Coarctation of the aorta (COA)

Having identified your child’s heart condition, the specialists at Children’s Hospital Boston can begin caring for him.

We treat coarctation of the aorta by repairing the narrowed vessel. Infants who are very sick and require care in our cardiac intensive care unit (CICU) may need emergency repair of the coarctation. Others who show few symptoms may have the repair scheduled on a less urgent basis.

Pre-surgery for severe cases If your child’s obstruction is severe, he’ll most likely be admitted to the CICU, and he may receive IV (intravenous) medications before surgery to stabilize him and keep his ductus arteriosus from closing.

Surgery

For most infants, surgery is the standard treatment. The surgeon cuts out the narrowed segment and sews the two healthy ends of the aorta back together, reconstructing the arch and establishing normal blood flow through the vessel.

If your child also has a large VSD, the surgeon will repair the defect at the time of the coarctation surgery. If there’s a bicuspid aortic valve, it will need to be followed by the cardiologist to make sure that no obstruction (stenosis) or backwards flow (regurgitation) develops later.

Surgery preferable for infants

The fibrous band of tissue that creates the coarctation is not usually amenable to balloon dilation; it has to be cut out surgically. Surgery also gives the surgeon a chance to neatly reconstruct the aortic arch.

But that repaired area can also become narrowed, fibrous tissue; so, as a second procedure, a catheterization/balloon dilation can be effective. In older children, teens or adults who are diagnosed for the first time, cardiologists might also insert a stent, since these patients are close enough to fully grown that they won’t outgrow the stent.

Cardiac catheterization (usually as a second procedure for older children)

During the procedure, your child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Once the catheter is in the heart, the cardiologist will pass an inflated balloon through the narrowed section of the aorta to stretch the area open. A small, rigid metal tube, called a stent, may also be placed in the narrowed area after the balloon dilation to keep the aorta open.

Complications and risks

Except for treatable complications that can from any surgery (such as bleeding or infection), complications from COA surgery are rare.

• The greatest risk—but still very rare—is damage to nerves in the region of the aortic arch. (In extremely rare instances, this could result in larynx/vocal cord damage.)
• Another complication (rarely seen today) is bacterial endocarditis(inflammation of the heart lining) after the child’s discharge from the hospital. The risk is somewhat higher if:
• artificial patch material (instead of the child’s own native tissue) is used to repair the coarctation
• the patient has particularly poor dental hygiene
• the patient has another systemic infection

If endocarditis is a risk, your child's cardiologist may recommend preventive antibiotics.

• Risks of aneurysm: There are two types of aneurysm that can present a risk:
• A brain aneurysm—a burst blood vessel—can be associated with vascular disease and high blood pressure.
• An aneurysm at the site of the coarctation repair, particularly if the patient has had a balloon dilation, can weaken the blood vessel wall. If a patient has had a dilation, the doctor will follow him over time with echo, CT scans or MRIs to make sure an aneurysm doesn’t develop. If an aneurysm does develop, it must be repaired.
• Complications from not treating COA: Failure to treat mild to moderate COA can lead to high blood pressure, which puts a child’s vascular system at risk and can pose long-term risks of:
• stroke
• heart attack
• ventricular hypertrophy (thickening)
• impaired cardiac function
• rupture of the aorta
• brain (cerebral) aneurysm (see bullet above)
• organ failure (e.g., kidney, liver) resulting from blood pressure that’s too low

At home: caring for your child after COA repair

• Your child’s symptoms will disappear immediately after COA repair.
• He’ll feel better soon, especially if his treatment was catheterization.
• He should avoid heavy lifting or rigorous sports for a few months while his body heals.
• He may need medication on an ongoing basis.

Your child’s cardiologist will follow his progress closely, and will offer recommendations for post-operative follow-up care—including wound care, a nutritional program to encourage weight gain, an appropriate exercise routine (which will help keep his blood pressure in check) and an oral hygiene program to prevent infection (if needed).

As he grows: your child’s longer-term outlook

If your child’s COA is repaired in infancy—before there’s any permanent damage such as chronic high blood pressure*—the long-term outlook is excellent. Your child may need repeat surgery or catheterization if the repaired area re-narrows (re-narrowing occurs in about 20 percent of infants in the first or second year after repair). But in general, most children who’ve had surgery for COA will live healthy lives, with activity levels, appetite and growth eventually returning to normal.

* After surgery, your child’s blood pressure may elevate temporarily, as his kidneys normalize. If this occurs, your doctor may prescribe medication to lower and stabilize your child’s blood pressure.                                                                                      

In rare instances, your child's cardiologist may recommend antibiotics to prevent your child from developing an inflammation of the heart lining (bacterial endocarditis) after his discharge from the hospital.

As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups. Your cardiologist will help you to create a longer-term care program as your baby matures into childhood and his teen years. We’ll treat any complications that develop, and we’ll advise later on daily-life issues such as exercise, activity levels, nutrition and (for females) pregnancy precautions.

Overall, most children lead a normal life after treatment. But your child will need periodic follow-up to watch for re-narrowing and to monitor his blood pressure. If your child has also had a bicuspid aortic valve, this condition can cause problems later in life; precautions against infection of heart tissue (endocarditis) will probably be needed. 

Outlook for patients diagnosed in childhood or adulthood

Patients diagnosed later in childhood or in adulthood who’ve already developed chronic high blood pressure need to be followed, and some will need to be treated for their high blood pressure. Once surgery repairs the COA, any thickening in the heart muscle (hypertrophy) that has formed because of continued obstruction will usually become normal over time.

Coping and support

At Children’s, we understand that a hospital visit can be very stressful. So, we offer many amenities to make your child’s and family’s hospital experience as pleasant as possible. Visit The Center for Families to learn all about:

• getting to Children’s
• accommodations
• navigating the hospital experience
• resources that are available for your family

We can connect you with lots of resources to help you and your family through this difficult time, including:

• patient education: From the office visit to pre-op to the recovery room, our nurses will be on hand to walk you through your child’s treatment and help answer any questions you may have—How long will I be separated from my child during surgery? What will the operating room be like? They will also reach out to you by phone, continuing the care and support you received while at Children’s.
• parent-to-parent: Want to talk with someone whose child has been treated for a coarctation of the aorta? We can often put you in touch with other families who’ve been through the same procedure that you and your child are facing, and who will share their experiences.
• faith-based support: If you’re in need of spiritual support, we’ll connect you with the Children’s chaplaincy. Clergy from Protestant, Jewish, Muslim, Roman Catholic and other faith traditions will listen to you and pray with you.
• social work: Our social workers and mental health clinicians have helped many families like yours. We offer counseling and assistance with the stresses relating to your child’s illness and dealing with financial difficulties.

As your child reaches adulthood, our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) can help. Children’s is a founding institution of BACH—an international center that provides long-term inpatient and outpatient care and advanced therapeutic options for congenital heart disease patients as they reach and progress through adulthood.

To find out more, visit the Family resources page of Children’s For Patients and Families website.