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Aortic Disease (Aortic Aneurysm and Aortic Dissection in Children)
Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).
If your child’s been diagnosed with aortic disease, including aortic aneurysm and aortic dissection in children, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
Aortic aneurysms and aortic dissections are a disease of the aorta (the main blood vessel that leaves the heart and brings blood out to the body). An “aortic aneurysm” is a condition in which there’s dilation (enlargement) of the blood vessel. An “aortic dissection” in children happens when the layers of the wall of the vessel begin to separate and blood begins to split the layers. In both cases, if the aortic wall becomes too thin it can burst or rupture. The risks and treatments of these diseases depend, to some extent, on their location within the aorta.
Normal Aortic Anatomy
The aorta is the main blood vessel that leaves the heart to take blood to the body. Anatomically, it leaves the heart and travels vertically toward the head. As it reaches the top of the thorax (the chest cavity) it makes a U-turn and travels back down toward the abdomen.
There are four regions to the aorta:
- ascending aorta, the portion between the heart and where it makes the U-turn at the top of the thorax. The coronary arteries, which feed the heart muscle itself, are connected to the ascending aorta immediately above the aortic valve (which separates the heart from the aorta).
- aortic arch, where the aorta makes the U-turn. The blood vessels feeding the head and arms branch off of the aortic arch in this region.
- descending aorta, which travels from the top of the thorax to the bottom. Along the way, small branches travel to the spinal cord.
- abdominal aorta, the portion within the abdomen. The branches in this area feed the kidneys, the stomach and intestines, as well as all of the other organs in the abdomen, eventually branching off toward the legs.
How Does the Heart Normally Work?
When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »
Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:
The symptoms and diagnosis vary with children, depending on whether they have aortic aneurysms or aortic dissections.
Diagnosing Aortic Aneurysm in Children
One of the challenges with aortic aneurysms is that they often don’t cause symptoms before rupturing. However, children may complain of chest pain, or develop symptoms of heart failure if the enlarged aorta causes the aortic valve to leak and allow blood to move backwards into the heart.
More often, an aneurysm is identified on routine follow-up or on imaging done for unrelated reasons. Sometimes findings on a chest X-ray may suggest the presence of an aneurysm (or dissection), but additional imaging studies (such as a CT scan or MRI) are required to make the diagnosis.
Certain children are at higher risk for developing aneurysm. That includes those with:
- family history
- certain genetic diseases, including Marfan syndrome (a genetic disorder that affects the body's connective tissue) and Loeys-Dietz syndrome (a condition similar to Marfan syndrome that affects the connective tissue and blood vessels throughout the body)
- other congenital heart disease, particularly those with “conotruncal abnormalities” (such as tetralogy of Fallot, transposition of the great arteries and other problems with the arteries), as well as patients with hypoplastic left heart syndrome
At the Nemours Cardiac Center, we have extensive experience with repairing aortic aneurysms in children with congenital heart disease.
Diagnosing Aortic Dissection in Children
Aortic dissections are much less common in children than in adults. Nearly all cases in children are related to genetic syndromes such as Marfan syndrome. Unlike aneurysms, an aortic dissection in children is more likely to be symptomatic (to cause symptoms).
Depending on the location, aortic dissections may cause:
- chest pain (often described as “tearing”)
- back pain
- cold or painful extremities
- kidney failure
All of these happen when the splitting of the layers of the aorta cause a blockage of blood flow down one of the branches. Again, a CT scan or MRI is usually used for diagnosis (although a chest X-ray may suggest the need for one of those studies).
Genetic Screening for an Aortic Aneurysm or Aortic Dissection in Children
Again, both aortic aneurysms and dissections can be caused by genetic defects that are sometimes inherited. The most common of those would be Marfan syndrome and Loeys-Dietz syndrome. (Learn more about Marfan syndrome from Nemours’ experts at KidsHealth.org.)
Overall, about 20 percent of the time, there’s a family history of aortic aneurysms and dissections. In these cases, we often recommend various genetic screenings of other members of the family, depending on the exact nature of the abnormality.
Nemours Cardiac Center’s certified genetic counselor offers counseling to our patients and families when the cause of any cardiac condition may have a genetic component. Providing further insight into the genetic cause of a condition frequently benefits the immediate and extended family as well as the child — so that you and your whole family will know if the heart condition could possibly affect siblings and generations of your family in the future.
At your request, our genetic counselor:
- analyzes your family history
- tests for various cardiac conditions at laboratories that are leaders in cardiac genetics
- counsels you related to inheritance and the implications of a positive genetic test result for your child and the entire family
Aortic aneurysms and aortic dissections are treated differently:
Treatment for Aortic Aneurysm in Children
The risk of aortic aneurysm rupture is directly related to the size of the aneurysm. Our Nemours Cardiac Center heart surgeons make a decision about whether or not to repair the problem based primarily on the size, although other factors may play a role as well, including the growth rate over time, a family history of similar problems, a known genetic disorder, and symptoms.
Surgery usually involves removing the enlarged portion of aorta and replacing it with a tube of artificial material (called a “graft”). Occasionally, an aneurysm may be repaired with a catheter procedure in which our cardiac catheterization specialist inserts a graft with a catheter (a hollow tube placed into an artery, usually in the leg) and then places it into the enlarged portion and inflated. This is called an “endograft,” which is more often an option for adults rather than children.
Treatment for Aortic Dissection in Children
We decide whether or not to repair a dissection with surgery based on the location and whether or not the child has symptoms. When the dissection is in the ascending aorta (the region between the heart and the blood vessels feeding the arms and head), a child will need emergency repair because of the risk of rupture or blockage of the coronary arteries (the small arteries which take blood to the heart muscle itself).
The survival rate without surgery is extremely low. But children who have surgery often do very well. When the dissection is in the descending aorta (the region between the vessels feeding the head and the abdomen), we only repair the problem if symptoms don’t go away with medical treatment alone. This is because the risk of blocking off a branch is less significant, and the risk of surgery is higher. In particular, the risk of paraplegia (paralysis and permanent loss of sensation) due to a loss of blood flow to the spinal cord is high during surgery on the descending aorta (this is because the blood vessels feeding the aorta branch off from this region of the aorta.
As with aortic aneurysms, the treatment of an aortic dissection in children is usually to replace the region of the aorta which has the dissection.
Surgical Treatment of Aortic Disease Affecting the Aortic Valve
The aortic valve is located at the start of the aorta as it leaves the heart. Its function is to prevent blood from flowing backward into the heart between contractions. When the aorta in this area (the ascending aorta) is enlarged or a dissection extends into the region of the valve (also called the “aortic root”), it may interfere with valve function and cause leakage of blood back into the heart. In these situations, surgery involves both replacing the abnormal aorta and either repairing or replacing the valve. For aortic problems in this area, there are a few options:
- Replacement of the ascending aorta alone. If the valve is working relatively well with only a small amount of leakage, it may be possible to replace the aorta above the valve without affecting or worsening valve function.
- Aortic root replacement. When the valve is leaking and the aorta is abnormal, one option is to replace both the aorta and the valve. There are several options including a:
- Bentall procedure. The aorta and valve are replaced with a “graft” and a manufactured valve (either a “mechanical” valve made mostly of carbon, or a “bioprosthetic” valve in which the leaflets are made of animal tissue). The aorta is replaced with an artificial graft.
- Homograft root replacement. This is similar to the Bentall procedure, but rather than using a manufactured valve and graft, a donated human valve and aorta (called a “homograft”) are used to replace the diseased portions.
- Valve-sparing aortic root replacement. In this operation (depending on the exact technique, it may also be called a “David” or “Yacoub” procedure), a graft is used to replace the abnormal aorta. But rather than replacing the child’s valve, the valve is preserved by attaching it to the graft (called “valve resuspension”). The advantage of this is that it preserves the native valve and doesn’t require blood thinners. But the long-term durability of this repair varies more than with either the Bentall or homograft root replacement.
In cases where surgery isn’t recommended, we’ll keep children on medicines to control the blood pressure, as well as other medicines that may help slow the speed of aortic enlargement. We’ll also perform imaging studies (usually CT scans or MRIs) on a schedule to keep an eye on any changes. Children with aortic aneurysms usually shouldn’t engage in weight-lifting or similar activities that may cause rapid increases in blood pressure, although they’re usually allowed to do aerobic exercise (activities that get the heart rate going such as walking, running, swimming, tennis, soccer, etc.).
Following surgery, children often need similar medicine as well as imaging studies to monitor the remaining aorta. Those who have a mechanical valve (made of carbon) will require life-long blood thinners. Children whose aortic disease is caused by a genetic syndrome will need to be monitored (usually either with MRI or CT scans) to make sure that the other areas of the aorta don’t develop similar problems.
If your child has an aortic aneurysm or dissection, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart disease journey — and to help your child live the healthiest, most fulfilling life possible.
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Information for Patients
Outpatient Services and Inpatient Units: (302) 651-6660
After 5 p.m. and Weekends:
Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690