Eva Marie DeStefano is a vivacious, pretty, 6-year-old girl wiht huge, dark hazel eyes and a head full of shiny brown hair. And she's a bundle of energy, bouncing merrily around, playing hide-and-seek with her 3-year-old sister, Victoria Rose. Looking at Eva Marie, you would never imagine that when she was 4, she underwent complicated heart surgery.

The purpose of the surgery was to repair a big hole in Eva Marie's heart (technically known as a ventricular septal defect), one that was there even before she was born. The hole was closed with minimally invasive surgery, which makes the operation much easier for the patient, can shorten hospital stays from 10 days to 2, and leaves only a tiny scar. Child was there from the moment Eva Marie arrived at New York University (NYU) Medical Center for surgery until she was discharged two days later--and we revisited her when she happily joined in an American Heart Association walk just six weeks later.

A HEARTBREAKING DISCOVERY
In the United States, about one child in 120 is born with the congenital heart defect. The hole in Eva Marie's heart was located between the two lower chambers; one can also occur between the two upper chambers. Although some heart defects are found by ultrasound examinations during pregnancy, that wasn't the case with Eva Marie.

By the time she had surgery, the holewas the size of a dime. "Your heart is the size of your fist. Think how big a 4-year-old's fist is. Then imagine how big a dime-sized hole in a 4-year-old's heart is," explains Eva Marie's pediatric cardiologist, Alan Langsner, M.D., who practices in West Orange, NJ, not far from Eva Marie's home in Hackettstown.

Because of the hole, Eva Marie's heart was pumping the same blood twice. Instead of being pumped from her heart to the rest of her body, freshly oxygenated blood coming from her lungs spurted through the hole from one side of her heart to the other and was being immediately recycled through the lungs.

Her problem was found during a routine checkup two weeks after she was born, in March 1994. Later Ultrasounds showed Dr. Langsner exactly where the hole was. He told Eva Marie's parents, Katherine DeStefano, 25, and Michael Dyda, 26, that they could wait for a few years before doing anything about it--such holes often close by themselves, and if surgery was needed, it would be safer when Eva Marie was bigger.

But the hole did not close. When Eva Marie was 3 years old, Dr. Langsner said she needed surgery soon. "I asked what would happen if we did nothing at all," DeStefano recalls. Dr. Langsner said Eva Marie could become a cardiac cripple.

In time, he said, the abnormal blood flow would cause high blood pressure in her lungs (pulmonary hypertension) and her heart and lungs would be damaged forever. She would probably not live to a normal life expectancy. He also cautioned DeStefano and Dyda that the longer they waited, the more Eva Marie would become aware of her problem, ask questions, be frightened, and remember the operation.

HEARTWARMING TECHNOLOGY
Dr. Langsner did have good news: Eva Marie could benefit from minimally invasive surgery that required operating through a tine incision; it would be a quicker, less complicted operation for Eva Marie. She would have a shorter hospital stay, faster recovery time, and a much smaller scar.

The traditional method of repairing defect like Eva Marie's entailed sawing through the center of the breast-bone, spreading the ribs apart, stopping the heart and cutting it open, repairing the hole, then stitching the heart, restarting it, and bringing the ribs and breastbone together again. A patient would in in the intensive care unit for several days and then stay in the hospital for another week. She'd need morphine for pain, and would always have a big scar down the middle of her chest, from the base of her neck all the way to the bottom of her rib cage.

Clearly, minimally invasive surgery was the more desirable operation. So DeStefano and Dyda made an appointment for a surgical consultation wiht Dr. Langsner's colleague Stephen Colvin, M.D., the head of cardiothoracic surgery at NYU Medical Center. He had already used the technique to repair heart defects in more than 150 children over the past three years.

Meanwhile DeStefano and Dyda wanted to get a second opinion from the Deborah Heart and Lung Center in Browns Mills, NJ. "The doctor we met with said minimally invasive surgery was impossible. He laughed," DeStefano says. "He said, 'Her heart defect is on the left side of her chest and it's not possible to reach it from the right side, I'd like to meet the surgeon who can do that.'"

DeStefano and Dyda scheduled the surgery with Dr. Colvin for Thursday August 27, 1998.

PREPARING FOR SURGERY
DeStefano, then a stay-at-home mother, spent hours reading library books, hospital literature, and on-line information to learn more about her daughter's surgery. "Once we decided on surgery I spent the next four or five months heavily researching it. I wanted to learn what every tube that would go in her body means, how long the surgery takes, what happens in recovery . I did not want to be unprepared for what we'd see."

DeStefano also had to prepare Eva Marie. "I told her she had a boo-boo in her heart that needed to be fixed," she says. "She asked how they'd get to her heart. I said they'd make a little opening in her chest. She asked how they'd close the hole. We thought they'd need a patch, so I said they'd use a Band-Aid. I said she'd feel nothing because she'd be asleep: 'You'll be going night-night and when you wake up it'll all be done.' I stayed away from words like 'cut' or 'needles' or 'hurt.' But she was confused, and it broke my heart."

THE SURGERY
On Thursday morning, DeStefano, Dyda, and grandma Laura DeStefano brought Eva Marie to NYU Medical Center. (A Child photographer and I joined the family, too.) At 7:30am, Eva Marie drank a cup of juice containing a sedative. By 8am, she was drowsy and Dyda carried her into a preparation room. Fifteen minutes later, Eva Marie was unconscious and being wheeled into the operating room.

There, the surgical team moved into action. A tube-like ultrasound probe was slipped down Eva Marie's esophagus. It would send pictures to the surgeons for guidance. Dr. Colvin looked at a picture of the hole in Eva Marie's heart on the ultrasound screen.

It showed him something he could not have seen before: A jet of blood spurted through the hole and hit the valve between the upper and lower chambers. The jet battered the valve, which seemed to be scarred by every heartbeat--60 times a minute or more. Dr. Colvin made believed the scarring would repair itself when the hole was closed.

Dr. Colvin made an incision about two inches long on the right side of Eva Marie's chest between her forth and fifth ribs. (The tiny scar will eventually be under her right breast.) He held her ribs apart with a retractor and tacked her lung to one side. He connected Eva Marie to the heart-lung machine, which added vital oxygen to her blood, then pumped the blood through her body.

Next, Dr. Colvin stopped Eva Marie's heart by injecting a potassium solution into her aorta. He then opened her right atrium and looked down through her tricuspid valve. He saw the hole, and sewed it closed in two layers, using 15 stitches that will be a part of Eva Marie's heart for the rest of her life.

Dr. Colvin then closed the right atrium. He put in a temporary pacing wire, in case Eva Marie developed an irregular heartbeat after surgery. Finally, he made sure there were no air bubbles in her heart that night cause brain or lung damage. All by itself, Eva Marie's heart started beating again. Ultrasound showed the hole was closed. And her heart was already smaller because it no longer worked overtime to pump the same blood twice. After the doctors closed the two-inch incision on Eva Marie's chest, she was transferred to the recovery room. Less than a day after surgery, she was in a regular room on the pediatric floor. The next morning, Eva Marie was allowed to return home.

LIFE AFTER SURGERY
Dr. Langsner expected Eva Marie to recover quickly, and he was right. He says that often the full impact of the surgery is not clear until afterward, when parents realize how much the heart defect held their child back.

That was the case with Eva Marie: "Even in the recovery room, her cheeks were already a healthier, redder color," recalls DeStefano. "She's gained weight and gotten taller. And before, she'd always been mellow. She'd sit and read. When she did run around, she'd pant and sweat; her lips would turn purple when she went swimming. We didn't know we'd see any difference, and neither did she. Now there's just an explosion of energy."

Medical writer Janice Hopkins Tanne has won nine journalism awards.