History of Cone Procedure for Ebstein's Anomaly

Ebstein's anomaly is a rare congenital heart disease. It accounts for just 1% of all congenital heart diseases. Ebstein's anomaly affects the tricuspid valve and causes blood to leak through the valve when the heart pumps.

The abnormality may be mild with minimal symptoms or leak so severely that it's barely functional. This can affect the structures of the heart in different ways. This variation in anatomical types has made treating Ebstein's anomaly a challenge.

Valve Repair Before the Cone Procedure

We've known for a long time that it's better to repair a valve than replace it with an artificial one. Artificial valves wear out, and the child needs further surgery to replace them. They may also stop working so well as a child's heart outgrows them.

In the early 1970s, a physician at the Mayo Clinic developed a technique to repair the tricuspid valve. For decades, this was the most widely used valve repair surgery for Ebstein's anomaly.

However, the surgery only worked for specific anatomical types of the disease. It wasn't an option for 65% of kids with Ebstein's anomaly under 12. They still needed to have their tricuspid valve replaced.

The First Cone Procedure

In 1993, José da Silva, MD, saw a 12-year-old girl with a type of Ebstein's anomaly that current techniques couldn't repair. Dr. da Silva had been working on a new kind of tricuspid valve repair since 1989. He offered this option to her parents.

The new surgery would avoid a lifetime of valve replacements and give the girl her best shot at a healthy life. So, they agreed.

That was the first time Dr. da Silva did what he called the "cone procedure". He called it this because it involved creating a new cone-shaped valve.

He shaped this new valve from the child's existing heart valve tissue. This meant her body wouldn't reject it — a common problem with artificial or donated valves. The surgery worked but left some residual leaking that needed a second surgery a few years later.

Dr. da Silva has followed up with her over the years. Watch her story here.

Nearly three decades later, her tricuspid valve functions as normal, and she was also able to have a successful pregnancy. Often, people with heart valve diseases have high-risk pregnancies. Dr. da Silva wanted a treatment that didn't make pregnancy dangerous.

Hundreds of people have had similar success stories in the 30 years since the first surgery.

How the Cone Procedure Became the Standard of Care

The cone method differed from other approaches because it focused on repairing the existing valve. Dr. da Silva could use the cone method on nearly any child with a faulty tricuspid valve, no matter how severe.

In 2006, Dr. da Silva and his wife, Luciana da Silva, MD, presented their data about the cone technique. The highlights included:

  • Excellent results: They performed the surgery on 40 patients of all ages, and all recovered well.
  • No valve replacements: Some kids would have needed a valve replacement without the cone surgery. With the surgery, none of them did.

Since then, cone surgery has become the standard approach for tricuspid valve repair for Ebstein's anomaly. Drs. da Silva can repair any case of Ebstein's anomaly using the cone method. They have never had to replace a valve they've operated on in a person under 30.

The da Silvas perform cone surgeries here in Pittsburgh at the center Dr. da Silva founded. They also travel the world doing the surgery and teach other surgeons how to do it.

See our data on the cone method and learn more about outcomes at the Da Silva Center for Ebstein's Anomaly.

Contact the Da Silva Center for Ebstein's Anomaly

Our expert team members can always offer you or your child a consultation or second opinion. Our team will provide you and your child with a customized consultation and detailed care plan.

You can contact the Da Silva Center by email at dasilvacenter@chp.edu, by phone at 412-692-5218, or through our online form.

People outside the U.S. can contact our International Services Department. Reach them by phone at +1-412-692-3000 or by email at international@chp.edu.