Sweet Heart: Emma's Story

A MIGHTY HEART: Baby’s cardiac crisis spurs family to raise awareness, help create miracles for others

Within minutes of Emma Provenzano’s birth, her parents knew something was wrong. She wasn’t breathing well, and the nurses in the delivery room looked worried.

“They expect you to turn pink within a few seconds,” says her father, Chuck, 44. “But she stayed blue.”

Emma’s mother, Shannon, 33, had been present at the birth of two of her nephews, so she knew enough to realize her own newborn was in trouble. The baby was whisked away, but no one was explaining anything to the Provenzanos. Then Shannon saw the pediatrician they’d picked for Emma rushing down the hallway. He made eye contact with her but didn’t say a word as he made his way into the critical-care area of the hospital. That’s when Shannon’s blood ran cold.

“It was almost at a point of hysteria,” Shannon says. “Finally, her pediatrician came in and said that they couldn’t tell what it was, but something was very wrong.”

It turned out to be little Emma’s heart. The 7-pound, 13-ounce newborn was transferred immediately to Arnold Palmer Medical Center, where specialists knew how to diagnose an infant’s heart problems.

By that night, doctors had told Shannon and Chuck that Emma had been born with multiple heart defects, including transposition of the great vessels, pulmonary atresia and ventrical septal defect, all of which had gone undiagnosed during Shannon’s pregnancy. In a few hours, the Provenzanos had gone from experiencing one of the happiest moments of their lives – the birth of their first child – to the cold terror of realizing they could lose their little girl at any moment.

Six days later, Emma underwent open-heart surgery to implant a BT shunt, which would ensure proper blood flow while she grew and gained strength for a bigger surgical procedure. It was just the beginning for the little fighter, who would undergo three more open-heart procedures over the next four years.

Today, Emma has just turned 7, and although her heart continues to be an issue and she’ll likely need more operations down the road, she runs, laughs and plays just like any other child her age.

“She’s just go-go-go now,” Shannon says. “She’s thriving.”

If Emma had been born a decade or two earlier, that may not have been the case because pediatric heart surgeries weren’t always what they are today, says Chuck. That’s why it’s so important to the Provenzano family to raise money and awareness for organizations such as the American Heart Association, the Arnold Palmer Medical Center Foundation and Children’s Miracle Network, which help fund research and patient care. In late April, they’ll hold their seventh annual Emma's Miracle Golf Invitational at MetroWest Golf Club to raise funds for Children’s Miracle Network.

“Organizations like that will give more kids a chance,” Shannon says. “The more involved people can be, and the more gifts they give, the more they’ll be giving children a chance to thrive.”

Emma is one of those children who has benefited from – and survived due to -- the kind of work the American Heart Association and Children’s Miracle Network do.

Every 25 seconds, an American will have a coronary event, according to the Centers for Disease Control and Prevention. Heart disease is the leading cause of death in the United States. And according to the American Heart Association, nine out of every 1,000 babies born will have some sort of congenital heart defect, most of which prove to be minor. Some, like Emma, will be born with conditions that are much more serious. In Emma’s case, diagnosing her heart problems quickly and operating on her heart within a week of her birth likely saved her life.

That’s why her parents are so dedicated to organizations that help fund research to develop similar life-saving procedures.

Emma had her second open-heart surgical procedure when she was just 5½ months old. By that time, her heart had grown, thanks in part to the shunt that had been inserted, and doctors felt confident in performing a Glenn procedure. “This surgery provided re-plumbing of the blood flow through and around Emma’s heart,” Shannon explains.

By the time Emma was 3½, she wasn’t growing at the rate of a normal child and doctors knew they’d have to perform a third open-heart operation. In June 2008, Emma, who weighed just 25 pounds, went in for a Rastelli procedure, in which doctors use a conduit to re-route blood flow and give a patient use of all four chambers of the heart.

The surgery itself was successful, but for Emma, it would be a long time before she was able to go home. First, her lungs collapsed when doctors attempted to take her off a ventilator. Then she developed a rare form of pneumonia. Finally, doctors noticed her blood cell count rapidly dropping, a situation that wasn’t helped by multiple transfusions. Emma was ultimately diagnosed with a condition called heparin-induced thrombocytopenia, which is very rare in children. It meant that Emma could no longer be given the drug heparin, which is typically used for cardiopulmonary bypass during heart surgery.

At first her parents weren’t concerned. Emma’s next scheduled surgery was years away. But within days, doctors discovered a tear in Emma’s aortic valve, which necessitated a fourth operation.

“This was an extremely terrifying situation, as the primary drug used during cardiac surgery is heparin,” Shannon says. “Emma’s doctors and the staff at Arnold Palmer had to research alternative drugs for use during surgery while Emma was on the heart and lung bypass machine.”

Doctors decided that the drug bivalirudin was a safe alternative and they were able to go in and repair Emma’s aortic tear. Shortly after surgery, Emma was finally able to go home.

Within a year, Emma had gained 10 pounds and grown six inches, making her much closer to the size of a typical child her age. Now, if all goes well, Emma won’t have to return for surgery for another 10 or 20 years, Shannon says. And even then, the procedure may well be minor. She’ll need to have the conduit in her heart replaced, but advances in medical science might mean it won’t take an open-heart procedure to do it.

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If you met Emma today, you’d never know that this little girl’s heart almost gave out on her. You’d never guess that she’s had four open-heart surgeries, or that she knows her cardiac doctors so well that her mother jokingly calls one of them “Emma’s boyfriend.”

That’s because Emma is just a typical little girl – with a big mission. She and her little brother, Brandon, who turns 5 in March, along with their parents, are active in raising awareness and funds for several medical charities. Shannon estimates that the family takes part in a Children’s Miracle Network event at least once a month, and the kids even walk together during the American Heart Association’s annual Heart Walk.

“We do the tournaments and the fundraising knowing that they affect Emma and all the heart friends she has,” Shannon says. “Emma is so much more than your typical young girl. We do not hide her conditions, and we try to explain to her as much as she can understand about what she has gone through in her short life. She loves being involved with various charities to spread awareness for children in need.”

Emma is also active in organizing her namesake fundraising golf tournament, which is scheduled for April 28, but beyond that, she’s simply a normal little girl. She takes a hip-hop dance class and loves to sing and joke around with her brother.

“I'm just happy that she's going to be able to live a normal life,” Chuck says. “That wasn't always the case. There were so many different steps in the process where if something went just a little wrong, she would have had a drastically different life. Everything we could have hoped for happened.”

In fact, says Shannon, Emma’s doctors are impressed by how active and healthy she is when she visits them for checkups. There’s no reason to think that her future will be anything but bright.

“She continues to amaze us,” Shannon says with a smile. “She can be anything that her beautiful heart desires.”

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To find out more about:

Emma’s charity golf tournament, email This e-mail address is being protected from spambots. You need JavaScript enabled to view it .
Greater Orlando Children’s Miracle Network, visit www.gocmn.org.
The American Heart Association, visit www.heart.org.