The first thing you notice about Ann Louise Atherton (her real name is Hazel, but everyone calls her Ann Louise) is her eyes: playful, brilliant blue and sparkling with humor. When she throws a toy and you pick it up, she beams, knowing she’s hooked you into playing fetch. Sitting in her Princess stroller and playing with her mother’s iPhone, a small catheter tube by her hip is the only sign of the risky, life-saving heart surgery that Ann Louise went through before she was even born.
Early in her pregnancy, Mary Beth Atherton and her husband, Andrew, learned that Ann Louise’s still-developing heart was showing signs of critical aortic stenosis, a congential defect that prevents the aortic valve from opening completely. If left unrepaired, aortic stenosis causes the left ventricle to work harder to get blood to the body. Over time, the left ventricle becomes thicker and pumps blood at a higher rate, damaging the heart while failing to pump blood efficiently. At home in North Carolina, Mary Beth’s doctors warned her that repairing her baby’s heart after birth would involve several risky surgeries, and would likely result in serious mental delays—if Ann Louise survived at all. Despite the bleak prognosis, they also advised Mary Beth and Andrew against in utero surgery, warning of low survival rates and poor quality of life for the baby. They recommended preparing for the worst.
“We found Children’s Hospital Boston on our own,” Mary Beth says. Andrew called Gerald Marx, MD, senior associate in Cardiology, directly and spoke to him for two hours about Ann Louise’s chances if they came to Children’s for in utero heart surgery. Mary Beth’s doctors advised strongly against the trip, but Marx made a convincing case. “After talking to Dr. Marx, we didn’t even need to think about it,” says Mary Beth. “He gave us his pager and said to call him directly when we made up our minds. We ended up paging him on Thanksgiving, but he called back immediately and said ‘come to Boston…now!’” Two days and one Med Flight later, Children’s surgeons operated on Ann Louise in utero, repairing her aorta.
Ann Louise was due on March 24, 2008, but she arrived a month early and spent the first five weeks of her life in the Cardiac Intensive Care Unit. It was there that she first met her brothers, Graham and Reed, who travelled to Children’s to meet their new baby sister. “They had to keep her sedated because she was still healing from surgery,” says Mary Beth. “But when Graham held her hand, she squeezed back.”
Since her first surgery, Ann Louise has returned to Children’s 12 times for additional surgeries and check-ups. She and Mary Beth have spent more than 200 days in the hospital, and Ann Louise now looks forward to seeing the Cardiology team. When given a picture of Marx, she laughs and blows kisses at him. “He’s been so good to us,” she says. “Ann Louise thinks he’s part of our family.” Mary Beth also credits the Children’s Center for Families with “helping keep us sane” through the long visits.
Last month, Children’s surgeons replaced Ann Louise’s mitral valve; her last major surgery, for now. The 18-month-old will need her mitral valve replaced again when she’s 10, and her pulmonary valve will need to be replaced when she’s in her early teens. “The doctors we saw before coming to Children’s said Ann Louise wouldn’t live past 25 if we came to Boston for in utero surgery,” Mary Beth says. “The doctors at Children’s told us there’s no realistic basis for that statistic. This surgery’s only been done for 20 years! For all anyone knows, Ann Louise could live a normal, long life.”
For now, Mary Beth and Andrew are looking forward to bringing home a happy, healthy little girl who loves chewing on her toes, watching the Imagination Movers and flirting with doctors. “When we contacted Children’s, there was such a difference in how they approached things,” says Mary Beth. “We’d been told that Ann Louise had a 25 percent chance of dying. Dr. Marx said there was a 90 percent chance that she’d live. That’s what gives you hope.”