The Cone Procedure for Ebstein's Anomaly

The cone method treats Ebstein's anomaly, a heart valve condition affecting the heart's right side. Ebstein's anomaly is congenital, meaning the person is born with the defect.

Our center's founder and surgical director, José Pedro da Silva, MD, invented the cone procedure in 1993. It's now the worldwide standard for treating Ebstein's anomaly.

What is The Cone Procedure?

With Ebstein's anomaly, the heart's tricuspid valve doesn't work right. This means that blood doesn't flow as it should through the heart. This leads to many problems and can cause damage that worsens if not treated.

In the past, surgeons could only repair the valve in certain cases. This left many patients needing to have their valves replaced, which has poorer outcomes.

But Dr. da Silva's cone method offers a way to repair the tricuspid valve in nearly any case of Ebstein's anomaly. The surgery uses the patient's own tissue to create a new, cone-shaped valve that grows with them.

The cone method involves open-heart surgery. This means you or your child will be under general anesthesia and spend several days in the hospital.

Cone Procedure Benefits and Risks

Not every child born with Ebstein's anomaly needs surgery. Some have very mild cases and only need to be monitored as they get older.

Also, some babies may be too young for cone surgery. Other surgeries can keep them healthy until they are old enough for cone surgery.

Every surgery has risks for bleeding and infection. But for patients who have severe symptoms from Ebstein's anomaly, the benefits outweigh the risks.

Without treatment, research has found that the mean survival age for people with Ebstein's anomaly is just 32 years old. But with valve repair surgery, patients can live whole, healthy lives.

There are many benefits to cone surgery over other methods:

  • Better outcomes: The patient's body is more willing to accept the rebuilt valve because it's made from their tissue.
  • Fewer surgeries in the future: As the patient grows, the valve grows with them. By contrast, new valves need replacement over and over, adding more surgeries.
  • Still an option after the Starnes surgery: Starnes surgery is life-saving for some infants with Ebstein's anomaly. Dr. da Silva has experience performing cone surgery after Starnes.

How to Prepare for the Cone Procedure

Your team will review detailed instructions to help you prepare for you or your child's surgery. This includes when you or your child should stop eating or taking medicines. We also have a guide for families to help you get ready for surgery.

Many families travel from out of town for the cone surgery. We aim to ensure you only have to travel to Pittsburgh once. But even patients from Pittsburgh will likely spend several nights in the hospital after surgery.

Parents or guardians may want to bring:

  • A pillow and blanket for themselves.
  • Slippers and other comfort items for themselves.
  • Toiletries.
  • Snacks (preferably high-protein).
  • Electronic devices and charging cords.
  • Comfort items for your child (special blanket or stuffed animal).
  • Pajamas that button (for older children).
  • Snap-up onesies and sleepers or zipper sleepers.
  • Large swaddle blanket for babies.
  • Sound machine to create a calming environment.
  • You may also want to speak to a child life specialist about your child's special needs.

What to Expect Before, During, and After the Cone Procedure

Your team will talk with you in more detail about the cone surgery. Here's what to expect if you come to the Da Silva Center for Ebstein's anomaly surgery.

Before surgery

We encourage people who are traveling from out of town come to the clinic the day before surgery.

During this visit, we do testing and imaging if needed. You'll get to meet the whole team and get final instructions. Either Dr. da Silva or his surgical partner, Luciana da Fonseca da Silva, MD, will be there to answer your questions.

Morning of surgery

Your child will stay with you until the day of the surgery. On surgery day, you should park in the Penn Garage and take the Grape elevator to floor four. You'll meet the anesthesiologist and again meet with the surgical team.

During surgery

The cone method usually takes between 4 and 5 hours. During the surgery, a circulating nurse visits the operating room each hour and will provide updates to families and caregivers.

When the surgery is complete, the doctors will come to talk to families or caregivers and let them know how it went. You'll usually be able to see your child within the hour.

Immediately after surgery

Patients recover in the cardiac intensive care unit (CICU). They get specialized round-the-clock care. We make sure to manage any pain they have.

One parent or guardian can stay overnight with the child while they are in the CICU.

In the days after surgery

After recovering for a few days in the CICU, you or your child will move to a step-down intensive care unit (ICU).

They will continue to get pain medicine as needed. We keep children comfortable but are also careful not to over-prescribe. We also use music, art, and pet therapy to help children cope and speed recovery.

Two parents or guardians can stay overnight with children once they are in the step-down ICU.

Follow up visit

Patients have a follow-up visit around two weeks after surgery (it may be sooner for some children). Most children are ready to go back to school after the follow-up visit. The chest incision takes six to eight weeks to heal fully, so parents should limit the child's activity during that time frame.

You will continue to follow up with your regular cardiologist, who always has a line of communication with us.

Cone Procedure Resources for Families

Kids and their families can learn more about traveling to Pittsburgh for cone repair surgery.

You can also request financial help through Ebstein's Traveling Hearts. This nonprofit organization supports families traveling for surgery.

You may also wish to connect with other families through these private Facebook support groups: