Nine-year-old Addison is a headstrong “fighter” who never gives up. A talented athlete, she plays highly competitive travel soccer and club lacrosse, and according to her mom, is usually the fastest on the field. 

“I smile every time I see her run,” says Addison’s mom, Amy. “You would never know she was born with a heart condition.” 

A heart condition that nearly went undetected. 

When Addison was born via C-section, doctors at her local hospital discovered she had a heart murmur. They assured her parents, Amy and Matthew, that it would soon resolve; however, after several days, when Amy and Addison were ready for discharge, the murmur was still present. 

Testing showed the possibility of an underlying heart condition, and Addison was referred to Children’s Hospital of Philadelphia (CHOP) for a cardiac evaluation.  

At two weeks old, Addison underwent a series of tests to determine the cause of her heart murmur and was ultimately diagnosed with an atrial septal defect (a hole between the upper chambers of the heart) and partially anomalous pulmonary veinous return (PAPVR). 

PAPVR is a rare form of congenital (born with) heart disease that affects the flow of oxygen-rich blood from the lungs to the heart. In a healthy heart, oxygen-rich blood from the lungs is carried to the upper left heart chamber (the left atrium) and then travels throughout the body. In PAPVR, some of the blood vessels that move blood from the lungs to the heart (pulmonary veins) are attached to the wrong side of the heart. Blood from the lungs flows to the upper right heart chamber (the right atrium), which should only store oxygen-poor blood. This leads to too much blood in the right atrium, which takes a toll on the heart and a person’s overall health.  

Addison would eventually need surgery to reconnect the pulmonary veins to the left atrium and close the septal defect. However, since the newborn wasn’t experiencing symptoms, her cardiologist recommended waiting a few years to give her tiny heart a chance to grow.  

“We were grateful we had a chance to prepare,” says Amy. 

A world-class heart surgeon

Addison continued to be monitored by CHOP cardiology until – at two years old – her heart reached the appropriate size for surgery. 

During this time, Amy and Matthew had researched pediatric cardiothoracic surgeons and determined they wanted Thomas Spray, MD, the now retired Chief of Cardiac Surgery at CHOP to perform Addison’s open-heart surgery.  

“We were so grateful to be in CHOP’s backyard and to have access to a world-class heart surgeon,” says Amy. 

Addison’s surgery was scheduled; however, the toddler’s pre-op blood work uncovered a complication. Addison had autoimmune neutropenia, a rare disorder that causes lower levels of the white blood cells that protect against infection. A low white blood cell count makes surgery unsafe, and Amy and Matthew feared they would need to postpone Addison’s operation. Thanks to collaboration between the Cardiac Center and CHOP’s Division of Hematology, Addison was able to receive daily treatments to increase her white blood count. By the fourth day of treatment, she was approved for surgery. 

A holistic experience

Addison underwent open-heart surgery with Dr. Spray in June 2017. Her parents received regular updates throughout the procedure, and within 90 minutes, were able to visit their toddler in the Cardiac Intensive Care Unit (CICU).  

“The nurses in the CICU were fantastic,” Amy remembers. Addison has several food and medication sensitivities and couldn’t tolerate the calming medicine typically used to keep young children relaxed after surgery. “The nurses brought me a rocking chair, so I could rock her all night,” says Amy. “Her care was so individualized, from her food to her medication. Everything was specialized for her.” 

Within 24 hours, Addison was transferred to the Cardiac Care Unit, where she would spend the rest of her recovery time at CHOP.  

“I loved how holistic the experience was,” says Amy. “PT, OT and Child Life visited us right away. Child Life even visited by six-year-old son, Jackson, to answer his questions and give him gifts. Everything was done with excellence.”  

She also reflects on how important it was to be included in daily rounds. “I never felt like I shouldn’t be there,” she says. “I was able to be the point of continuity on the family side, and it was very helpful to always understand exactly what was happening and why.” 

Within two days of her transfer to the CCU, Addison was able to return home with her parents and brother.  

Beyond surviving – thriving!

Since her surgery, Addison has only needed routine annual cardiac evaluations. Last summer, she was seen by Jonathan Edelson, MD, an attending cardiologist and Medical Director of CHOP’s Sports Cardiology Clinic. “He said if it wasn’t for her scar, her heart would tell him she never had a defect,” says Amy.  

The fierce athlete has continued to thrive, always facing challenges with support from her loving family. Says Amy, “we remind her that she is an open-heart surgery survivor. She can do anything!”  

Matthew and Amy continue to support CHOP, hoping to accelerate advances in early diagnostics and in utero treatments that can lead to better outcomes for children born with CHD.  

“CHOP is near and dear to our hearts,” says Amy. “This is truly best-in-class medical care.”