In the nick of time: Kayley’s life-saving treatment for midaortic syndrome

teenager with midaortic syndrome
PHOTOS: COURTESY OF THE ESTEVES FAMILY

At 10, Kayley Esteves could often be found on the soccer field, dribbling a ball up and down the grass. She seemed the picture of health, but when she started experiencing chest pains, her mother, Sue, brought her to their local hospital. An initial chest x-ray revealed nothing. “But something still didn’t seem right,” remembers Sue. She made an appointment with a cardiologist at Boston Children’s Hospital, where Kayley had previously received care.

Although her blood pressure had seemed fine just a few weeks earlier, it had now skyrocketed to 201/160 — nearly twice what’s considered normal. Further testing and imaging scans suggested a diagnosis of midaortic syndrome. In this rare condition, the part of the aorta (the heart’s largest blood vessel) that runs through the chest and abdomen is narrow, leading to dangerously high blood pressure and other concerns.

girl with midaortic syndrome and her physician
Kayley with Dr. Kim

A mother’s instinct

After meeting with nephrologist Dr. Michael Ferguson, co-director of Boston Children’s Midaortic Syndrome and Renovascular Hypertension Center, and surgeon Dr. Heung Bae Kim, the family decided that surgery presented the best treatment option. As it turned out, the procedure — a bypass graft surgery, which makes detours that go around narrowed arteries to create new paths for blood flow — wasn’t just the best decision. It very well may have saved Kayley’s life.

During the procedure, Kim and his colleagues uncovered a large aortic aneurysm, which they believe could have proved fatal if it burst. They also confirmed that her aorta wasn’t fully formed: “It was like her blood was taking back roads to get to its destination instead of taking the highway,” explains Sue. The surgeons also completed an autotransplant of her kidneys, which relocated them within her body to improve blood flow.

“We were told later that Kayley probably wouldn’t have survived another year if she hadn’t received treatment,” says her mom. “Dr. Ferguson and the rest of team were amazed that we came to see them when we did — but I guess it was mother’s instinct. A 10-year-old shouldn’t have chest pains.”

girl with midaortic syndrome meeting Red Sox players
Kayley with Wally and some of the Boston Red Sox

Doing everything they can

 Five years later, Kayley has grown into driven teenager who likes watching movies, hanging with her friends and going to school — “She’s the only 15-year-old I know who loves school,” laughs Sue. And she’s still a familiar face on the athletic field, playing both soccer and lacrosse. The sports serve another purpose, too, acting as a barometer of her health. “If she gets out of breath or her chest hurts again, we’ll know there’s a problem,” explains her mom.

Like any kid with medical complexities, Kayley can get frustrated with her health, and with the unknown. But with regular follow-up appointments with Dr. Ferguson and the team, she knows she’s in good hands. “Kayley has a complicated case, and sometimes I get nervous thinking about her care as she transitions to adulthood,” Sue admits. “But her team at Boston Children’s has been wonderful. They are very honest with us that they can’t promise anything — but they do everything they can.”

Learn about the Midaortic Syndrome and Renovascular Hypertension Center.