fusion of the conus swellings with the aorticopulmonary septum, or abnormalities in neural crest development or migration. These pathological changes in d-TGA disrupt normal cardiac physiology. Because the 2 circuits run parallel, deoxygenated blood continuously recirculates through the systemic circulation, while oxygenated blood is confined to the pulmonary circuit. […] Understanding the normal formation of the conotruncal septum is essential for grasping the development of d-TGA. In the fifth week of gestation, pairs of opposing ridges emerge in the truncus arteriosus, specifically the right superior and left inferior truncus swellings. The right superior truncus swelling grows distally to the left, while the left inferior truncus swelling grows distally to the right. This interaction results in the twisting of the swellings around one another, setting the stage for the formation of an anatomically normal spiral septum.

• #19 Transposition of the Great Arteries – Presentation – TeachMePaediatrics

  https://teachmepaediatrics.com/cardiology/congenital-heart-defects/transposition-great-arteries/

  The exact embryological mechanisms that result in TGA is unknown; however, there are currently two theories that try to explain the phenomenon. […] Goor and Edwards suggest that the aorta does not rotate normally towards the left ventricle embryologically and imply that TGA is an extreme form of dextroposition of the aorta. […] De la Cruz theorises that there is no rotation of the aorto-pulmonary septum at the infundibular level. This causes the fourth aortic arch, which will later become the aorta, to interact with the anterior conus on the right ventricle.

• #20 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  The precise etiology of D-TGA has not been fully determined and its pathogenesis is multifactorial and controversial, especially because D-TGA is difficult to reproduce with animal models. The embryological mechanism of D-TGA has traditionally been explained by two main theories. The “extracardiac theory” is based on abnormal spiralization of the aorto-pulmonary septum during cardiac development. D-TGA occurs when the conotruncal septum fails to follow its spiral course and forms in a linear orientation instead. The “infundibular theory” suggests that D-TGA is caused by abnormal resorption or the underdevelopment of the subpulmonary conus, with a persistence of the subaortic conus which results in the aorta being placed above the anterior right ventricle. […] Many factors, both genetic and environmental, influence the formation of D-TGA. Several genes associated with the pathogenesis of D-TGA, located on different chromosomes, have been identified to date. D-TGA is usually considered to have a low risk of familial recurrence; however, multiple mutations in laterality genes, confirming a pathogenetic relation between TGA and heterotaxy, have been found in some families. Other studies have postulated the influence of associated risk factors, such as gestational diabetes mellitus, maternal exposure to pesticides, maternal use of drugs during the first trimester, a respiratory tract infection during pregnancy, or exposure to ionizing radiation, as well as in the case of in vitro fertilization.

• #21 Transposition of the great arteries | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-3-27

  Significant advances in the understanding of the underlying genetic mechanisms have been achieved over the last decade. Several mutations have been implicated as the cause of discordant ventriculoarterial connections. The genes involved so far are the growth differentiation factor-1 gene, the thyroid hormone receptor-associated protein-2 gene, and the gene encoding the cryptic protein. They are localised in different chromosomes and their mutations only explain a small minority of the clinical cases.

• #22 Analysis of mutations in 7 candidate genes for dextro- Transposition of the great arteries in Chinese population – Lei – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/2345/html

  Transposition of great arteries (TGA) represents the most frequent cyanotic heart defect diagnosed in the neonatal period. Several genes had been identified to be associated with the pathogenesis of dextro-transposition of the great arteries (d-TGA). […] The exact etiology of TGA is still unknown. […] The genes being thought to be associated with the pathogenesis of TGA so far are the thyroid hormone receptor-associated protein-2 gene (MED13L), nucleocytoplasmic shuttling protein gene (ZIC3), the forkhead activin signal transducer 1 (FOXH1), cryptic family 1 (CFC1), growth differentiation factor 1 (GDF1) and TGF-beta gene (NODAL), the latter five genes of which affect the LR-axis development. […] For the pathogenesis of d-TGA, the involvement of 7 genes has already been discussed. […] The low mutation frequency of MED13L, ZIC3, CFC1, NODAL, FOXH1, GDF1 and NKX2-5 in d-TGA patients underlines the heterogeneity of this disease, in which the candidate gene screening had limited success.

• #23 Transposition of the great arteries | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-3-27

  Significant advances in the understanding of the underlying genetic mechanisms have been achieved over the last decade. Several mutations have been implicated as the cause of discordant ventriculoarterial connections. The genes involved so far are the growth differentiation factor-1 gene, the thyroid hormone receptor-associated protein-2 gene, and the gene encoding the cryptic protein. They are localised in different chromosomes and their mutations only explain a small minority of the clinical cases.

• #24 Analysis of mutations in 7 candidate genes for dextro- Transposition of the great arteries in Chinese population – Lei – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/2345/html

  This study highlights the fact that the underlying genetic etiology of d-TGA can be complex. Thirteen known mutations in MED13L, ZIC3, CFC1, NODAL, FOXH1, GDF1 and NKX2-5 may not be a common cause of d-TGA in Chinese population due to racial variation. […] These reported 13 mutations may not be a common cause of d-TGA in Chinese population due to racial variation and genetic heterogeneity of TGA. New approaches including the whole exome sequencing technology are required to effectively identify genetic variants in TGA patients in China.

• #25 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  The precise etiology of D-TGA has not been fully determined and its pathogenesis is multifactorial and controversial, especially because D-TGA is difficult to reproduce with animal models. The embryological mechanism of D-TGA has traditionally been explained by two main theories. The “extracardiac theory” is based on abnormal spiralization of the aorto-pulmonary septum during cardiac development. D-TGA occurs when the conotruncal septum fails to follow its spiral course and forms in a linear orientation instead. The “infundibular theory” suggests that D-TGA is caused by abnormal resorption or the underdevelopment of the subpulmonary conus, with a persistence of the subaortic conus which results in the aorta being placed above the anterior right ventricle. […] Many factors, both genetic and environmental, influence the formation of D-TGA. Several genes associated with the pathogenesis of D-TGA, located on different chromosomes, have been identified to date. D-TGA is usually considered to have a low risk of familial recurrence; however, multiple mutations in laterality genes, confirming a pathogenetic relation between TGA and heterotaxy, have been found in some families. Other studies have postulated the influence of associated risk factors, such as gestational diabetes mellitus, maternal exposure to pesticides, maternal use of drugs during the first trimester, a respiratory tract infection during pregnancy, or exposure to ionizing radiation, as well as in the case of in vitro fertilization.

• #26 Transposition of the Great Arteries (TGA) | Embryo Project Encyclopedia

  https://embryo.asu.edu/pages/transposition-great-arteries-tga

  Exposure to retinoic acid is one of the most well understood risk factors in the development of TGA. […] Another possibility for increased risk of TGA is maternal influence such as diabetes. […] Advanced maternal age is another maternal risk factor that puts the fetus at risk for developmental issues such as TGA.

• #27 Transposition of the great vessels pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Transposition_of_the_great_vessels_pathophysiology

  Right atrium (RA) is connected to a morphologic right ventricle (RV). The morphologic left atrium (LA) is connected to the morphologic left ventricle (LV). This is called atrio-ventricular concordance. In a normal heart, the great arteries (aorta and pulmonary arteries) are concordant with the morphologic LV and RV. This is termed ventriculo-arterial concordance. In addition, the aorta and pulmonary trunk ascend in a spiral relationship. In the TGA the aorta arises from the morphologic right ventricle via a subaortic infundibulum and the pulmonary artery arises from the morphologic left ventricle, without a subpulmonary infundibulum. These ventriculoarterial connection is known as ventriculoarterial discordance. The abnormal origin of the great arteries results in an altered spiral relationship resulting in parallel circulation.

• #28 Transposition of the Great Arteries: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/153400-overview

  The pulmonary and systemic circulations function in parallel, rather than in series. Oxygenated pulmonary venous blood returns to the left atrium and left ventricle but is recirculated to the pulmonary vascular bed via the abnormal pulmonary arterial connection to the left ventricle. Deoxygenated systemic venous blood returns to the right atrium and right ventricle where it is subsequently pumped to the systemic circulation, effectively bypassing the lungs. This parallel circulatory arrangement results in a deficient oxygen supply to the tissues and an excessive right and left ventricular workload. It is incompatible with prolonged survival unless mixing of oxygenated and deoxygenated blood occurs at some anatomic level.

• #29 Transposition of the Great Arteries (TGA) – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/transposition-of-the-great-arteries-tga

  Transposition of the great arteries (in this case, dextro-transposition) occurs when the aorta arises directly from the right ventricle and the pulmonary artery arises from the left ventricle, resulting in independent, parallel pulmonary and systemic circulations; oxygenated blood cannot reach the body except through openings connecting the right and left sides of the heart (eg, patent foramen ovale, ventricular septal defect [VSD]). […] Systemic and pulmonary circulations are completely separated in transposition of the great arteries. After returning to the right heart, desaturated systemic venous blood is pumped into the systemic circulation without being oxygenated in the lungs; oxygenated blood entering the left heart goes back to the lungs rather than to the rest of the body. This anomaly is not compatible with life unless desaturated and oxygenated blood can mix through openings at one or more levels (eg, atrial, ventricular, or great artery level).

• #30 Transposition of the Great Arteries | Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/transposition

  The „great arteries” in this defect refer to the aorta and the pulmonary artery. These are the two major arteries carrying blood away from the heart. […] In cases of transposition of the great arteries, these vessels begin from the wrong ventricle. They are „transposed” from their normal position. The aorta starts from the right ventricle and the pulmonary artery from the left ventricle. […] Transposition creates a situation where the systemic (to the body) and pulmonary (to the lungs) circulations are working side by side and not together. This means the oxygen-poor („blue”) blood returning from the body and coursing through the right atrium and right ventricle is pumped out to the aorta and to the body. The oxygen-rich („red”) blood returning from the lungs and going through the left atrium and ventricle is sent back to the lungs by the pulmonary artery.

• #31 Transposition of the great arteries – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/transposition-of-the-great-arteries/symptoms-causes/syc-20350589

  The switched arteries cause changes in blood flow. Oxygen-poor blood now flows through the right side of the heart. It goes back to the body without passing through the lungs. Oxygen-rich blood now flows through the left side of the heart. It goes directly back into the lungs without being pumped to the rest of the body. […] In this less common type, also called levo-transposition of the great arteries (L-TGA), the two lower heart chambers are reversed. […] The blood usually still flows correctly through the heart and body. But the heart can have long-term trouble pumping blood. People with L-TGA may also have problems with the tricuspid heart valve.

• #32 Transposition of the great vessels pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Transposition_of_the_great_vessels_pathophysiology

  The fetus circulation in-utero is different compared to the extra-uterine circulation. The fetus tolerates a D-TGA well in-utero due to this difference in circulation. The high resistance in the pulmonary circulation compared to the placenta, allows the blood to flow to the descending aorta rather than to the lung. Due to this the fetus gets blood with a higher oxygen tension. […] In Uncorrected D-TGA the systemic and pulmonary circulations are parallel circuits which means that the deoxygenated systemic venous blood comes to the right ventricle and inplace of going to the lungs, drains back to the systemic circulation via the aorta. Similarly, oxygenated pulmonary venous blood is recirculated to the lungs via the pulmonary artery. This parallel circulation is incompatible to life. For a child with dextro-TGA to survive, a communication between the two parallel circuits is necessary. Various connections that allow mixing in these patients are: patent foramen ovale, ventricular septal defect, atrial septal defect, patent ductus arteriosus or the bronchopulmonary collateral circulation.

• #33 Transposition of the great arteries | PPT

  https://www.slideshare.net/slideshow/transposition-of-the-great-arteries-231230462/231230462

  Infants survive only if a shunt between the two circulations exist to mix oxygenated blood into the systemic circulation patent ductus arteriosus (PDA) ventricular septal defect (VSD) atrial septal defect (ASD) patent foramen ovale (PFO). […] Oxygenated blood cannot reach the body except through openings connecting the right and left sides (eg, patent foramen ovale, ventricular septal defect [VSD]). Symptoms are primarily severe neonatal cyanosis and occasionally heart failure, if there is an associated VSD. […] Severe cyanosis occurs within hours of birth, followed rapidly by metabolic acidosis; there are no murmurs unless other anomalies are present. […] Diagnosis is by echocardiography. […] Relieve cyanosis by giving prostaglandin E1 infusion to keep the ductus arteriosus open and sometimes by using a balloon catheter to enlarge the foramen ovale. […] Do definitive surgical repair during the first week of life.

• #34 Transposition of the great arteries | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/transposition-of-the-great-arteries?lang=us

  Transposition of the great arteries (TGA), also known as transposition of the great vessels (TGV), is the most common cyanotic congenital cardiac anomaly presenting during the newborn period, with cyanosis in the first 24 hours of life. […] It occurs as a result of ventriculoarterial discordance, with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. […] Transposition of the great arteries occurs as a result of ventriculoarterial discordance, with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. […] An isolated TGA is incompatible with life at birth without one of the following additional anomalies (which are a common occurrence): atrial septal defect (ASD): uncommon, ventricular septal defect (VSD): ~35%, patent ductus arteriosus (PDA): unstable due to closure following birth, patent foramen ovale (PFO): unstable. […] Unstable associations account for 60-65% of occurrences.

• #35 What is Transposition of the Great Arteries?

  https://www.tinytickers.org/support-info/what-is-chd/transposition-great-arteries-tga/

  Transposition of the great arteries (TGA) is a condition where the two main blood vessels (the ‘great arteries’) leaving the heart, are swapped over. The pulmonary artery (which takes blood to the lungs to pick up oxygen) and the aorta (which takes oxygen rich blood from the heart, to the body). […] In TGA, the opposite occurs. The pulmonary artery arises from the left ventricle and the aorta from the right ventricle. […] This means that blood flows to the lungs and picks up oxygen but is then pumped back to the lungs instead of travelling around the body. Blood flowing around the body is unable to reach the lungs to pick up oxygen and continues circulating. […] Unless there is some place in the circulation where the oxygen-rich and oxygen-poor blood can mix, all of the organs of the body will be deprived of necessary oxygen.

• #36 Transposition of the great vessels pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Transposition_of_the_great_vessels_pathophysiology

  The fetus circulation in-utero is different compared to the extra-uterine circulation. The fetus tolerates a D-TGA well in-utero due to this difference in circulation. The high resistance in the pulmonary circulation compared to the placenta, allows the blood to flow to the descending aorta rather than to the lung. Due to this the fetus gets blood with a higher oxygen tension. […] In Uncorrected D-TGA the systemic and pulmonary circulations are parallel circuits which means that the deoxygenated systemic venous blood comes to the right ventricle and inplace of going to the lungs, drains back to the systemic circulation via the aorta. Similarly, oxygenated pulmonary venous blood is recirculated to the lungs via the pulmonary artery. This parallel circulation is incompatible to life. For a child with dextro-TGA to survive, a communication between the two parallel circuits is necessary. Various connections that allow mixing in these patients are: patent foramen ovale, ventricular septal defect, atrial septal defect, patent ductus arteriosus or the bronchopulmonary collateral circulation.

• #37 Transposition of the Great Arteries (TGA) – RCEMLearning

  https://www.rcemlearning.co.uk/modules/congenital-heart-disease-in-the-emergency-department/lessons/righttoleft-shunts/topic/transposition-of-the-great-arteries-tga/

  Second most common cause of cyanotic CHD diagnosed by 1 year of age. […] The aorta lies anteriorly and arises from the right ventricle; the pulmonary artery is relatively posterior and connected to the left ventricle (ventriculoarterial discordance). Deoxygenated blood is therefore returned to the body and oxygenated blood is returned to the lungs. The pulmonary and systemic circuits are arranged in parallel. Unless there is mixing of blood between them, this condition is incompatible with life. […] The presentation for infants with TGA depends on the degree of blood mixing. […] Initial survival depends on the presence of a shunt, allowing mixing between the systemic and pulmonary circulations. […] These infants present with cyanosis at birth because of right-to-left shunting, and the cyanosis becomes more severe when the ductus arteriosus closes.

• #38 Managing transposition of the great arteries in the womb – Children’s National

  https://innovationdistrict.childrensnational.org/managing-transposition-of-the-great-arteries-in-the-womb/

  Case in point: Transposition of the great arteries, a congenital defect characterized by reversal of the hearts two main arteriesthe aorta, which distributes oxygenated blood throughout the body, and the pulmonary artery, which carries deoxygenated blood from the heart to the lungs. The single abnormality means that the oxygenated red blood flows back to the lungs while deoxygenated blue blood flows out to the body. […] If the fetal connections between the two sides of the heart no longer remain, the brain and other organs in infants with this defect are severely deprived of oxygen. The condition may be fatal if something is not done immediately to reopen the fetal connections to stabilize the circulation before surgery can be done. […] Dr. Donofrio explains there are two pathways that can allow the blood to get to where it needs to go such that the circulation is stabilized and the damage mitigated. One is the fetal blood vessel known as the ductus arteriosus that typically stays open for a day or two after birth. The second is an opening between the hearts two upper chambers, known as the foramen ovale, which usually closes upon delivery. By keeping those two pathways open, blood can cross from one side of the heart to the other, buying time in the delivery room so that babies can be stabilized before they receive surgery to permanently move the arteries back to their normal position.

• #39 Transposition of the Great Arteries | TGA Heart | Children’s Wisconsin

  https://childrenswi.org/medical-care/herma-heart/conditions/transposition-of-the-great-arteries

  Two separate circuits are formed – one that circulates oxygen-poor (blue) blood from the lungs back to the lungs, and another that recirculates oxygen-rich (red) blood from the body back to the body. […] Other heart defects are often associated with TGA, and they actually may be necessary in order for an infant with transposition of the great arteries to live. […] The problem occurs in the middle of these weeks, allowing the aorta and pulmonary artery to be attached to the incorrect chamber. […] Without an additional heart defect that allows mixing of oxygen-poor (blue) and oxygen-rich (red) blood, such as an atrial or ventricular septal defect, infants with TGA will have oxygen-poor (blue) blood circulating through the body – a situation that is fatal. […] The left ventricle, which in TGA is connected to the pulmonary artery, is the stronger of the two ventricles since it normally has to generate a lot of force to pump blood to the body. The right ventricle, connected to the aorta in TGA, is the weaker of the two ventricles.

• #40 Dextro-transposition of the great arteries pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Dextro-transposition_of_the_great_arteries_pathophysiology

  The course of TGA is determined by the degree of hypoxia, and the ability of each ventricle to sustain an increased work load in the presence of reduced coronary arterial oxygenation. […] The pulmonic flow is increased in those cases with transposition and large VSD or large PDA without obstruction to left ventricular outflow.

• #41 25: Transposition of the Great Arteries | Veterian Key

  https://veteriankey.com/25-transposition-of-the-great-arteries/

  In the weeks after birth, pulmonary vascular resistance decreases and, in the absence of a large VSD or outflow tract obstruction, the afterload on the LV decreases. […] Thus, anatomic repair is performed in patients with TGA and intact ventricular septum or a small VSD within the first days to weeks of life in order to avoid LV remodeling.

• #42 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5–7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly’s development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations.

• #43 Transposition of the Great Arteries Diagnosis & Treatments | Mount Sinai – New York

  https://www.mountsinai.org/locations/childrens-heart/conditions/transposition-great-arteries

  TGA is a condition that affects the two large arteries of your baby’s heart—the main pulmonary artery and the aorta. They carry blood from the heart to the lungs and the rest of your baby’s body. […] In TGA, the pulmonary artery is connected to the left ventricle instead of the right ventricle. And the aorta comes from the right ventricle instead of the left. This switch, or transposition, changes how blood flows. With TGA, oxygen-poor blood pumps back into the body instead of going to the lungs. And the already oxygen-rich blood is misdirected to the lungs, where it just came from. This means that the body does not receive the oxygen and nutrients it needs. […] TGA can often have other heart defects associated with it. These commonly include: Aortic coarctation—the aortic arch may be narrowed or obstructed, Pulmonary valve abnormality—affecting the connection between the left ventricle and pulmonary artery, Ventricular septal defect (VSD)—a hole between the bottom chambers of the heart.

• #44 Transposition of the great vessels – Wikipedia

  https://en.wikipedia.org/wiki/Transposition_of_the_great_vessels

  Levo-Transposition of the great arteries (also known as Levo-TGA, congenitally corrected TGA, double discordance, or ventricular inversion) is a rare, acyanotic heart defect in which the primary arteries are transposed, with the aorta anterior and to the left of the pulmonary artery, and the morphological left and right ventricles with their corresponding atrioventricular valves are also transposed. […] In many cases, TGV is accompanied by other heart defects, the most common type being intracardiac shunts such as atrial septal defect including patent foramen ovale, ventricular septal defect, and patent ductus arteriosus. […] All infants with TGA will need surgery to correct the defect. Life expectancy is only a few months if corrective surgery is not performed. […] For newborns with transposition, prostaglandins can be given to keep the ductus arteriosus open which allows for the mixing of the otherwise isolated pulmonary and systemic circuits. Thus, oxygenated blood that recirculates back to the lungs can mix with blood that circulates throughout the body and can keep the body oxygenated until surgery can be performed. […] The arterial switch operation is a surgery where the pulmonary artery and the aorta are moved to their normal positions. This is the most common surgery done to correct dextro-TGA, and is considered the definitive treatment.

• #45 Transposition of the great arteries | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/transposition-of-the-great-arteries?lang=us

  Transposition of the great arteries (TGA), also known as transposition of the great vessels (TGV), is the most common cyanotic congenital cardiac anomaly presenting during the newborn period, with cyanosis in the first 24 hours of life. […] It occurs as a result of ventriculoarterial discordance, with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. […] Transposition of the great arteries occurs as a result of ventriculoarterial discordance, with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. […] An isolated TGA is incompatible with life at birth without one of the following additional anomalies (which are a common occurrence): atrial septal defect (ASD): uncommon, ventricular septal defect (VSD): ~35%, patent ductus arteriosus (PDA): unstable due to closure following birth, patent foramen ovale (PFO): unstable. […] Unstable associations account for 60-65% of occurrences.

• #46 Transposition of the great arteries | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/transposition-of-the-great-arteries?lang=us

  Transposition of the great arteries (TGA), also known as transposition of the great vessels (TGV), is the most common cyanotic congenital cardiac anomaly presenting during the newborn period, with cyanosis in the first 24 hours of life. […] It occurs as a result of ventriculoarterial discordance, with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. […] Transposition of the great arteries occurs as a result of ventriculoarterial discordance, with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. […] An isolated TGA is incompatible with life at birth without one of the following additional anomalies (which are a common occurrence): atrial septal defect (ASD): uncommon, ventricular septal defect (VSD): ~35%, patent ductus arteriosus (PDA): unstable due to closure following birth, patent foramen ovale (PFO): unstable. […] Unstable associations account for 60-65% of occurrences.

• #47 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5–7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly’s development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations.

• #48 Transposition of the Great Arteries Diagnosis & Treatments | Mount Sinai – New York

  https://www.mountsinai.org/locations/childrens-heart/conditions/transposition-great-arteries

  TGA is a condition that affects the two large arteries of your baby’s heart—the main pulmonary artery and the aorta. They carry blood from the heart to the lungs and the rest of your baby’s body. […] In TGA, the pulmonary artery is connected to the left ventricle instead of the right ventricle. And the aorta comes from the right ventricle instead of the left. This switch, or transposition, changes how blood flows. With TGA, oxygen-poor blood pumps back into the body instead of going to the lungs. And the already oxygen-rich blood is misdirected to the lungs, where it just came from. This means that the body does not receive the oxygen and nutrients it needs. […] TGA can often have other heart defects associated with it. These commonly include: Aortic coarctation—the aortic arch may be narrowed or obstructed, Pulmonary valve abnormality—affecting the connection between the left ventricle and pulmonary artery, Ventricular septal defect (VSD)—a hole between the bottom chambers of the heart.

• #49 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5–7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly’s development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations.

• #50 Transposition of the Great Arteries (DTGA) | Pediatric Echocardiography

  https://pedecho.org/library/chd/dtga

  Transposition of the great arteries (D-TGA) is a conotruncal abnormality characterized by discordant ventricular-arterial connections. In D-TGA, the aorta arises from the right ventricle and pulmonary artery arises from the left ventricle. The aorta is anterior and rightward. The pulmonary and aortic circulations are in parallel instead of in series. […] D-TGA occurs in ~31.5 in 100,000 live births. It is the 10th most common form of congenital heart disease, and the second most common cyanotic heart disease following Tetralogy of Fallot. […] Diagnosis can be missed if the fetal ultrasound does not sweep to the outflow tracts, highlighting the importance of this view. […] In some cases, neonates can present with reverse differential cyanosis (defined as higher saturation in lower extremities compared to upper extremities) from right to left shunting at the PDA in cases of D-TGA with coarctation, D-TGA with pulmonary hypertension or D-TGA with interrupted aortic arch (IAA). […] Abnormal coronary artery branching patterns are common in D-TGA. […] The arterial switch operation (ASO or Jatene operation) with LeCompte maneuver is currently the most frequent procedure for D-TGA.

• #51 Transposition of the Great Arteries – Children’s Hospital of Orange County

  https://choc.org/heart/congenital-heart-defects/transposition-of-the-great-arteries/

  Most of the time this heart defect occurs by chance, with no clear reason for its development. […] Babies with TGA have two separate blood flow circuits — one that circulates oxygen-poor (blue) blood from the body back to the body, and another that recirculates oxygen-rich (red) blood from the lungs back to the lungs. […] Without an additional heart defect that allows mixing of oxygen-poor (blue) and oxygen-rich (red) blood, such as an atrial or ventricular septal defect or a patent ductus arteriosus, infants with TGA will only have oxygen-poor (blue) blood circulating through the body—a situation that is fatal. […] Within the first 1 to 2 weeks of age, transposition of the great arteries is surgically repaired. The procedure that accomplishes this is called an “arterial switch,” which roughly describes the surgical process.

• #52 Transposition of the Great Arteries (TGA) – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=transposition-of-the-great-arteries-tga-90-P01823

  Most of the time, the cause of TGA isn’t known. […] All children with a TGA will need to have surgery to fix it. […] Typically, in the first few weeks of life, your baby will need surgery for TGA. This procedure is called an arterial switch. Your child’s surgeon will connect the aorta and pulmonary artery to their normal ventricles. […] Complications of untreated TGA include: […] Heart valve problems […] Heart muscle problems or problems with the arteries that supply blood to the heart muscle […] Abnormal heart rhythm […] Heart failure […] Lung problems […] Death. […] In TGA, your child’s aorta is connected to the right ventricle, and the pulmonary artery is connected to the left ventricle. This is the reverse of a normal heart. […] All children with a TGA will need to have surgery to fix it. […] Most children who have surgery for this condition will grow and develop normally. Your child will still need to see his or her heart doctor for checkups and may require more surgery in the future.

• #53 Transposition of the great arteries | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/transposition-great-arteries/

  If left untreated, over 50% of infants with transposition of the great arteries will die in the first month of life. About 90% will die in the first year. […] When a baby is first suspected of having D-type transposition of the great arteries (D-TGA), the first focus is to help the child maintain safe oxygen levels as well as safe heart and lung function. […] After a diagnosis is suspected or confirmed, doctors may: Give a steady dose of prostaglandins, a medicine that helps keep the ductus arteriosus open. This allows oxygen-rich blood to mix with some of the oxygen-poor blood. […] Once the diagnosis is confirmed, doctors may: Perform a balloon atrial septostomy. […] Corrective surgery usually is performed during the first week of life. […] Before surgeons developed the arterial switch procedure a generation ago, half the babies born with this defect died within a month. Up to 90% of babies didn’t live to see their first birthday. Now, some of the first babies to have this surgery to correct transposition of the great vessels are young adults and living full, active lives.

• #54 Transposition of the Great Arteries | Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/transposition

  In most cases of transposition, an arterial switch surgery is done. The arterial switch surgery involves cutting off the aorta and pulmonary arteries just above the point where they leave the heart. Part of this surgery is reconnecting them to the proper ventricle. The valve stays attached to the ventricle, so what was once the pulmonary valve is now the aortic valve. The aortic valve becomes the pulmonary valve. […] Since the arterial switch surgery reconstructs the heart to a normal situation, long-term cardiac function should be excellent. […] In a small percentage of children, narrowing (stenosis) may occur at the sites where the aorta and pulmonary artery are reattached. The narrowing may occur months or years following surgery. It may need additional treatment. Options for treating this narrowing include cardiac catheterization with balloon dilation of the narrowed area or another surgery.

• #55 Transposition of the great arteries (TGA) – BHF

  https://www.bhf.org.uk/informationsupport/conditions/transposition-of-the-great-arteries

  Your surgeon makes a man-made tunnel between the bottom two chambers of your heart. This lets blood move from the left ventricle to the aorta. Your surgeon adds a tube (called a conduit) from the right ventricle to the pulmonary artery. This lets blood go around (bypass) the narrowed pulmonary artery. […] As you get older, its common to have problems with your heart. Youre more likely to have issues if your TGA was repaired by an atrial switch or Rastelli procedure. […] The complications can include: narrowing (stenosis) of the pulmonary artery, leaky heart valves (regurgitation), baffle narrowing or leaking, conduit narrowing or leaking, heart rhythm problems (arrhythmias), weakening of your heart muscle and heart failure. […] You may need more surgeries in the future to fix these problems, like a leaking valve or narrowed pulmonary artery.

• #56 Echocardiographic Evaluation of Transposition of the Great Arteries – Congenital Cardiac Anesthesia Society

  https://ccasociety.org/education/echoimage/echocardiographic-evaluation-of-transposition-of-the-great-arteries/

  Transposition of the great arteries (TGA) is the most common cyanotic cardiac lesion in newborns, accounting for 5-10% of all congenital heart disease (CHD). The malformation occurs in about 20-30 per 100,000 live births and is seen more often in male babies. Without surgical correction, TGA is almost universally fatal during the first year of life. However, contemporary outcome studies have shown that survival rates after surgical intervention are excellent and that most patients live into adulthood. […] […] In TGA, the ventriculoarterial connections or alignments are discordant, meaning that the great arteries originate from the morphologically inappropriate supporting ventricles (the aorta from the right ventricle [RV] and the pulmonary artery [PA] from the left ventricle [LV]). Transposition is the most common colloquial term for this defect. The most common variant of transposition ({S,D,D}, 80% of cases) is d-TGA, in which the d refers to the dextro looping pattern of the ventricles. Looping of the heart tube to the right during cardiac development positions the morphologic RV toward the right side relative to the morphologic LV (d-bulboventricular loop). Less frequently, transposition can be seen in the setting of situs inversus and l-looped ventricles ({I,L,L}). […]

• #57 Echocardiographic Evaluation of Transposition of the Great Arteries – Congenital Cardiac Anesthesia Society

  https://ccasociety.org/education/echoimage/echocardiographic-evaluation-of-transposition-of-the-great-arteries/

  The physiology in TGA is characterized by a parallel circulation, where deoxygenated blood recirculates through the body and oxygenated blood through the lungs. Without the presence of an anatomic site of intercirculatory shunting, the pulmonary and systemic circulations are completely separated, a condition incompatible with life. In the neonate, the foramen ovale and ductus arteriosus are communications that, if of adequate size, may allow for intercirculatory mixing. Enlargement of a small or restrictive interatrial communication by balloon atrial septostomy (BAS) may be necessary to improve systemic arterial oxygenation. Prostaglandin E1 therapy may be used to maintain ductal patency and enhance intercirculatory mixing in affected neonates. […] […] The important role of echocardiography in patients with TGA is well established. The recently published guidelines for multimodality imaging have described in detail the significant contributions of echocardiography in the diagnosis, surgical planning, and long-term surveillance of patients with TGA. […] Transthoracic echocardiography (TTE) plays a major role in characterizing the anatomic and hemodynamic abnormalities in the neonate with transposition and, in most cases, provides all the necessary information for medical management and surgical planning. In fact, the need for additional cardiovascular imaging modalities for this lesion is highly unusual in the neonatal period. […]

• #58 Echocardiographic Evaluation of Transposition of the Great Arteries – Congenital Cardiac Anesthesia Society

  https://ccasociety.org/education/echoimage/echocardiographic-evaluation-of-transposition-of-the-great-arteries/

  A complete TTE evaluation in the neonate with TGA typically consists of two-dimensional (2D) imaging, Doppler interrogation (pulsed, continuous, and color modalities), M-mode, and tissue Doppler. The diagnosis is based on the sequential segmental analysis that characterizes the detailed assessment of CHD and requires multiple cross-sectional imaging of cardiac and vascular structures. The hallmark feature of the interrogation is the demonstration of the presence of concordant atrioventricular connections and discordant ventriculoarterial connections. […] Echocardiography allows for the detailed characterization of associated lesions in TGA such as VSD(s), LVOT obstruction, aortic arch obstruction, and tricuspid valve abnormalities. This examination requires complementary imaging obtained by multiple cross-sectional views at various levels. […]

• #59 Transposition of the Great Arteries (DTGA) | Pediatric Echocardiography

  https://pedecho.org/library/chd/dtga

  Transposition of the great arteries (D-TGA) is a conotruncal abnormality characterized by discordant ventricular-arterial connections. In D-TGA, the aorta arises from the right ventricle and pulmonary artery arises from the left ventricle. The aorta is anterior and rightward. The pulmonary and aortic circulations are in parallel instead of in series. […] D-TGA occurs in ~31.5 in 100,000 live births. It is the 10th most common form of congenital heart disease, and the second most common cyanotic heart disease following Tetralogy of Fallot. […] Diagnosis can be missed if the fetal ultrasound does not sweep to the outflow tracts, highlighting the importance of this view. […] In some cases, neonates can present with reverse differential cyanosis (defined as higher saturation in lower extremities compared to upper extremities) from right to left shunting at the PDA in cases of D-TGA with coarctation, D-TGA with pulmonary hypertension or D-TGA with interrupted aortic arch (IAA). […] Abnormal coronary artery branching patterns are common in D-TGA. […] The arterial switch operation (ASO or Jatene operation) with LeCompte maneuver is currently the most frequent procedure for D-TGA.

• #60 Transposition of the Great Arteries (DTGA) | Pediatric Echocardiography

  https://pedecho.org/library/chd/dtga

  Transposition of the great arteries (D-TGA) is a conotruncal abnormality characterized by discordant ventricular-arterial connections. In D-TGA, the aorta arises from the right ventricle and pulmonary artery arises from the left ventricle. The aorta is anterior and rightward. The pulmonary and aortic circulations are in parallel instead of in series. […] D-TGA occurs in ~31.5 in 100,000 live births. It is the 10th most common form of congenital heart disease, and the second most common cyanotic heart disease following Tetralogy of Fallot. […] Diagnosis can be missed if the fetal ultrasound does not sweep to the outflow tracts, highlighting the importance of this view. […] In some cases, neonates can present with reverse differential cyanosis (defined as higher saturation in lower extremities compared to upper extremities) from right to left shunting at the PDA in cases of D-TGA with coarctation, D-TGA with pulmonary hypertension or D-TGA with interrupted aortic arch (IAA). […] Abnormal coronary artery branching patterns are common in D-TGA. […] The arterial switch operation (ASO or Jatene operation) with LeCompte maneuver is currently the most frequent procedure for D-TGA.

• #61 Managing transposition of the great arteries in the womb – Children’s National

  https://innovationdistrict.childrensnational.org/managing-transposition-of-the-great-arteries-in-the-womb/

  Case in point: Transposition of the great arteries, a congenital defect characterized by reversal of the hearts two main arteriesthe aorta, which distributes oxygenated blood throughout the body, and the pulmonary artery, which carries deoxygenated blood from the heart to the lungs. The single abnormality means that the oxygenated red blood flows back to the lungs while deoxygenated blue blood flows out to the body. […] If the fetal connections between the two sides of the heart no longer remain, the brain and other organs in infants with this defect are severely deprived of oxygen. The condition may be fatal if something is not done immediately to reopen the fetal connections to stabilize the circulation before surgery can be done. […] Dr. Donofrio explains there are two pathways that can allow the blood to get to where it needs to go such that the circulation is stabilized and the damage mitigated. One is the fetal blood vessel known as the ductus arteriosus that typically stays open for a day or two after birth. The second is an opening between the hearts two upper chambers, known as the foramen ovale, which usually closes upon delivery. By keeping those two pathways open, blood can cross from one side of the heart to the other, buying time in the delivery room so that babies can be stabilized before they receive surgery to permanently move the arteries back to their normal position.

• #62 Echocardiographic Evaluation of Transposition of the Great Arteries – Congenital Cardiac Anesthesia Society

  https://ccasociety.org/education/echoimage/echocardiographic-evaluation-of-transposition-of-the-great-arteries/

  The physiology in TGA is characterized by a parallel circulation, where deoxygenated blood recirculates through the body and oxygenated blood through the lungs. Without the presence of an anatomic site of intercirculatory shunting, the pulmonary and systemic circulations are completely separated, a condition incompatible with life. In the neonate, the foramen ovale and ductus arteriosus are communications that, if of adequate size, may allow for intercirculatory mixing. Enlargement of a small or restrictive interatrial communication by balloon atrial septostomy (BAS) may be necessary to improve systemic arterial oxygenation. Prostaglandin E1 therapy may be used to maintain ductal patency and enhance intercirculatory mixing in affected neonates. […] […] The important role of echocardiography in patients with TGA is well established. The recently published guidelines for multimodality imaging have described in detail the significant contributions of echocardiography in the diagnosis, surgical planning, and long-term surveillance of patients with TGA. […] Transthoracic echocardiography (TTE) plays a major role in characterizing the anatomic and hemodynamic abnormalities in the neonate with transposition and, in most cases, provides all the necessary information for medical management and surgical planning. In fact, the need for additional cardiovascular imaging modalities for this lesion is highly unusual in the neonatal period. […]

• #63 Transposition of the Great Arteries (TGA) – RCEMLearning

  https://www.rcemlearning.co.uk/modules/congenital-heart-disease-in-the-emergency-department/lessons/righttoleft-shunts/topic/transposition-of-the-great-arteries-tga/

  In the sick cyanosed neonate, the key is to improve mixing. The outcome depends on the degree of blood mixing, the magnitude of tissue hypoxia, and the ability of the right ventricle to maintain the systemic circulation. Without surgery, most patients die within months. […] Maintaining the patency of the ductus arteriosus with a prostaglandin infusion is helpful. However, this systemic-to-pulmonary connection tends to close early and thus intervention is required to create a new shunt such as balloon atrial septostomy within the first few days of life.

• #64 Managing transposition of the great arteries in the womb – Children’s National

  https://innovationdistrict.childrensnational.org/managing-transposition-of-the-great-arteries-in-the-womb/

  The cardiac intervention team immediately created a hole where the foramen ovale should have been by using a balloon to open the tissue that had closed. The care team also administered a prostaglandin infusion, a drug that can keep the ductus arteriosis open. This time, however, the medicine did not work. The baby was stabilized with several cardiac medications and, with little time to spare, the cardiac surgeons operated on the one-day-old baby to switch his great arteries back to the normal position, saving his life.

• #65 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  Correction of the defect during infancy is the preferred treatment of D-TGA. Balloon atrial septostomy (BAS) is needed preoperatively in the case of restrictive atrial-level communication leading to cyanosis. […] The arterial switch operation (ASO) has been used instead of the AtrSR operation since the 1980s and is now the standard surgical correction for D-TGA. […] The prognosis for patients with D-TGA is generally excellent following surgical correction. The current survival rates are greater than 90%. The ASO has the best long-term survival and functional outcome. Some studies report a >95% survival rate at fifteen to twenty-five years following the discharge from hospital.

• #66 Transposition of the great arteries: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001568.htm

  Permanent treatment involves heart surgery during which the great arteries are cut and stitched back to their correct position. This is called an arterial switch operation (ASO). Prior to the development of this surgery, a surgery called an atrial switch (or Mustard procedure or Senning procedure) was used.

• #67 Transposition of the Great Arteries | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/transposition-of-the-great-arteries

  Most adults with transposition of the great arteries have been treated with a different operation that created an atrial switch and corrected the path of blood flow. These operations carry the names of the surgeons who first described them, the Senning or the Mustard procedure. The most common complications to arise from these types of operations include heart failure, abnormal heart rhythms and leaks in the „baffle” that diverts the blood flow along the right path. Nevertheless, many adults lead relatively normal lives and women have had successful pregnancies.

• #68 Transposition of the great arteries (TGA) – BHF

  https://www.bhf.org.uk/informationsupport/conditions/transposition-of-the-great-arteries

  This is a procedure thats usually done in the first few days of your life. Its where a balloon is used to open a hole in your heart to help blood high in oxygen move around your body. […] There are a few options to treat your TGA, all of which involve major surgery. The arterial switch operation is now the most common procedure. Before that was available, the atrial switch was most common. Sometimes the atrial switch or a Rastelli procedure are done when its not possible to do an arterial switch. […] Your surgeon switches your aorta and pulmonary artery back to their normal positions. Your surgeon also makes sure the blood flow to your heart is fixed. […] Your surgeon makes a tunnel (called a baffle) between the two sides of your heart, letting blood move around your heart. The procedure is called a Senning if the tunnel is made from part of your heart (tissue). Its called a Mustard if the tunnel is man-made (synthetic).

• #69 Transposition of the great arteries (TGA) – BHF

  https://www.bhf.org.uk/informationsupport/conditions/transposition-of-the-great-arteries

  Your surgeon makes a man-made tunnel between the bottom two chambers of your heart. This lets blood move from the left ventricle to the aorta. Your surgeon adds a tube (called a conduit) from the right ventricle to the pulmonary artery. This lets blood go around (bypass) the narrowed pulmonary artery. […] As you get older, its common to have problems with your heart. Youre more likely to have issues if your TGA was repaired by an atrial switch or Rastelli procedure. […] The complications can include: narrowing (stenosis) of the pulmonary artery, leaky heart valves (regurgitation), baffle narrowing or leaking, conduit narrowing or leaking, heart rhythm problems (arrhythmias), weakening of your heart muscle and heart failure. […] You may need more surgeries in the future to fix these problems, like a leaking valve or narrowed pulmonary artery.

• #70 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  Correction of the defect during infancy is the preferred treatment of D-TGA. Balloon atrial septostomy (BAS) is needed preoperatively in the case of restrictive atrial-level communication leading to cyanosis. […] The arterial switch operation (ASO) has been used instead of the AtrSR operation since the 1980s and is now the standard surgical correction for D-TGA. […] The prognosis for patients with D-TGA is generally excellent following surgical correction. The current survival rates are greater than 90%. The ASO has the best long-term survival and functional outcome. Some studies report a >95% survival rate at fifteen to twenty-five years following the discharge from hospital.

• #71 Transposition of the great arteries | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/transposition-great-arteries/

  If left untreated, over 50% of infants with transposition of the great arteries will die in the first month of life. About 90% will die in the first year. […] When a baby is first suspected of having D-type transposition of the great arteries (D-TGA), the first focus is to help the child maintain safe oxygen levels as well as safe heart and lung function. […] After a diagnosis is suspected or confirmed, doctors may: Give a steady dose of prostaglandins, a medicine that helps keep the ductus arteriosus open. This allows oxygen-rich blood to mix with some of the oxygen-poor blood. […] Once the diagnosis is confirmed, doctors may: Perform a balloon atrial septostomy. […] Corrective surgery usually is performed during the first week of life. […] Before surgeons developed the arterial switch procedure a generation ago, half the babies born with this defect died within a month. Up to 90% of babies didn’t live to see their first birthday. Now, some of the first babies to have this surgery to correct transposition of the great vessels are young adults and living full, active lives.

• #72 Transposition of the Great Arteries (TGA) – Cardiothoracic Surgery

  https://lsom.uthscsa.edu/ct-surgery/patient-care/congenital-heart/conditions-we-treat/transposition-great-arteries-tga/

  This is the most common of the cyanotic heart diseases. In transposition of the great arteries, the aorta arises from the right ventricle (RV) and the pulmonary artery arises from the left ventricle (LV). Survival is dependent upon mixing, usually by an atrial level connection or ASD. Up to 50% of patients will also have a VSD. The aorta is usually anterior and to the right of the PA. […] TGA is the most common congenital heart defect requiring early intervention. Currently, anatomic correction is performed early in life using the arterial switch operation. Since the LV is ejecting against the lower resistance pulmonary circulation, the repair must be carried out early or the LV will fail to thicken and be unable to support the systemic circulation following the switch. If LV pressure has already fallen to less than 60% of systemic, a two-staged approach involving initial PA banding may be used. The PA band restricts pulmonary blood flow and causes the LV to work harder, allowing for growth and thickening.

• #73 25: Transposition of the Great Arteries | Veterian Key

  https://veteriankey.com/25-transposition-of-the-great-arteries/

  In the weeks after birth, pulmonary vascular resistance decreases and, in the absence of a large VSD or outflow tract obstruction, the afterload on the LV decreases. […] Thus, anatomic repair is performed in patients with TGA and intact ventricular septum or a small VSD within the first days to weeks of life in order to avoid LV remodeling.

• #74 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  The etiology for the transposition of the great arteries is unknown; however, it is presumed to be multifactorial. The embryogenesis of TGA is complex and associated with conotruncal defects, the aortopulmonary septum, and disturbances of the bulb rotation that take place in that period. Currently, there are two main theories which try to explain the embryological mechanisms of TGA development. These are (1) the “extracardiac theory”—an anomaly of the aortopulmonary septum—and (2) the “infundibular theory”—anomalous infundibular rotation. […] The clinical features of D-TGA are solely dependent on the degree of mixing between the parallel circuits. Most patients present with signs and symptoms manifested during the neonatal period (first 30 days of life). The typical clinical manifestations of D-TGA include cyanosis, tachypnea, and a murmur resulting from a ventriculoseptal defect (VSD) or from pulmonic stenosis.

• #75 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK538434/

  Transposition of the great arteries (TGA) is a congenital cardiac defect characterized by an embryological discordance of the aorta and pulmonary trunk. In dextro-transposition of the great arteries (d-TGA), the aorta arises from the right ventricle and the pulmonary trunk from the left ventricle, resulting in 2 parallel circuits that are incompatible with life unless there is a shunt, such as a patent ductus arteriosus (PDA) or septal defect, to allow for blood mixing. […] The exact cause of d-TGA remains unclear, but it is believed to result from multiple complex factors. Understanding the pathogenesis is challenging, particularly due to difficulties replicating the condition in animal models. Traditionally, 2 main theories have been proposed to explain the embryological development of d-TGA. The „extracardiac theory” suggests that d-TGA arises from abnormal spiralization of the aortopulmonary septum during heart development, where the conotruncal septum follows a straight path instead of its normal spiral trajectory.

• #76 Echocardiographic Evaluation of Transposition of the Great Arteries – Congenital Cardiac Anesthesia Society

  https://ccasociety.org/education/echoimage/echocardiographic-evaluation-of-transposition-of-the-great-arteries/

  The management of patients with TGA has evolved significantly since the malformation was first described in the eighteenth century. Studies have extensively documented the importance of echocardiography in the comprehensive anatomic and hemodynamic assessment of this congenital cardiac lesion as well as its role in preoperative planning, intraoperative monitoring, and postoperative surveillance.

• #77 Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II

  https://www.mdpi.com/2077-0383/13/16/4823

  Correction of the defect during infancy is the preferred treatment of D-TGA. Balloon atrial septostomy (BAS) is needed preoperatively in the case of restrictive atrial-level communication leading to cyanosis. […] The arterial switch operation (ASO) has been used instead of the AtrSR operation since the 1980s and is now the standard surgical correction for D-TGA. […] The prognosis for patients with D-TGA is generally excellent following surgical correction. The current survival rates are greater than 90%. The ASO has the best long-term survival and functional outcome. Some studies report a >95% survival rate at fifteen to twenty-five years following the discharge from hospital.

• #78 Transposition of the great arteries (TGA)

  https://www.aboutkidshealth.ca/transposition-of-the-great-arteries-tga

  The arterial switch operation switches or swaps the great arteries back to their normal position, so that the aorta is connected to the left ventricle and the pulmonary artery to the right ventricle. […] TGA is a lifelong condition and therefore it is important to keep seeing a cardiologist as many complications can be detected before symptoms develop.

• #79 Transposition of the great arteries | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/transposition-great-arteries/

  A child is expected to have long-term follow-up with a cardiologist to watch for any issues as they arise. Follow-up will include regular heart exams, scheduled echocardiograms and perhaps exercise stress tests. When the child becomes an adult, they will need to see an adult congenital heart disease (ACHD) specialist to provide care.

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