Coarctation of the aorta is a congenital heart defect characterized by a narrowing or constriction of the aorta. This main artery carries oxygenated blood from the heart to the rest of the body. This constriction can occur anywhere along the length of the aorta, but it is most commonly found near the ductus arteriosus, which is a blood vessel that is present in the fetal circulation and usually closes shortly after birth.
Coarctation of the aorta is typically present at birth and results from abnormal development of the aorta during fetal growth. The exact cause is not always known, but it is believed to involve a combination of genetic and environmental factors. It may occur as an isolated defect or in association with other congenital heart abnormalities.
Epidemiology
Coarctation of the aorta accounts for approximately 5-8% of all congenital heart defects. The incidence is estimated to be around 1 in 2,500 live births, making it one of the more common congenital heart anomalies. There is a slight male preponderance, with CoA being more common in males than females. Coarctation of the aorta is often diagnosed in infancy or childhood.
However, the severity of the condition can vary, and some cases may not become apparent until later in life, particularly in cases with milder constriction. There may be variations in the incidence of CoA among different racial and ethnic groups, although various factors can influence these differences.
Anatomy
Pathophysiology
Coarctation of the aorta is a congenital heart defect, meaning it is present at birth. The exact cause of CoA is not always clear, but it is believed to involve a combination of genetic and environmental factors. During fetal development, abnormalities in the formation of the aorta can lead to a narrowed segment, impeding normal blood flow. The constriction in the aorta can occur in various locations.
However, it is most commonly found near the ductus arteriosus, a blood vessel that connects the pulmonary artery to the aorta in fetal circulation. The site of coarctation can influence the severity of symptoms and the age at which the condition becomes clinically apparent.
The narrowing of the aorta leads to increased pressure in the vessels upstream to the coarctation, particularly in the upper body and arms. The increased pressure in the proximal aorta leads to an increased workload on the left ventricle of the heart as it pumps blood against the constriction. Over time, this increased workload can lead to left ventricular hypertrophy and dysfunction.
Etiology
Chromosomal Abnormalities: CoA is occasionally associated with chromosomal abnormalities, such as Turner syndrome. Individuals with certain chromosomal disorders may have an increased likelihood of developing congenital heart defects.
Environmental Factors: Certain environmental factors during pregnancy may contribute to the development of CoA. Maternal factors such as exposure to certain medications, infections, or toxins may increase the risk.
Multifactorial Inheritance: CoA is often considered to have a multifactorial inheritance pattern, meaning that both genetic and environmental factors interact to influence the risk. The interplay of multiple factors may lead to the development of the defect.
Hemodynamic Changes: Hemodynamic changes in the fetal circulation, especially around the time of ductus arteriosus closure, can influence the development and manifestation of CoA. Abnormalities in the closure of the ductus arteriosus can affect blood flow patterns and contribute to the constriction of the aorta.
Genetics
Prognostic Factors
The prognosis of aorta coarctation has significantly improved with advancements in medical and surgical interventions. The prognosis for individuals with CoA depends on several factors, including the severity of the coarctation, the presence of associated heart defects, the age at diagnosis, and the timing of intervention.
Clinical History
Some individuals with coarctation of the aorta may present with symptoms in the neonatal period. The clinical history may include signs of left heart failure and shock shortly after birth. In milder cases, symptoms may not become apparent until later in infancy or childhood. Parents may report delayed developmental milestones or poor weight gain in infants with significant CoA.
In older children and adults, a common clinical feature is hypertension, particularly in the upper extremities. Individuals may experience exercise intolerance or fatigue due to the increased workload on the heart. Older individuals may experience headaches, nosebleeds, or other symptoms associated with hypertension.
Some individuals may develop claudication in the lower extremities due to reduced blood flow. In severe cases, symptoms may become evident within the first one to two weeks after birth, coinciding with the closure of the ductus arteriosus. The onset of symptoms in milder cases can be more gradual, and the diagnosis may be made in infancy, childhood, or even later in adolescence.
Physical Examination
Measurement of blood pressure in both arms and legs may reveal a significant gradient, with higher pressures in the arms and lower pressures in the legs. A characteristic heart murmur may be audible upon auscultation. The murmur is often heard in the left infraclavicular or left back region and is typically a systolic ejection murmur.
Inspection and palpation may reveal evidence of left ventricular hypertrophy, especially in more severe cases. Pain or discomfort in the legs during physical activity may be experienced by individuals in severe cases. In neonates with severe CoA, signs of shock, including pallor, lethargy, and poor perfusion, may be evident.
Collateral vessels may be visible on examination, especially in individuals with longstanding CoA. In infants, assessment of growth and developmental milestones is important, as delays may be associated with significant CoA. Radial pulses in the arms may be strong and bounding.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Aortic dissection
Myocarditis
Pediatric hypoplastic left heart syndrome
Coarctation of the aorta
Pediatric sepsis
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
For neonates and small children, surgical correction is frequently the preferred method. The surgical procedure involves removing the narrowed segment of the aorta and directly connecting the healthy segments. In adolescents and adults, cardiac catheterization and transcatheter techniques such as balloon angioplasty and stent placement may be considered.
Balloon angioplasty involves using a balloon to widen the narrowed portion of the aorta, while stent placement helps to maintain the expanded diameter. Following surgical intervention, there is a risk of coarctation, estimated at around 10% in neonates. If coarctation occurs, balloon angioplasty is often recommended as a secondary intervention.
Even after intervention, individuals with coarctation of the aorta are at an elevated risk of developing essential hypertension. Long-term follow-up and blood pressure management are crucial components of post-treatment care. Both treated and untreated individuals with coarctation of the aorta face an increased risk of cerebral aneurysms.
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
The treatment for coarctation of the aorta involves the removal of the narrowed segment, a process achievable through either surgical or transcatheter techniques. Surgical intervention entails the excision of the coarctation segment, followed by the direct anastomosis of the normal aorta. On the other hand, the transcatheter approach employs balloon and stent angioplasty.
Typically, surgical procedures are preferred for neonates and small children in most medical institutions. In contrast, primary stent angioplasty and cardiac catheterization are often considered for adolescents and adults. Despite these interventions, it is crucial to note that neither surgical nor interventional techniques provide a definitive cure for coarctation.
In neonates, the risk of coarctation following surgical intervention is approximately 10%. If coarctation occurs, balloon angioplasty is recommended. Additionally, individuals treated with balloon angioplasty alone face a lifelong risk of developing an aortic aneurysm, which appears to be higher compared to those who undergo other treatments.
Moreover, even after intervention, there is an elevated risk of developing essential hypertension. It is important to highlight that both treated and untreated individuals with coarctation of the aorta face an increased risk of cerebral aneurysms.
Coarctation of the aorta is a congenital heart defect characterized by a narrowing or constriction of the aorta. This main artery carries oxygenated blood from the heart to the rest of the body. This constriction can occur anywhere along the length of the aorta, but it is most commonly found near the ductus arteriosus, which is a blood vessel that is present in the fetal circulation and usually closes shortly after birth.
Coarctation of the aorta is typically present at birth and results from abnormal development of the aorta during fetal growth. The exact cause is not always known, but it is believed to involve a combination of genetic and environmental factors. It may occur as an isolated defect or in association with other congenital heart abnormalities.
Coarctation of the aorta accounts for approximately 5-8% of all congenital heart defects. The incidence is estimated to be around 1 in 2,500 live births, making it one of the more common congenital heart anomalies. There is a slight male preponderance, with CoA being more common in males than females. Coarctation of the aorta is often diagnosed in infancy or childhood.
However, the severity of the condition can vary, and some cases may not become apparent until later in life, particularly in cases with milder constriction. There may be variations in the incidence of CoA among different racial and ethnic groups, although various factors can influence these differences.
Coarctation of the aorta is a congenital heart defect, meaning it is present at birth. The exact cause of CoA is not always clear, but it is believed to involve a combination of genetic and environmental factors. During fetal development, abnormalities in the formation of the aorta can lead to a narrowed segment, impeding normal blood flow. The constriction in the aorta can occur in various locations.
However, it is most commonly found near the ductus arteriosus, a blood vessel that connects the pulmonary artery to the aorta in fetal circulation. The site of coarctation can influence the severity of symptoms and the age at which the condition becomes clinically apparent.
The narrowing of the aorta leads to increased pressure in the vessels upstream to the coarctation, particularly in the upper body and arms. The increased pressure in the proximal aorta leads to an increased workload on the left ventricle of the heart as it pumps blood against the constriction. Over time, this increased workload can lead to left ventricular hypertrophy and dysfunction.
Chromosomal Abnormalities: CoA is occasionally associated with chromosomal abnormalities, such as Turner syndrome. Individuals with certain chromosomal disorders may have an increased likelihood of developing congenital heart defects.
Environmental Factors: Certain environmental factors during pregnancy may contribute to the development of CoA. Maternal factors such as exposure to certain medications, infections, or toxins may increase the risk.
Multifactorial Inheritance: CoA is often considered to have a multifactorial inheritance pattern, meaning that both genetic and environmental factors interact to influence the risk. The interplay of multiple factors may lead to the development of the defect.
Hemodynamic Changes: Hemodynamic changes in the fetal circulation, especially around the time of ductus arteriosus closure, can influence the development and manifestation of CoA. Abnormalities in the closure of the ductus arteriosus can affect blood flow patterns and contribute to the constriction of the aorta.
The prognosis of aorta coarctation has significantly improved with advancements in medical and surgical interventions. The prognosis for individuals with CoA depends on several factors, including the severity of the coarctation, the presence of associated heart defects, the age at diagnosis, and the timing of intervention.
Some individuals with coarctation of the aorta may present with symptoms in the neonatal period. The clinical history may include signs of left heart failure and shock shortly after birth. In milder cases, symptoms may not become apparent until later in infancy or childhood. Parents may report delayed developmental milestones or poor weight gain in infants with significant CoA.
In older children and adults, a common clinical feature is hypertension, particularly in the upper extremities. Individuals may experience exercise intolerance or fatigue due to the increased workload on the heart. Older individuals may experience headaches, nosebleeds, or other symptoms associated with hypertension.
Some individuals may develop claudication in the lower extremities due to reduced blood flow. In severe cases, symptoms may become evident within the first one to two weeks after birth, coinciding with the closure of the ductus arteriosus. The onset of symptoms in milder cases can be more gradual, and the diagnosis may be made in infancy, childhood, or even later in adolescence.
Measurement of blood pressure in both arms and legs may reveal a significant gradient, with higher pressures in the arms and lower pressures in the legs. A characteristic heart murmur may be audible upon auscultation. The murmur is often heard in the left infraclavicular or left back region and is typically a systolic ejection murmur.
Inspection and palpation may reveal evidence of left ventricular hypertrophy, especially in more severe cases. Pain or discomfort in the legs during physical activity may be experienced by individuals in severe cases. In neonates with severe CoA, signs of shock, including pallor, lethargy, and poor perfusion, may be evident.
Collateral vessels may be visible on examination, especially in individuals with longstanding CoA. In infants, assessment of growth and developmental milestones is important, as delays may be associated with significant CoA. Radial pulses in the arms may be strong and bounding.
Aortic dissection
Myocarditis
Pediatric hypoplastic left heart syndrome
Coarctation of the aorta
Pediatric sepsis
For neonates and small children, surgical correction is frequently the preferred method. The surgical procedure involves removing the narrowed segment of the aorta and directly connecting the healthy segments. In adolescents and adults, cardiac catheterization and transcatheter techniques such as balloon angioplasty and stent placement may be considered.
Balloon angioplasty involves using a balloon to widen the narrowed portion of the aorta, while stent placement helps to maintain the expanded diameter. Following surgical intervention, there is a risk of coarctation, estimated at around 10% in neonates. If coarctation occurs, balloon angioplasty is often recommended as a secondary intervention.
Even after intervention, individuals with coarctation of the aorta are at an elevated risk of developing essential hypertension. Long-term follow-up and blood pressure management are crucial components of post-treatment care. Both treated and untreated individuals with coarctation of the aorta face an increased risk of cerebral aneurysms.
The treatment for coarctation of the aorta involves the removal of the narrowed segment, a process achievable through either surgical or transcatheter techniques. Surgical intervention entails the excision of the coarctation segment, followed by the direct anastomosis of the normal aorta. On the other hand, the transcatheter approach employs balloon and stent angioplasty.
Typically, surgical procedures are preferred for neonates and small children in most medical institutions. In contrast, primary stent angioplasty and cardiac catheterization are often considered for adolescents and adults. Despite these interventions, it is crucial to note that neither surgical nor interventional techniques provide a definitive cure for coarctation.
In neonates, the risk of coarctation following surgical intervention is approximately 10%. If coarctation occurs, balloon angioplasty is recommended. Additionally, individuals treated with balloon angioplasty alone face a lifelong risk of developing an aortic aneurysm, which appears to be higher compared to those who undergo other treatments.
Moreover, even after intervention, there is an elevated risk of developing essential hypertension. It is important to highlight that both treated and untreated individuals with coarctation of the aorta face an increased risk of cerebral aneurysms.
medtigo
Coarctation of aorta
Updated :
November 26, 2023
Coarctation of the aorta is a congenital heart defect characterized by a narrowing or constriction of the aorta. This main artery carries oxygenated blood from the heart to the rest of the body. This constriction can occur anywhere along the length of the aorta, but it is most commonly found near the ductus arteriosus, which is a blood vessel that is present in the fetal circulation and usually closes shortly after birth.
Coarctation of the aorta is typically present at birth and results from abnormal development of the aorta during fetal growth. The exact cause is not always known, but it is believed to involve a combination of genetic and environmental factors. It may occur as an isolated defect or in association with other congenital heart abnormalities.
Coarctation of the aorta accounts for approximately 5-8% of all congenital heart defects. The incidence is estimated to be around 1 in 2,500 live births, making it one of the more common congenital heart anomalies. There is a slight male preponderance, with CoA being more common in males than females. Coarctation of the aorta is often diagnosed in infancy or childhood.
However, the severity of the condition can vary, and some cases may not become apparent until later in life, particularly in cases with milder constriction. There may be variations in the incidence of CoA among different racial and ethnic groups, although various factors can influence these differences.
Coarctation of the aorta is a congenital heart defect, meaning it is present at birth. The exact cause of CoA is not always clear, but it is believed to involve a combination of genetic and environmental factors. During fetal development, abnormalities in the formation of the aorta can lead to a narrowed segment, impeding normal blood flow. The constriction in the aorta can occur in various locations.
However, it is most commonly found near the ductus arteriosus, a blood vessel that connects the pulmonary artery to the aorta in fetal circulation. The site of coarctation can influence the severity of symptoms and the age at which the condition becomes clinically apparent.
The narrowing of the aorta leads to increased pressure in the vessels upstream to the coarctation, particularly in the upper body and arms. The increased pressure in the proximal aorta leads to an increased workload on the left ventricle of the heart as it pumps blood against the constriction. Over time, this increased workload can lead to left ventricular hypertrophy and dysfunction.
Chromosomal Abnormalities: CoA is occasionally associated with chromosomal abnormalities, such as Turner syndrome. Individuals with certain chromosomal disorders may have an increased likelihood of developing congenital heart defects.
Environmental Factors: Certain environmental factors during pregnancy may contribute to the development of CoA. Maternal factors such as exposure to certain medications, infections, or toxins may increase the risk.
Multifactorial Inheritance: CoA is often considered to have a multifactorial inheritance pattern, meaning that both genetic and environmental factors interact to influence the risk. The interplay of multiple factors may lead to the development of the defect.
Hemodynamic Changes: Hemodynamic changes in the fetal circulation, especially around the time of ductus arteriosus closure, can influence the development and manifestation of CoA. Abnormalities in the closure of the ductus arteriosus can affect blood flow patterns and contribute to the constriction of the aorta.
The prognosis of aorta coarctation has significantly improved with advancements in medical and surgical interventions. The prognosis for individuals with CoA depends on several factors, including the severity of the coarctation, the presence of associated heart defects, the age at diagnosis, and the timing of intervention.
Some individuals with coarctation of the aorta may present with symptoms in the neonatal period. The clinical history may include signs of left heart failure and shock shortly after birth. In milder cases, symptoms may not become apparent until later in infancy or childhood. Parents may report delayed developmental milestones or poor weight gain in infants with significant CoA.
In older children and adults, a common clinical feature is hypertension, particularly in the upper extremities. Individuals may experience exercise intolerance or fatigue due to the increased workload on the heart. Older individuals may experience headaches, nosebleeds, or other symptoms associated with hypertension.
Some individuals may develop claudication in the lower extremities due to reduced blood flow. In severe cases, symptoms may become evident within the first one to two weeks after birth, coinciding with the closure of the ductus arteriosus. The onset of symptoms in milder cases can be more gradual, and the diagnosis may be made in infancy, childhood, or even later in adolescence.
Measurement of blood pressure in both arms and legs may reveal a significant gradient, with higher pressures in the arms and lower pressures in the legs. A characteristic heart murmur may be audible upon auscultation. The murmur is often heard in the left infraclavicular or left back region and is typically a systolic ejection murmur.
Inspection and palpation may reveal evidence of left ventricular hypertrophy, especially in more severe cases. Pain or discomfort in the legs during physical activity may be experienced by individuals in severe cases. In neonates with severe CoA, signs of shock, including pallor, lethargy, and poor perfusion, may be evident.
Collateral vessels may be visible on examination, especially in individuals with longstanding CoA. In infants, assessment of growth and developmental milestones is important, as delays may be associated with significant CoA. Radial pulses in the arms may be strong and bounding.
Aortic dissection
Myocarditis
Pediatric hypoplastic left heart syndrome
Coarctation of the aorta
Pediatric sepsis
For neonates and small children, surgical correction is frequently the preferred method. The surgical procedure involves removing the narrowed segment of the aorta and directly connecting the healthy segments. In adolescents and adults, cardiac catheterization and transcatheter techniques such as balloon angioplasty and stent placement may be considered.
Balloon angioplasty involves using a balloon to widen the narrowed portion of the aorta, while stent placement helps to maintain the expanded diameter. Following surgical intervention, there is a risk of coarctation, estimated at around 10% in neonates. If coarctation occurs, balloon angioplasty is often recommended as a secondary intervention.
Even after intervention, individuals with coarctation of the aorta are at an elevated risk of developing essential hypertension. Long-term follow-up and blood pressure management are crucial components of post-treatment care. Both treated and untreated individuals with coarctation of the aorta face an increased risk of cerebral aneurysms.
The treatment for coarctation of the aorta involves the removal of the narrowed segment, a process achievable through either surgical or transcatheter techniques. Surgical intervention entails the excision of the coarctation segment, followed by the direct anastomosis of the normal aorta. On the other hand, the transcatheter approach employs balloon and stent angioplasty.
Typically, surgical procedures are preferred for neonates and small children in most medical institutions. In contrast, primary stent angioplasty and cardiac catheterization are often considered for adolescents and adults. Despite these interventions, it is crucial to note that neither surgical nor interventional techniques provide a definitive cure for coarctation.
In neonates, the risk of coarctation following surgical intervention is approximately 10%. If coarctation occurs, balloon angioplasty is recommended. Additionally, individuals treated with balloon angioplasty alone face a lifelong risk of developing an aortic aneurysm, which appears to be higher compared to those who undergo other treatments.
Moreover, even after intervention, there is an elevated risk of developing essential hypertension. It is important to highlight that both treated and untreated individuals with coarctation of the aorta face an increased risk of cerebral aneurysms.
Loading...
Free CME credits
Both our subscription plans include Free CME/CPD AMA PRA Category 1 credits.
Digital Certificate PDF
On course completion, you will receive a full-sized presentation quality digital certificate.
medtigo Simulation
A dynamic medical simulation platform designed to train healthcare professionals and students to effectively run code situations through an immersive hands-on experience in a live, interactive 3D environment.
medtigo Points
medtigo points is our unique point redemption system created to award users for interacting on our site. These points can be redeemed for special discounts on the medtigo marketplace as well as towards the membership cost itself.
Community Forum post/reply = 5 points
*Redemption of points can occur only through the medtigo marketplace, courses, or simulation system. Money will not be credited to your bank account. 10 points = $1.
All Your Certificates in One Place
When you have your licenses, certificates and CMEs in one place, it's easier to track your career growth. You can easily share these with hospitals as well, using your medtigo app.
