David – Ebstein's Anomaly Patient Story

David Figueroa Flores with mom and dad
David Figueroa Flores as a baby

David Antonio Figueroa Flores looks and sounds like a typical 4-year-old from his home in Puerto Rico. He’s energetic with a big smile on his face, when he stops long enough to see his face over the computer screen.

You definitely wouldn’t think that David is a child who had been born with a serious heart condition – Ebstein’s anomaly. In Ebstein’s anomaly, the tricuspid valve is in the wrong position and the valve’s leaflets are malformed, so the valve does not work correctly. Ebstein’s anomaly is a rare, congenital heart defect.

David was diagnosed with Ebstein’s anomaly before he was born – his mother, Maritza Flores, had a sonogram when she was 32 weeks pregnant, and the sonogram detected an abnormality with the baby’s heart. He was born otherwise healthy. Both the sonogram and David’s birth took place in Puerto Rico.

David’s parents, Anthony and Maritza, say he did well for the first two years of his life, even with this congenital heart abnormality, Ebstein’s anomaly.

David Figueroa Flores days after surgeryThen, when he was 2, in December 2019, David was diagnosed with a cardiac arrhythmia, or an irregular heartbeat. His parents had realized he had an irregular heartbeat because they frequently checked his oxygen level and his pulse. His pediatrician referred him to a pediatric cardiologist in Puerto Rico, who wanted to have a cardiac catheterization done to investigate the arrythmia. But due to the COVID-19 pandemic, the procedure had to be delayed by a full year. During this time, David continued to be treated with medication for the irregular heartbeat.

In December 2020, David finally had his catheterization. The result? David needed surgery. But rather than undergo an open heart surgery called the Glenn procedure, David’s cardiologist called Victor Morell, MD, chief of Pediatric Cardiothoracic Surgery and co-director of the Heart Institute at UPMC Children’s Hospital of Pittsburgh. After evaluating the cath results, Dr. Morell thought David would need the Cone procedure to repair David’s tricuspid valve – the Ebstein’s anomaly he’d been diagnosed with before he was born.

The Cone procedure was pioneered by Jose Pedro Da Silva, MD, founding director of the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s. It’s the gold standard for treating Ebstein’s anomaly, in part because Dr. Da Silva uses the patient’s own tissue to create a “cone” – so the repair grows along with the patient. There is therefore usually not a need for a second surgery in patients who have had the Cone procedure.

The family came to Pittsburgh on June 7, 2021 – it was their first trip out of Puerto Rico.

“It was a little bit traumatic at first, but the surgery and everything went so well and the way they treated us was also really nice,” says Anthony.

David Figueroa Flores six months after surgeryThey were in Pittsburgh until July 16, 2021, staying at the Ronald McDonald House. David, an only child, is doing well and has even been able to stop taking his medication since his Cone procedure. They have done all their follow-up care with David’s cardiologist and pediatrician in Puerto Rico.

To other families in the same situation, the Figueroas have three pieces of advice:

  • Listen to your doctors.
  • Don’t hesitate to go to Pittsburgh to receive treatment.
  • Continue doing the recommended follow-up care.

Contact the Da Silva Center via email at dasilvacenter@chp.edu or call 412-692-5218.