Aortic Stenosis: Sarah's Story

Born with a heart defect called aortic stenosis, Sarah had her first open heart surgery when she was just 3½ months old.

sarahIn the summer of 2008, she had her second open heart surgery at the Cardiac Center at The Children's Hospital of Philadelphia (CHOP).

Four days later, she was home. Two weeks later, she was off to her first day of second grade. And three weeks later, she was back to gymnastics.

"We did ask her to take it easy on the bars," her mother, Lynn, recalls.

Today, Sarah is one of the many happy children in whom CHOP's Cardiac Center team takes great pride.

The diagnosis: aortic stenosis

Hours after Sarah was born, a pediatrician recognized a serious heart murmur. Sarah's parents decided to bring her to the Cardiac Center. Here, a team diagnosed Sarah with moderate to severe aortic stenosis. That means that the heart valve that opens to allow blood to flow into the aorta was malformed and wasn't functioning correctly.

Sarah, then a tiny infant, spent a week in the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU). After careful evaluation, a team of cardiologists, anesthesiologists and surgeons made the decision to delay surgery until Sarah was stronger.

First surgery to treat Sarah’s aortic stenosis

Three-and-a-half months later, she returned to the Cardiac Center. Thomas Spray, MD, performed a surgery called the Ross procedure. In this operation, the aortic valve is replaced with the patient's pulmonary valve. The pulmonary valve is then replaced with one from a donated organ.

Doctors told Sarah's parents it was possible she would need another surgery before she turned 4. She would also return to the Hospital regularly for outpatient checkups.

Second surgery to replace donated pulmonary valve

"She went for 7 years with no deterioration in physical ability," her mother says. "Then they noticed some change."

The Cardiac Center team performed a catheterization to gather information about Sarah's heart that would help them decide on the proper course of treatment. They determined Sarah was ready for a larger pulmonary valve. During the surgery, the valve was replaced with an adult valve from a donated organ.

Recovery made easier

After surgery, Sarah spent several days in the Cardiac Center's patient-care units on the sixth floor of Children's Hospital. The Cardiac Center has 66 beds for its patients; the beds are on the same floor as the Cardiac Operative and Imaging Complex, as well as a playroom, schoolroom and family lounges. This contiguous space enhances communication among the many people on the Cardiac Center staff, and patients and families like having everything on the same floor for convenience and comfort.

"She had so much personal attention and the facility was beautiful," Sarah's mom says.

The Cardiac Center's child life specialist, who helps children cope with the stress of hospitalization, used teddy bears to encourage Sarah and another little girl to think about their experience.

"The girls cut open the front of the teddy bears," Sarah's mother says. "It was such a wonderful exercise for them to think about what they had just been through in a very fun way, without having to discuss anything scary."

Sarah's room was down the hall from the Cardiac Center playroom, where she and another patient watched movies together and played on the computer.

"The second day, as soon as she could walk, she lived in that playroom," her mother recalls.

Sarah’s surgery outcome

The surgery was successful and Sarah has made a complete recovery. Doctors don't know whether she will need more surgeries in the future, so they continue to monitor her carefully.

Sarah visits the Cardiac Center as an outpatient for checkups, and will continue to see a cardiologist for the rest of her life. Otherwise, she is a great, regular kid, who does well in school and loves tumbling and gymnastics.

"She'll tell people about her experience, but otherwise none of the other kids would ever know there was anything wrong," Sarah's mom says.

Originally posted: June 2009