About 20 weeks into their pregnancy, expecting parents Steph and Mondo attended an ultrasound appointment in their home city, San Antonio. While they were hoping to find out the gender of their baby, Steph and Mondo instead received news that something was wrong with their baby’s heart.

The doctor diagnosed their son, Aries, with Ebstein’s anomaly, a rare congenital heart defect with no known cause. This defect causes the tricuspid valve, which separates the right ventricle from the right atrium, to leak blood into the right atrium from the wrong direction. Symptoms can vary from a cough and shortness of breath to abnormal heart rhythms and a blueish coloring of the lips and skin.

Steph and Mondo knew that their son would need to undergo open heart surgery following his birth. After multiple surgeons declined their case due to the severity of Aries’ condition, their cardiologist referred them to a surgeon who was willing to accept their case.

“Aries had two open heart surgeries only two days apart at eight months old,” said Steph. “Our doctor at the time told us that he had not seen a case that severe in his career.”

The doctors performed the cone procedure on Aries, a technique to repair the malformation of the tricuspid valve using the patient's own tissue, but this procedure was not successful. The next day, Aries’ surgeon performed the Starnes and Glenn procedures, older techniques for Ebstein’s anomaly patients that temporarily improve heart function. Following Aries’ recovery from these surgeries, the family left with the right side of his heart closed off with a patch.

The family decided to seek care in Pittsburgh with Jose Pedro da Silva, MD, founding director of the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s Hospital of Pittsburgh. Dr. da Silva pioneered the cone procedure in 1993 and has been dedicated to using his expertise to treat patients from around the world in Pittsburgh.

At UPMC Children’s, Dr. Jose da Silva and Dr. Luciana da Fonseca da Silva were eager to accept this case and provide the family with hope for their son’s future.

"In Pittsburgh, the da Silvas were actually able to reverse the Starnes, keep the Glenn procedure, and perform a modified Cone procedure to fix Aries’ heart,” explained Mondo.

The surgery on their son was a success, and Aries has made an excellent recovery.

“We are so blessed and happy with how this procedure went. Aries looks so much healthier now,” says Steph.

Aries’ parents are thankful for the team at UPMC Children’s and the outstanding care that they provided. Their son is strong and continues to live a healthy life thanks to the da Silvas and the entire care team.

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

Aries’ treatment and results may not be representative of similar cases.