Edward – Ebstein's Anomaly Patient Story

Image of Edward Dompenciel.
Edward Dompenciel in a black polo.

Not every patient at UPMC Children’s Hospital of Pittsburgh is a child. Take Edward Dompenciel, for example.

Edward, 53, was born with Ebstein’s anomaly, a congenital heart malformation that involves the tricuspid valve and the right ventricle. As a young boy of 2 or 3 years old, he remembers going to the cardiologist often in his native Puerto Rico.

Despite his heart condition, Edward was physically active and an athlete until his 20s. He did karate and played both basketball and baseball.

“My symptoms started after I stopped exercising,” he says.

Those symptoms included tiring more easily, heart palpitations, and an arrhythmia, or irregular heartbeat.

“They happened more often as I got older,” Edward adds.

Edward's Condition Worsens

Edward continued visiting the cardiologist as he continued into his adult years. The physician routinely ordered Edward to have echocardiograms regularly to check how his heart’s chambers and valves were working and how efficiently they were pumping blood through his heart.

Edward Dompenciel in a Def Leppard band tee.After Edward’s echo in 2020, his cardiologist found that his heart had grown. Moreover, his pulmonary artery pressure was found to be high. Moderate pulmonary artery hypertension, according to IJC Heart & Vasculature, is between 50 and 70 mm Hg. Edward’s was about 60 mm Hg.

Edward’s cardiologist referred him to a cardiac surgeon, who ultimately referred him to Jose Pedro da Silva, MD, at the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s Hospital of Pittsburgh. Dr. da Silva pioneered the Cone procedure, which is now known as the gold standard for repairing Ebstein’s anomaly.

Surgery in Pittsburgh

Edward took time away from his job at a bank in the summer of 2021 to come to Pittsburgh with his wife, Laura, for his surgery.

Edward Dompenciel and his family.

He was feeling “kind of OK” when he arrived in Pittsburgh, he says, despite being fatigued and experiencing palpitations.

After the surgery on July 30, 2021, though, he felt really well. Together with his wife and sister, who’s a physician in Ohio, Edward says the experience at UPMC Children’s was not just good but “grand.”

Edward had another echocardiogram at the beginning of February.

He says, “I am happy to tell you that the valve that used to be high up is now in the right place. I have a new heart. I feel so much better – I am working out 45 minutes a day now.”

Asked what words of advice he has for other patients or families who may be facing surgery for Ebstein’s anomaly, Edward says, “I am my cardiologist’s only patient with Ebstein’s anomaly. I haven’t met anyone else with my condition but I would tell them, don’t think about it even once, go to Pittsburgh. They are the best.”