Transposition of the Great Arteries is a congenital heart defect in which the two main arteries that carry blood away from the heart, the aorta and the pulmonary artery, are switched (transposed). This condition occurs during fetal development, and it results in a circulation where oxygen-poor blood from the body is pumped back into the body.
Oxygen-rich blood from the lungs is circulated back to the lungs. This leads to a lack of oxygen in the body, which can be life-threatening if not treated. Babies born with TGA usually show signs of cyanosis shortly after birth. This is due to the mixing of oxygen-poor and oxygen-rich blood in the circulatory system. The severity of symptoms depends on the presence of other heart defects or associated conditions.
Epidemiology
TGA has a prevalence of 4.7 cases per 10,000 live births, comprising three percent of all congenital heart diseases and constituting 20 percent of cases within the category of cyanotic heart diseases.
Anatomy
Pathophysiology
Transposition of the Great Arteries is characterized by the abnormal positioning of the two major arteries, the aorta, and the pulmonary artery, during fetal development. In a typical heart, the aorta arises from the left ventricle, facilitating the delivery of oxygen-rich blood to the body. At the same time, the pulmonary artery emerges from the right ventricle, directing deoxygenated blood to the lungs. However, in TGA, these vessels are transposed, leading to a discordant circulation pattern.
Consequently, oxygen-poor blood from the body is returned to the body, and oxygen-rich blood from the lungs circulates back to the lungs. This critical anatomical malformation results in systemic cyanosis, where insufficient oxygenated blood reaches the body tissues, leading to a bluish discoloration of the skin and mucous membranes. The impaired oxygenation poses a life-threatening condition for the affected neonate, necessitating prompt medical attention and intervention.
The underlying pathophysiology of TGA underscores the importance of surgical correction to restore a normal blood flow pattern, typically through procedures such as the arterial switch operation, which involves repositioning the aorta and pulmonary artery to their appropriate ventricles, thereby enabling a more physiologically sound circulation. Successful surgical correction is pivotal in mitigating the detrimental effects of TGA and promoting a favorable prognosis for affected individuals.
Etiology
Genetic Factors: There is evidence suggesting a genetic predisposition to congenital heart defects, including TGA. Mutations in certain genes involved in cardiac development may increase the risk. However, TGA typically does not follow a clear pattern of inheritance and often occurs sporadically.
Maternal Factors: Certain maternal factors during pregnancy may contribute to the development of congenital heart defects. These factors include maternal diabetes, exposure to certain medications, infections during pregnancy (such as rubella), and maternal age, particularly in older mothers.
Chromosomal Abnormalities: TGA can be associated with certain chromosomal abnormalities, such as Down syndrome (Trisomy 21). However, the majority of cases of TGA occur in individuals without known chromosomal abnormalities.
Multifactorial Causes: In many cases, the etiology of TGA is likely multifactorial, involving a combination of genetic and environmental factors. The interplay of various factors during critical stages of cardiac development can disrupt the normal formation and alignment of the heart and major vessels.
Genetics
Prognostic Factors
The outlook for individuals with Dextro-Transposition of the Great Arteries (D-TGA) is typically favorable after undergoing surgical correction. Present survival rates exceed 90%, indicating a high likelihood of positive outcomes following the intervention.
Clinical History
Physical Examination
The extent of cyanosis is contingent on the degree of mixing between the two parallel circulatory pathways. Factors influencing intracardiac mixing encompass the size and existence of an Atrial Septal Defect (ASD) or Ventricular Septal Defect (VSD). Neither exertion nor supplemental oxygen has an impact on cyanosis.
Patients typically maintain a respiratory rate exceeding 60 breaths per minute, yet without evident grunting, retractions, or flaring, presenting a comfortable appearance. Murmurs are generally absent unless a small VSD or pulmonic stenosis is present. A VSD-related murmur, if present, will manifest as pansystolic and be pronounced at the lower left sternal border. Pulmonic stenosis induces a systolic ejection murmur, notable at the upper left sternal border.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Double-Outlet Right Ventricle
Pulmonary Atresia
Tricuspid Atresia
Truncus arteriosus
Tetralogy of Fallot
Total anomalous pulmonary venous return
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
The treatment paradigm for Transposition of the Great Arteries (TGA) involves surgical interventions aimed at correcting the abnormal positioning of the aorta and pulmonary artery. The primary procedures include the Arterial Switch Operation (ASO) for cases without central pulmonic stenosis and the Rastelli procedure for individuals with D-TGA, a large VSD, and pulmonary stenosis.
During the ASO, the surgeon repositions the pulmonary trunk and aorta to their anatomically correct locations, with the coronary arteries reimplanted into the aortic trunk. In the Rastelli procedure, a baffle is used to close the VSD, redirecting oxygenated blood to the aorta, and a conduit is placed to shunt deoxygenated blood from the right ventricle to the pulmonary artery.
These surgical interventions are crucial for restoring normal blood flow patterns and improving the overall prognosis for individuals with TGA. The choice of procedure depends on specific anatomical considerations and associated cardiac abnormalities.
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Arterial switch operation: The arterial switch operation stands as the customary intervention for individuals with Dextro-Transposition of the Great Arteries (D-TGA) devoid of significant pulmonic stenosis. In the course of the arterial switch operation (ASO), the surgeon performs the transection of both the pulmonary trunk and the aorta, subsequently relocating them to their correct anatomical positions.
The coronary arteries are then mobilized and reimplanted into the aortic trunk. In cases where a Ventricular Septal Defect (VSD) is present, its repair is also undertaken during this surgical procedure.
Rastelli procedure: The Rastelli procedure is recommended for patients diagnosed with Dextro-Transposition of the Great Arteries (D-TGA), accompanied by a sizable Ventricular Septal Defect (VSD) and pulmonary stenosis.
In the course of this surgical intervention, the VSD is closed utilizing a baffle, redirecting oxygenated blood from the left ventricle into the aorta. Subsequently, a conduit is implanted, connecting the right ventricle to the pulmonary artery facilitating the shunting of deoxygenated blood into the pulmonary artery.
Transposition of the Great Arteries is a congenital heart defect in which the two main arteries that carry blood away from the heart, the aorta and the pulmonary artery, are switched (transposed). This condition occurs during fetal development, and it results in a circulation where oxygen-poor blood from the body is pumped back into the body.
Oxygen-rich blood from the lungs is circulated back to the lungs. This leads to a lack of oxygen in the body, which can be life-threatening if not treated. Babies born with TGA usually show signs of cyanosis shortly after birth. This is due to the mixing of oxygen-poor and oxygen-rich blood in the circulatory system. The severity of symptoms depends on the presence of other heart defects or associated conditions.
TGA has a prevalence of 4.7 cases per 10,000 live births, comprising three percent of all congenital heart diseases and constituting 20 percent of cases within the category of cyanotic heart diseases.
Transposition of the Great Arteries is characterized by the abnormal positioning of the two major arteries, the aorta, and the pulmonary artery, during fetal development. In a typical heart, the aorta arises from the left ventricle, facilitating the delivery of oxygen-rich blood to the body. At the same time, the pulmonary artery emerges from the right ventricle, directing deoxygenated blood to the lungs. However, in TGA, these vessels are transposed, leading to a discordant circulation pattern.
Consequently, oxygen-poor blood from the body is returned to the body, and oxygen-rich blood from the lungs circulates back to the lungs. This critical anatomical malformation results in systemic cyanosis, where insufficient oxygenated blood reaches the body tissues, leading to a bluish discoloration of the skin and mucous membranes. The impaired oxygenation poses a life-threatening condition for the affected neonate, necessitating prompt medical attention and intervention.
The underlying pathophysiology of TGA underscores the importance of surgical correction to restore a normal blood flow pattern, typically through procedures such as the arterial switch operation, which involves repositioning the aorta and pulmonary artery to their appropriate ventricles, thereby enabling a more physiologically sound circulation. Successful surgical correction is pivotal in mitigating the detrimental effects of TGA and promoting a favorable prognosis for affected individuals.
Genetic Factors: There is evidence suggesting a genetic predisposition to congenital heart defects, including TGA. Mutations in certain genes involved in cardiac development may increase the risk. However, TGA typically does not follow a clear pattern of inheritance and often occurs sporadically.
Maternal Factors: Certain maternal factors during pregnancy may contribute to the development of congenital heart defects. These factors include maternal diabetes, exposure to certain medications, infections during pregnancy (such as rubella), and maternal age, particularly in older mothers.
Chromosomal Abnormalities: TGA can be associated with certain chromosomal abnormalities, such as Down syndrome (Trisomy 21). However, the majority of cases of TGA occur in individuals without known chromosomal abnormalities.
Multifactorial Causes: In many cases, the etiology of TGA is likely multifactorial, involving a combination of genetic and environmental factors. The interplay of various factors during critical stages of cardiac development can disrupt the normal formation and alignment of the heart and major vessels.
The outlook for individuals with Dextro-Transposition of the Great Arteries (D-TGA) is typically favorable after undergoing surgical correction. Present survival rates exceed 90%, indicating a high likelihood of positive outcomes following the intervention.
The extent of cyanosis is contingent on the degree of mixing between the two parallel circulatory pathways. Factors influencing intracardiac mixing encompass the size and existence of an Atrial Septal Defect (ASD) or Ventricular Septal Defect (VSD). Neither exertion nor supplemental oxygen has an impact on cyanosis.
Patients typically maintain a respiratory rate exceeding 60 breaths per minute, yet without evident grunting, retractions, or flaring, presenting a comfortable appearance. Murmurs are generally absent unless a small VSD or pulmonic stenosis is present. A VSD-related murmur, if present, will manifest as pansystolic and be pronounced at the lower left sternal border. Pulmonic stenosis induces a systolic ejection murmur, notable at the upper left sternal border.
Double-Outlet Right Ventricle
Pulmonary Atresia
Tricuspid Atresia
Truncus arteriosus
Tetralogy of Fallot
Total anomalous pulmonary venous return
The treatment paradigm for Transposition of the Great Arteries (TGA) involves surgical interventions aimed at correcting the abnormal positioning of the aorta and pulmonary artery. The primary procedures include the Arterial Switch Operation (ASO) for cases without central pulmonic stenosis and the Rastelli procedure for individuals with D-TGA, a large VSD, and pulmonary stenosis.
During the ASO, the surgeon repositions the pulmonary trunk and aorta to their anatomically correct locations, with the coronary arteries reimplanted into the aortic trunk. In the Rastelli procedure, a baffle is used to close the VSD, redirecting oxygenated blood to the aorta, and a conduit is placed to shunt deoxygenated blood from the right ventricle to the pulmonary artery.
These surgical interventions are crucial for restoring normal blood flow patterns and improving the overall prognosis for individuals with TGA. The choice of procedure depends on specific anatomical considerations and associated cardiac abnormalities.
Arterial switch operation: The arterial switch operation stands as the customary intervention for individuals with Dextro-Transposition of the Great Arteries (D-TGA) devoid of significant pulmonic stenosis. In the course of the arterial switch operation (ASO), the surgeon performs the transection of both the pulmonary trunk and the aorta, subsequently relocating them to their correct anatomical positions.
The coronary arteries are then mobilized and reimplanted into the aortic trunk. In cases where a Ventricular Septal Defect (VSD) is present, its repair is also undertaken during this surgical procedure.
Rastelli procedure: The Rastelli procedure is recommended for patients diagnosed with Dextro-Transposition of the Great Arteries (D-TGA), accompanied by a sizable Ventricular Septal Defect (VSD) and pulmonary stenosis.
In the course of this surgical intervention, the VSD is closed utilizing a baffle, redirecting oxygenated blood from the left ventricle into the aorta. Subsequently, a conduit is implanted, connecting the right ventricle to the pulmonary artery facilitating the shunting of deoxygenated blood into the pulmonary artery.
medtigo
Transposition of great vessels
Updated :
June 24, 2024
Transposition of the Great Arteries is a congenital heart defect in which the two main arteries that carry blood away from the heart, the aorta and the pulmonary artery, are switched (transposed). This condition occurs during fetal development, and it results in a circulation where oxygen-poor blood from the body is pumped back into the body.
Oxygen-rich blood from the lungs is circulated back to the lungs. This leads to a lack of oxygen in the body, which can be life-threatening if not treated. Babies born with TGA usually show signs of cyanosis shortly after birth. This is due to the mixing of oxygen-poor and oxygen-rich blood in the circulatory system. The severity of symptoms depends on the presence of other heart defects or associated conditions.
TGA has a prevalence of 4.7 cases per 10,000 live births, comprising three percent of all congenital heart diseases and constituting 20 percent of cases within the category of cyanotic heart diseases.
Transposition of the Great Arteries is characterized by the abnormal positioning of the two major arteries, the aorta, and the pulmonary artery, during fetal development. In a typical heart, the aorta arises from the left ventricle, facilitating the delivery of oxygen-rich blood to the body. At the same time, the pulmonary artery emerges from the right ventricle, directing deoxygenated blood to the lungs. However, in TGA, these vessels are transposed, leading to a discordant circulation pattern.
Consequently, oxygen-poor blood from the body is returned to the body, and oxygen-rich blood from the lungs circulates back to the lungs. This critical anatomical malformation results in systemic cyanosis, where insufficient oxygenated blood reaches the body tissues, leading to a bluish discoloration of the skin and mucous membranes. The impaired oxygenation poses a life-threatening condition for the affected neonate, necessitating prompt medical attention and intervention.
The underlying pathophysiology of TGA underscores the importance of surgical correction to restore a normal blood flow pattern, typically through procedures such as the arterial switch operation, which involves repositioning the aorta and pulmonary artery to their appropriate ventricles, thereby enabling a more physiologically sound circulation. Successful surgical correction is pivotal in mitigating the detrimental effects of TGA and promoting a favorable prognosis for affected individuals.
Genetic Factors: There is evidence suggesting a genetic predisposition to congenital heart defects, including TGA. Mutations in certain genes involved in cardiac development may increase the risk. However, TGA typically does not follow a clear pattern of inheritance and often occurs sporadically.
Maternal Factors: Certain maternal factors during pregnancy may contribute to the development of congenital heart defects. These factors include maternal diabetes, exposure to certain medications, infections during pregnancy (such as rubella), and maternal age, particularly in older mothers.
Chromosomal Abnormalities: TGA can be associated with certain chromosomal abnormalities, such as Down syndrome (Trisomy 21). However, the majority of cases of TGA occur in individuals without known chromosomal abnormalities.
Multifactorial Causes: In many cases, the etiology of TGA is likely multifactorial, involving a combination of genetic and environmental factors. The interplay of various factors during critical stages of cardiac development can disrupt the normal formation and alignment of the heart and major vessels.
The outlook for individuals with Dextro-Transposition of the Great Arteries (D-TGA) is typically favorable after undergoing surgical correction. Present survival rates exceed 90%, indicating a high likelihood of positive outcomes following the intervention.
The extent of cyanosis is contingent on the degree of mixing between the two parallel circulatory pathways. Factors influencing intracardiac mixing encompass the size and existence of an Atrial Septal Defect (ASD) or Ventricular Septal Defect (VSD). Neither exertion nor supplemental oxygen has an impact on cyanosis.
Patients typically maintain a respiratory rate exceeding 60 breaths per minute, yet without evident grunting, retractions, or flaring, presenting a comfortable appearance. Murmurs are generally absent unless a small VSD or pulmonic stenosis is present. A VSD-related murmur, if present, will manifest as pansystolic and be pronounced at the lower left sternal border. Pulmonic stenosis induces a systolic ejection murmur, notable at the upper left sternal border.
Double-Outlet Right Ventricle
Pulmonary Atresia
Tricuspid Atresia
Truncus arteriosus
Tetralogy of Fallot
Total anomalous pulmonary venous return
The treatment paradigm for Transposition of the Great Arteries (TGA) involves surgical interventions aimed at correcting the abnormal positioning of the aorta and pulmonary artery. The primary procedures include the Arterial Switch Operation (ASO) for cases without central pulmonic stenosis and the Rastelli procedure for individuals with D-TGA, a large VSD, and pulmonary stenosis.
During the ASO, the surgeon repositions the pulmonary trunk and aorta to their anatomically correct locations, with the coronary arteries reimplanted into the aortic trunk. In the Rastelli procedure, a baffle is used to close the VSD, redirecting oxygenated blood to the aorta, and a conduit is placed to shunt deoxygenated blood from the right ventricle to the pulmonary artery.
These surgical interventions are crucial for restoring normal blood flow patterns and improving the overall prognosis for individuals with TGA. The choice of procedure depends on specific anatomical considerations and associated cardiac abnormalities.
Arterial switch operation: The arterial switch operation stands as the customary intervention for individuals with Dextro-Transposition of the Great Arteries (D-TGA) devoid of significant pulmonic stenosis. In the course of the arterial switch operation (ASO), the surgeon performs the transection of both the pulmonary trunk and the aorta, subsequently relocating them to their correct anatomical positions.
The coronary arteries are then mobilized and reimplanted into the aortic trunk. In cases where a Ventricular Septal Defect (VSD) is present, its repair is also undertaken during this surgical procedure.
Rastelli procedure: The Rastelli procedure is recommended for patients diagnosed with Dextro-Transposition of the Great Arteries (D-TGA), accompanied by a sizable Ventricular Septal Defect (VSD) and pulmonary stenosis.
In the course of this surgical intervention, the VSD is closed utilizing a baffle, redirecting oxygenated blood from the left ventricle into the aorta. Subsequently, a conduit is implanted, connecting the right ventricle to the pulmonary artery facilitating the shunting of deoxygenated blood into the pulmonary artery.
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