Coarctation of the aorta

Answer 1

Definition

Aortic coarctation is a narrowing of part of the aorta (the major artery leading out of the heart). It is a type of birth defect. Coarctation means narrowing.

Alternative Names

Aortic coarctation

Causes, incidence, and risk factors

The aorta carries blood from the heart to the vessels that supply the body with blood and nutrients. If part of the aorta is narrowed, it is hard for blood to pass through the artery.

Aortic coarctation is more common in persons with certain genetic disorders, such as Turner syndrome. However, it can also be due to birth defects of the aortic valves.

Aortic coarctation is one of the more common heart conditions that are present at birth (congenital heart conditions). It is usually diagnosed in children or adults under age 40.

Coarctation of the aorta may be seen with other congenital heart defects, such as:

• Bicuspid aortic valve
• Defects in which only one ventricle is present
• Ventricular septal defect

Symptoms

Symptoms depend on how much blood can flow through the artery. Other heart defects may also play a role.

Around half of newborns with this problem will have symptoms in the first few days of life.

In milder cases, symptoms may not develop until the child has reached adolescence. Symptoms include:

• Dizziness or fainting
• Shortness of breath
• Pounding headache
• Chest pain
• Cold feet or legs
• Nosebleed
• Leg cramps with exercise
• High blood pressure (hypertension) with exercise
• Decreased ability to exercise
• Failure to thrive
• Poor growth

Note: There may be no symptoms.

Signs and tests

The health care provider will perform a physical exam and take your blood pressure and pulse in your arms and legs.

• The pulse in the femoral (groin) area or feet will be weaker than the pulse in the arms or the carotid (neck). Sometimes, the femoral pulse may not be felt at all.
• The blood pressure in your legs is usually weaker than in the arms. Blood pressure is usually higher in the arms after infancy.

The doctor will use a stethoscope to listen to your heart and check for murmurs. People with aortic coarctation have a harsh-sounding murmur that can be heard from the back. Other types of murmurs may also be present.

Coarctation is often discovered during a newborn's first examination or well-baby exam. Taking the pulses in an infant is an important part of the examination, because there may not be any other symptoms or findings until the child is older.

Tests to diagnose this condition may include:

• Echocardiography is the most common test to diagnose this condition, and it may also be used to monitor the patient after surgery
• Chest x-ray
• Heart CT may be needed in older children
• MRI or MR angiography of the chest may be needed in older children
• Cardiac catheterization and aortography

Both Doppler ultrasound and cardiac catheterization can be used to see if there are any differences in blood pressure in different areas of the aorta.

Treatment

Most newborns with symptoms will have surgery either right after birth or soon afterward. First they will receive medications to stabilize them.

Children who are diagnosed when they are older will also need surgery. Usually, the symptoms are not as severe, and more time will be taken to plan for surgery.

During surgery, the narrowed part of the aorta will be removed or opened. If the problem area is small, the two free ends of the aorta may be re-connected. This is called anastomosis. If a large part of the aorta is removed, a Dacron graft (a man-made material) or one of the patient's own arteries is used to fill the gap. A tube graft connecting two parts of the aorta may also be used.

Sometimes, balloon angioplasty may be done instead of surgery, but it has a higher rate of failure.

Older children usually need medicines to treat high blood pressure after surgery. Some will need lifelong treatment for this problem.

Expectations (prognosis)

Coarctation of the aorta can be cured with surgery. Symptoms quickly get better after surgery.

However, there is an increased risk for death due to heart problems among those who have had their aorta repaired. Without treatment, most people die before age 40. For this reason, doctors usually recommend that the patient has surgery before age 10. Most of the time, surgery to fix the coarctation is done during infancy.

Narrowing or coarctation of the artery can return after surgery. This is more likely in persons who had surgery as a newborn.

Complications

Complications that may occur before, during, or soon after surgery include:

• Aortic aneurysm
• Aortic dissection
• Aortic rupture
• Bleeding in the brain
• Endocarditis(infection in the heart)
• Heart failure
• Hoarseness caused by injury to the nerve to the larynx
• Impaired kidney function
• Paralysis of the lower half of the body (rare complication of surgery to repair coarctation)
• Premature development of coronary artery disease(CAD)
• Severe high blood pressure
• Stroke

Long-term complications include:

• Continued narrowing of the aorta
• Endocarditis (infection in the heart)
• High blood pressure

Calling your health care provider

Call your health care provider if:

• You or your child has symptoms of coarctation of the aorta
• You develop fainting or chest pain (these may be signs of a serious problem)

Prevention

There is no known way to prevent this disorder; however, being aware of your risk may make early diagnosis and treatment possible.

References

Fraser CD Jr., Carberry KE. Congenital Heart disease. In: Townsend CM, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 60.

In: Anderson RH, Baker EJ, Macartney FJ, Rigby ML, eds. Paediatric Cardiology. 3rd. Philadelphia, Pa: Churchill Livingstone Elsevier;2009:chap 46.

Webb GD, Smallhorn JF, Therrien J, et al. Congenital heart disease. Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 8th ed. St. Louis, Mo: WB Saunders; 2007: chap 61.

Answer 2

Definition

Aortic coarctation is a narrowing of part of the aorta (the major artery leading out of the heart). It is a type of birth defect. Coarctation means narrowing.

Alternative Names

Aortic coarctation

Causes, incidence, and risk factors

The aorta carries blood from the heart to the vessels that supply the body with blood and nutrients. If part of the aorta is narrowed, it is hard for blood to pass through the artery.

Aortic coarctation is more common in persons with certain genetic disorders, such as Turner syndrome.

Aortic coarctation is one of the more common heart conditions that are present at birth (congenital heart conditions). It is usually diagnosed in children or adults under age 40.

This condition may be related to cerebral aneurysms, which can increase the risk for stroke.

Coarctation of the aorta may be seen with other congenital heart defects, such as:

• Bicuspid aortic valve
• Defects in which only one ventricle is present
• Ventricular septal defect

Symptoms

Symptoms depend on how much blood can flow through the artery. Other heart defects may also play a role.

Around half of newborns with this problem will have symptoms in the first few days of life.

In milder cases, symptoms may not develop until the child has reached adolescence. Symptoms include:

• Chest pain
• Cold feet or legs
• Dizziness or fainting
• Decreased ability to exercise
• Failure to thrive
• Leg cramps with exercise
• Nosebleed
• Poor growth
• Pounding headache
• Shortness of breath

Note: There may be no symptoms.

Signs and tests

The health care provider will perform a physical exam and take the blood pressure and pulse in the arms and legs.

• The pulse in the groin (femoral) area or feet will be weaker than the pulse in the arms or neck (carotid). Sometimes, the femoral pulse may not be felt at all.
• The blood pressure in the legs is usually weaker than in the arms. Blood pressure is usually higher in the arms after infancy.

The doctor will use a stethoscope to listen to the heart and check for murmurs. People with aortic coarctation have a harsh-sounding murmur that can be heard from the back. Other types of murmurs may also be present.

Coarctation is often discovered during a newborn's first examination or well-baby exam. Taking the pulse in an infant is an important part of the examination, because there may not be any other symptoms until the child is older.

Tests to diagnose this condition may include:

• Cardiac catheterization and aortography
• Chest x-ray
• Echocardiography is the most common test to diagnose this condition, and it may also be used to monitor the patient after surgery
• Heart CT may be needed in older children
• MRI or MR angiography of the chest may be needed in older children

Both Doppler ultrasound and cardiac catheterization can be used to see if there are any differences in blood pressure in different areas of the aorta.

Treatment

Most newborns with symptoms will have surgery either right after birth or soon afterward. First they will receive medications to stabilize them.

Children who are diagnosed when they are older will also need surgery. Usually, the symptoms are not as severe, and more time will be taken to plan for surgery.

During surgery, the narrowed part of the aorta will be removed or opened. If the problem area is small, the two free ends of the aorta may be re-connected. This is called an end-to-end anastomosis. If a large part of the aorta is removed, a Dacron graft (a man-made material) or one of the patient's own arteries may be used to fill the gap. A tube graft connecting two parts of the aorta may also be used.

Sometimes, doctors will try to stretch open the narrowed part of the aorta by using a balloon that is widened inside the blood vessel. This type of procedure is called a balloon angioplasty. It may be done instead of surgery, but it has a higher rate of failure.

Older children usually need medicines to treat high blood pressure after surgery. Some will need lifelong treatment for this problem.

Expectations (prognosis)

Coarctation of the aorta can be cured with surgery. Symptoms quickly get better after surgery.

However, there is an increased risk for death due to heart problems among those who have had their aorta repaired. Without treatment, most people die before age 40. For this reason, doctors usually recommend that the patient has surgery before age 10. Most of the time, surgery to fix the coarctation is done during infancy.

Narrowing or coarctation of the artery can return after surgery. This is more likely in persons who had surgery as a newborn.

Complications

Complications that may occur before, during, or soon after surgery include:

• Aortic aneurysm
• Aortic dissection
• Aortic rupture
• Bleeding in the brain
• Early development of coronary artery disease(CAD)
• Endocarditis(infection in the heart)
• Heart failure
• Hoarseness
• Kidney problems
• Paralysis of the lower half of the body (a rare complication of surgery to repair coarctation)
• Severe high blood pressure
• Stroke

Long-term complications include:

• Continued or repeated narrowing of the aorta
• Endocarditis
• High blood pressure

Calling your health care provider

Call your health care provider if:

• You or your child has symptoms of coarctation of the aorta
• You develop fainting or chest pain (these may be signs of a serious problem)

Prevention

There is no known way to prevent this disorder. However, being aware of your risk may lead to early diagnosis and treatment.

References

Fraser CD Jr., Carberry KE. Congenital heart disease. In: Townsend CM, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 60.

In: Anderson RH, Baker EJ, Macartney FJ, Rigby ML, eds. Paediatric Cardiology. 3rd. Philadelphia, Pa: Churchill Livingstone Elsevier;2009:chap 46.

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 65.

Reviewed By

Review Date: 12/01/2011

Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine; and Michael A. Chen, MD, PhD, Assistant Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.