A surgeon wearing a facemask in preparation for surgery.

A UPMC Children’s Hospital of Pittsburgh cardiothoracic surgeon is leading the way in treating a rare congenital heart defect. Jose Pedro da Silva, MD, pioneered a heart procedure to treat Ebstein’s anomaly that has become the worldwide standard. He has continued to expand the use of his cone procedure to improve care for patients with this rare birth defect.

Ebstein’s anomaly is a heart condition in which the tricuspid valve is displaced inside the right ventricle instead of being in the entrance of the ventricle chamber. The leaflets, or flaps, that open and close to allow blood to flow are also shaped wrong.

The tricuspid valve controls the flow of blood from the right atrium to the right ventricle. When it doesn’t work properly, the heart isn’t able to pump blood efficiently, which can lead to heart failure and other problems.

“Ebstein’s anomaly is often diagnosed in fetal life, and it affects life expectancy,” Dr. da Silva says.

Development of the Cone Procedure

In 1993, Dr. da Silva treated a 12-year-old girl in his native Brazil with severe symptoms from Ebstein’s anomaly. The patient was part of a small family and her mother was concerned that her daughter’s need for a tricuspid valve could make childbearing more difficult. Dr. da Silva began surgery planning to do the Danielson technique, which had been the standard approach since 1965.

But the little girl’s leaflets were displaced and adhered to the right ventricle wall. There wasn’t enough mobility to work with that technique.

“The only way to make the repair work was to mobilize the leaflets on the right ventricle,” he says. “I mobilized the septal leaflet and put it together with the anterior leaflet. With that maneuver, I made the valve work fairly well.”

After 3 years, he had to revisit the valve to make some improvements for the patient, which helped him further refine the technique. Later, Dr. da Silva was able to define this new procedure, dubbed the cone procedure because the repaired leaflets have a cone format. His first patient is now over 30 years old with children of her own.

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Early Care for Ebstein’s Anomaly

UPMC has a long history with the treatment of Ebstein’s anomaly. It started with Dr. Henry T. Bahnson who practiced in Pittsburgh and, in 1965, applied the surgery idealized by Hunter and Lillehey to repair the valve in Ebstein’s anomaly patients. However, challenges remained with treating the condition, requiring the need for further innovation in surgery.

“Many surgeons have been trying to develop techniques to repair Ebstein’s anomaly, but the malformation presents differently anatomically from patient to patient,” Dr. da Silva says. “The limitation with prior techniques is they didn’t cover the many variations within Ebstein’s anomaly.”

Prior treatments created a monocuspid valve and had limited long-term success, with more than half of children needing a valve replacement during the operation or later in life.

“When you repair a valve as a monocuspid valve, as in earlier methods, especially in young patients, the ring-shaped base of the valve called the annulus can grow at a different pace than the leaflet,” he says. “This type of repair can lead to regurgitation after a few years. The cone technique grows together with the heart.”

Doctors prefer to repair rather than replace the valve. Children who have valve replacement typically need future surgeries to replace the prosthesis because it does not grow with the child and can calcify. Each surgery comes with a risk of complications.

With the cone procedure, patients rarely need a valve replacement.

Continuing Innovation of the Cone Procedure

Dr. da Silva continues to push the boundaries of patients who are eligible for the cone procedure at the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s Hospital. The standard indication for the cone procedure is in patients who have symptoms from Ebstein’s anomaly, including cyanosis, heart failure, arrhythmia, or enlargement of the heart.

To avoid further damage to the heart, most patients undergo the procedure between the ages of 3 and 5. But with Dr. da Silva’s advances, the procedure now can be used in children who are just a few months old.

Newborns With Severe Symptoms

Some patients with severe illness at birth are in very critical condition and unable to undergo the cone procedure. Usually they first undergo the Starnes procedure, which makes the right ventricle smaller and improves the baby’s chance of survival in the first few months. This surgical technique saves the baby’s life, but the child has to live with a single ventricle, which can lead to long-term complications.

Dr. da Silva proposed doing the cone procedure in children a few months after the Starnes repair. Dr. da Silva’s team has performed this surgery in three patients in Pittsburgh so far, as well as additional patients in Brazil. His team is prepared to do more in this subgroup of patients with Ebstein’s anomaly.

“The application of the cone procedure in these patients is something Dr. da Silva developed at UPMC,” says Luciana da Fonseca da Silva, MD, PhD, a cardiothoracic surgeon at UPMC Children’s and wife of Dr. da Silva, who also performs the cone procedure. “We now can rescue the right ventricle and the heart will be closer to normal later on.”

“We are able to repair, rather than replace, almost all valves—even valves that are very rotated,” adds Dr. da Fonseca da Silva. “That is the difference in the work we are doing at UPMC Children’s Hospital of Pittsburgh for adults and children.”

Sainathan S, da Fonseca da Silva L, da Silva JP. Ebstein's anomaly: contemporary management strategies. J Thorac Dis. 2020;12(3):1161-1173. doi:10.21037/jtd.2020.01.18

Lopes LM, Bezerra RF, daSilva JP, da Fonseca da Silva L. Critical Ebstein's anomaly with circular shunt: from successful fetal therapy with non-steroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique [published online ahead of print, 2021 Mar 8]. Cardiol Young. 2021;1-4. doi:10.1017/S1047951121000081

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