Pediatric coarctation of the aorta (CoA) repair

What is a pediatric coarctation of the aorta (CoA) repair?

Coarctation of the aorta, or aortic coarctation, is a congenital heart disease, which means babies are born with the condition. It causes mild to serious symptoms depending on how severe the narrowing is.

Coarctation of the aorta is narrowing in the aorta, the body’s largest artery. The disease forces the heart to work harder to pump blood and oxygen to the body. Repair procedures open the narrowed aorta to prevent heart failure and send enough blood to the body. Treatment involves procedures to widen the aorta so it can provide enough blood and oxygen to the body.

Our pediatric heart team has extensive expertise in surgery and minimally invasive procedures for aortic coarctation repair, including:

Balloon angioplasty and stenting

Interventional cardiologists perform cardiac catheterization procedures to stretch the narrowed part of the aorta. This is done by inflating a special type of balloon to enlarge the narrowing and then placing a metallic support (stent) to hold the aorta at the larger size. Catheterization is a minimally invasive procedure that requires only a small skin puncture, rather than larger incisions.

Benefits of minimally invasive cardiac catheterization include less bleeding, less pain and a shorter hospital stay and recovery time. Balloon angioplasty and stenting may be a treatment option for older children and teens who have less severe cases.

Heart surgery

Heart surgeons perform cardiothoracic surgery to remove the narrowed section and reconnect the two ends of the aorta. Newborns who have extensive narrowing in the aorta usually need surgery soon after birth.

Techniques include:

• Resection with anastomosis - If the narrowed area is fairly short, surgeons may simply remove the narrowed part and sew the sections together end-to-end, or overlap them to create a wider opening.

• Aortic arch reconstruction - If the narrowed area is more extensive and involves the aortic arch, surgeons reconstruct it using open-heart surgery. They connect your baby to a heart-lung machine to circulate blood during the operation. They overlap the two ends and sometimes use a patch for a wider opening.

• Interposition graft repair - In older children or teens, the two ends may not reach each other. Surgeons attach a graft (synthetic or human-tissue tube) to connect the sections.

What are the benefits of a pediatric coarctation of the aorta (CoA) repair?

After repair of coarctation of the aorta, your baby’s health will improve, with:

• Less stress on the heart

• Lower blood pressure

• Better circulation of oxygen-rich blood to help your baby grow

• Easier breathing

• Easier feeding

Aortic coarctation repair helps prevent possibly life-threatening complications, such as:

• Heart failure because the baby’s heart is overworked

• High blood pressure in the upper body due to the increased force of blood from the heart

• Poor growth or organ and tissue damage in the lower body due to lack of oxygen

• Aortic aneurysm (weakened area that bulges from the aorta’s wall) or dissection (tear)

• Brain aneurysm, hemorrhage (bleeding) or a stroke

• Coronary artery disease

What are the risks of a pediatric coarctation of the aorta (CoA) repair?

Aortic coarctation repair procedures are safe, effective treatments that correct a life-threatening condition. At The Heart Center at Children’s Health, our team achieves excellent results with these procedures. Our outcomes show a very low risk of complications or a long-term need for unanticipated retreatments.

Your child’s doctor will discuss with you the specific risks for your child’s procedure.

Short-term risks of the repair procedures may include:

• Blockage or other problems in the artery used for catheterization

• Blood clots, which can lead to a stroke or other conditions

• Excess bleeding

• High blood pressure, which usually returns to normal during recovery

• Infection

Later in life, your child may develop complications such as:

• High blood pressure

• Aortic aneurysm or dissection, in very rare cases

What to expect with a pediatric coarctation of the aorta (CoA) repair

The right repair procedure for your child depends on their age, severity of the condition and other factors. Our pediatric heart surgeons and interventional cardiologists have expertise in the latest techniques to help your child thrive.

See general information about what to expect when you bring your child to Children’s Health for a procedure. There you’ll find details about what to expect with repair of coarctation of the aorta.

What to expect before a pediatric coarctation of the aorta (CoA) repair

What happens before a procedure or surgery depends on your child’s age and how serious the condition is. In all cases, your child’s treatment plan is developed by a multidisciplinary team of cardiologists, cardiac catheterization specialists, cardiac surgeons, cardiac anesthesiologists and cardiac intensive care specialists. You and your child will meet with the specialist who will perform the procedure to discuss that team’s recommended treatment plan.

Newborns - If the narrowing is severe, your baby may need to stay in the cardiac intensive care unit (CICU) after birth. Our cardiac critical care team takes care of your baby in preparation for emergency surgery to repair the coarctation.

Children and teens - Your child will have a preoperative visit the day before the procedure (either cardiac catheterization or surgery).

The care team will review what you need to do, including:

• What time to arrive the next day

• What time your child should stop eating and drinking

• Which medications to stop or continue taking and when

Before the procedure, your child may need tests, such as:

• Blood tests to check your child’s overall health

• Chest X-ray to view structures in and around the heart

• Echocardiogram (heart ultrasound) to view the narrowed area and examine the heart and its function

• Electrocardiogram (EKG) to assess heart rhythm

• Cardiac magnetic resonance imaging (MRI) or computed tomography (CT) scans for more detailed images of the narrowed area

What to expect during a pediatric coarctation of the aorta (CoA) repair

• Please prepare to spend most of a day at The Heart Center for the procedure, whether it’s surgery or catheterization. You and your child will arrive in the morning to check in and prepare.

• You and your child meet with the anesthesiologist, the doctor who plans and administers your child’s sedation or anesthesia during the procedure.

For catheterization procedures

You and your child meet the interventional cardiologist and other team members for the procedure. Balloon angioplasty includes these steps:

• The doctor makes a small puncture to access a blood vessel in your child’s groin. They insert a long, thin wire into the vessel to guide the catheter (thin, flexible tube).

• Using imaging as guidance, the doctor inserts a balloon-tipped catheter over the wire and threads it to the aorta.

• Once the catheter’s tip is inside the narrowed part of the aorta, the doctor inflates the balloon to stretch the area.

• In some cases, the doctor places a stent (small metal mesh tube) in the area to help keep it open.

• The doctor deflates the balloon, removes the catheter and closes and bandages the incision site in the groin.

For heart surgery

You and your child meet the surgeon and other team members who will help with the surgery. Each heart surgery technique differs, but in general, the procedures include these steps:

• The surgeon makes an incision down the center of your child's chest and separates the breastbone to access the heart. Or they make an incision in the side of the chest and separate the ribs to access the heart.

• For some procedures, we connect your child to a heart-lung machine. This equipment takes over the function of the heart and lungs to circulate oxygen-rich blood through your child’s body during the surgery.

• The surgeon removes the narrowed section of the aorta.

• Depending on which procedure your child is having, the surgeon reconnects the two ends of the aorta using various techniques.

• If the heart-lung machine was involved, we disconnect your child from it when their heart is beating as it should.

• The surgeon closes your child’s incision, and places a dressing. A small drainage tube is left in place and will be removed a few days after surgery. Your child will be sedated for this procedure.

What to expect after a pediatric coarctation of the aorta (CoA) repair

After either procedure, the care team helps you prepare for your child to come home, with instructions for:

• Healthy food and liquids during recovery

• Medications, including frequency, dosage and side effects to watch for

• Signs of illness in infants, such as poor weight gain, poor sleep patterns and difficulty feeding

• Exercise restrictions for children and teens, such as avoiding strenuous activities and those that could involve blows to the chest

• Wound care for the incision, including problems to watch for

• When to contact the care team about symptoms, such as a fever, chest pain, increased wound draining or pain or swelling that worsens.

After catheterization procedures

After the procedure, your child will spend a few hours in the recovery room as they wake up. Cardiac nurses monitor your child and check their vital signs, including heart rate, breathing and blood pressure. You can hold your child, and we may give them clear liquids if they aren’t nauseous.

The length of the hospital stay for a catheterization to repair coarctation of the aorta may vary:

• In some cases, your child can go home the same day of the procedure.

• Some children may need to stay overnight.

Your child may feel tired for several days after the procedure. Full recovery may take one to two weeks to get back to their usual activities.

After heart surgery

After surgery, we take your child to the cardiac intensive care unit (CICU) to begin recovery. As your child wakes up, our specialized CICU nurses closely monitor your child and check their vital signs.

For surgical aortic coarctation repair, the typical hospital stay is:

• CICU stay - Two to four days for infants, and one to two days for children and teens

• Regular inpatient unit - Another three to five days

After coming home from surgery, your child may feel tired for several days. Complete recovery can take four to six weeks.

Your child needs lifelong care with a cardiologist to monitor their heart health for problems such as:

• High blood pressure, which medications can manage

• Recoarctation (return of the condition), which, in rare cases, can occur even years after treatment, and can be treated with catheterization

• Other conditions, such as aortic aneurysms, coronary artery disease and aortic valve disease, such as aortic stenosis

Learn more about our Safe at Home Program.

How do I prepare my child for a pediatric coarctation of the aorta (CoA) repair?

A heart condition and the prospect of surgery or a procedure can make a child anxious and fearful, no matter what their age is. To comfort them and help reduce their stress, you can tailor your approach depending on your child’s age. Some tips to help your child have a positive patient experience include:

• Learn as much as you can about the procedures and their condition

• Be clear and honest when talking with your child about what will happen

• Ask your child questions to encourage them to talk with you about their thoughts and concerns

• Reassure your child that the procedure and hospital stay (if any) are temporary

• Be patient with your child and affirm their feelings

Child life services for pediatric coarctation of the aorta (CoA) repair

Our Child Life specialists help your child and family prepare for hospital stays and procedures with personalized support. Learn more about Child Life services, education and activities. Please call 214-456-6280 to speak with a Child Life specialist.

What questions should I ask my provider about a pediatric coarctation of the aorta (CoA) repair?

Your child’s care team at Children’s Health will answer all your questions about coarctation of the aorta, treatment options and other details.

You may want to ask questions such as:

• How will aortic coarctation repair help my child?

• Are there other treatment options for coarctation of the aorta?

• Will my child need to spend the night at the hospital after aortic coarctation repair?

• How do you decide whether catheterization or surgery is right for my child? If surgery, how do you decide which type is best?

• What can I do to help ensure that my child has a successful surgery or catheterization?

• What kind of anesthesia will my child receive for the procedure? What are the risks?

• What medications should my child stop taking before the procedure?

• Can I be with my child while they’re being prepped before the procedure?

• How soon will I be able to see my child after the procedure?

• What is your approach to pain management?

• How do this condition and the repair affect my child’s growth and development?

• Will my child need another surgery or procedure after this initial repair?