Coarctation of the Aorta
Before you begin reading about this heart defect, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
- What is coarctation of the aorta?
- What are the symptoms?
- How is it diagnosed?
- What are the treatment options?
- What kind of follow-up care is required?
What is coarctation of the aorta?
The aorta is the main artery that carries oxygenated blood out of the heart to the body. It starts at the left 
Coarctation of the Aorta
Enlarge+ventricle of the heart as one large vessel and branches out into the smaller vessels in the body. From the heart, the aorta arches up and then curves around. Arteries that deliver blood to the head, arms and other parts of the upper body branch off at the top of the arch. Arteries that deliver blood to the abdomen, legs, and other parts of the lower body branch off from the descending aorta.
In a child with coarctation of the aorta, the aorta is too narrow at the portion just after the upper-body arteries branch off - this obstructs blood flow. Because of this narrowing, the left ventricle of the heart must pump much harder than normal to move blood through the aorta to the lower body. This can lead to damage to the heart, or heart failure, and high blood pressure in the heart and brain as well as damage to organs in the lower body that don't get enough blood.
Sometimes children with this heart defect can also have a ventricular septal defect, a hole between the lower chambers of the heart, or a bicuspid aortic valve, which means the valve has two flaps instead of three.
What are the symptoms?
In severe cases, symptoms will appear within the first few days of life. The more the aorta is narrowed, the more severe the symptoms will be.
In infants where the narrowing is severe or moderate, symptoms can include:
- labored or rapid breathing
- weak femoral artery pulse (taken in the groin area)
- heavy sweating
- poor growth
- pale or gray appearance
- heart murmur - extra heart sound heard when the doctor listens with a stethoscope
If the narrowing is mild, coarctation of the aorta might not be diagnosed until the child is older or even an adult. In those cases, symptoms can include:
- high blood pressure
- cold feet or legs
- difficulty exercising (gets out of breath quickly)
- dizziness
- fainting
- nosebleeds
- headaches
- leg cramps
- heart murmur
How is it diagnosed?
When an infant has severe coarctation of the aorta, in most cases a doctor in the birth hospital will notice symptoms. Once your baby arrives at Children's Hospital, we will care for him or her in the Tabas Cardiac Intensive Care Unit (CICU).
Milder cases sometimes aren't diagnosed until the child is older. Pediatricians refer children to us for evaluation after parents notice symptoms or if they have high blood pressure.
Diagnosis may require some or all of these tests:
- echocardiogram - sound waves create an image of the heart
- electrocardiogram (ECG) - a record of the electrical activity of the heart
- chest X-ray
- pulse oximetry - a non-invasive way to monitor the oxygen content of the blood
- cardiac catheterization - a thin tube is inserted into the heart through a vein and/or artery in either the leg or through the umbilicus ("belly button")
- cardiac MRI - a three-dimensional image shows the heart's abnormalities
What are the treatment options?
This heart defect requires either cardiac catheterization or open-heart surgery, depending on the severity of the narrowing and on other factors such as the child's age and overall health.
Surgeons can remove the narrowed section of the aorta and then sew the ends of the aorta back together, or they can enlarge the aorta with a patch.
In catheterization, a cardiologist will thread a thin tube (catheter) with a balloon on the end of it through an artery in the leg up to the heart. Then, using the catheter, the cardiologist can inflate the balloon in the narrowed section of the aorta to open it, and might also place a stent, or a stiff metal cage, to keep it open.
Your child will recover from these procedures in the CICU.
What kind of follow-up care is required?
Through 18
Most children who have repair of coarctation of the aorta recover completely and won't require additional procedures. Rarely, the aorta becomes narrow again and balloon catheterization or surgery will be required.
Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physician. Patients will need to carefully follow doctors' advice. Sometimes, these children can have persistently elevated blood pressure, despite removal of the obstruction. This will mean that they will need to take blood pressure lowering medicines and may need to avoid certain isometric activities, like football, weight-training, and wrestling.
Into adulthood
It is important that children born with coarctation of the aorta continue to see a cardiologist. We will help patients transition care to an adult cardiologist.
The Philadelphia Adult Congenital Heart Center, a joint program of The Children's Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.
Because of enormous strides in medicine and technology, today most children with heart conditions go on to lead productive lives as adults.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: Jeffrey R. Boris, MD
Date: April 2009
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