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Pediatric Cone Procedure

The cone procedure is a surgery to treat a very rare heart condition called Ebstein’s anomaly. The heart’s tricuspid valve sits lower in the heart than it should. In addition, the valve's three flaps (leaflets) are shaped incorrectly and stuck to the heart muscle, which keeps them from closing.

Surgeons at The Heart Center at Children’s Health are experts at performing the cone procedure to treat this extremely rare congenital heart disease.

What is a Pediatric Cone Procedure?

During a cone procedure, our pediatric heart surgeons separate the tricuspid valve leaflets from the heart muscle. They reshape the leaflets into a cone shape, rotate the cone and reattach it to the heart in the correct position.

What are the benefits of a Pediatric Cone Procedure?

Many children need the cone procedure to fix the heart valve defect and restore proper blood flow. In rare instances, children get better with medications alone. Without treatment, Ebstein’s anomaly allows blood to leak back into the heart’s upper right chamber or atrium (regurgitation). The heart has to work harder to pump blood. The heart can become enlarged, increasing the risk of heart failure.

What are the risks of a Pediatric Cone Procedure?

Most children still have slight valve regurgitation after the cone procedure. If the problem worsens over time, they may need another surgery. Other potential problems include:

  • Arrhythmias like supraventricular tachycardia (rapid heart rhythm) or Wolff-Parkinson-White syndrome (malfunctioning electrical signals that cause a fast heart rate)
  • Heart block that interferes with (blocks) electrical signals between the upper and lower heart chambers
  • Narrowed or compressed coronary arteries

What to expect with a Pediatric Cone Procedure?

Experts at our Fetal Heart Program may detect Ebstein’s anomaly during a woman’s pregnancy. Doctors also may detect the condition soon after birth or when a child is older and shows signs of heart failure.

Your child will receive care from a dedicated team of pediatric cardiologists, heart surgeons, anesthesiologists and nurses. We will walk you through the procedure to make sure you’re comfortable with the treatment and know what to expect as your child recovers.

What to expect before a cone procedure?

Some children with Ebstein’s anomaly can wait until they’re around age 5 to get cone surgery. In the meantime, they take medicines to lower the risk of heart failure. Some kids need a procedure called an ablation to block irregular electrical signals that cause arrhythmias. Our electrophysiologists specialize in these procedures.

What to expect during a cone procedure?

A cone procedure can take 4 to 6 hours. This is an open-heart procedure that requires opening up the chest to access the heart. Your child is under the care of our cardiac anesthesiologists who specialize in sedating small children undergoing complex heart surgeries.

Many children with Ebstein’s anomaly also need treatment for atrial septal defects (ASD). An ASD is a hole in the heart between the heart’s upper right and left chambers (atria). This opening allows oxygen-rich blood to go from the left into the right chamber, where it doesn’t belong, and mix with oxygen-poor blood. Too much blood in the chamber causes the heart to work harder. Our surgeons close this hole when they perform the cone procedure.

What to expect after a cone procedure?

After surgery, your child recovers in the state’s largest cardiac intensive care unit (CICU). Your child should be able to go home within 1 to 2 weeks.

During your child’s hospital recovery, our dedicated nurses show you how to care for your child’s surgical incision and medical needs at home. We help you feel confident taking care of any situation that may arise after heart surgery.

What questions should I ask my provider about the Pediatric Cone Procedure?

  • How many cone procedures have you performed?
  • What is the success rate?
  • Are there other treatment options for my child’s heart condition?
  • Will my child need additional heart procedures? If so, when?
  • How soon will I be able to see my child after surgery?

Pediatric Cone Procedure Doctors and Providers

Frequently Asked Questions

  • What other procedures might my child need?

    If valve leakage (regurgitation) continues after a cone procedure, your child may need valve replacement surgery. Replacement valves don’t grow as a child’s heart grows. As your child gets older, they will need additional valve surgeries to insert valves that are the right size.

    A child with an arrhythmia or heart block may need a pacemaker or ablation procedure to help the heart keep a healthy rhythm.

  • How can Ebstein’s anomaly and the cone procedure affect my child’s development?

    Most children do well after the cone surgery. Still, a cardiologist should monitor your child’s health to catch problems like arrhythmias early. This doctor can determine whether it’s safe for your child to participate in sports and other high-impact activities.