THE FONTAN

The Bartholet family awaits Zach's most difficult surgery

By November 1999, doctors at Children's gave the Bartholets the green light for Zach's third and most complicated surgery. The timing of this final operation is what set Boston's Children Hospital apart for the Bartholets. Some facilities put off the procedure until the child is between four and eight years old. But doctors at Children's have refined the techniques, so a child like Zachary can reap the benefits of a strong heart during crucial developmental stages.

"It's always better to repair a child early in life," says Jonas. "Boston Children's led the concept of early repair and it's been our hallmark since the early 1970's."

With 18-month-old Zachary maintained by a heart/lung machine, Dr. Jonas and his team (under the watchful eye of Alan Alda) constructed a lateral tube called a Fontan. Like the first tube inserted more than a year earlier, this second tube allowed the blue blood from Zachary's lower body to also completely bypass his heart on its way to the lungs, relieving the heart of much of it's work-load. Shunting this used blood away from the fresh blood also allowed Zachary's blood oxygen levels to creep up towards 90 percent. The operation was a success.

		After four hours of surgery, Zach recovers in his mother's arms

Soon after his surgery, though, Zachary caught a common cold. The resulting congestion could have increased the pressure in his lungs to heart-stopping levels, if not for the small hole Jonas punctured in the Fontan tube. This procedure, now preformed in all Fontan patients, serves as a safety valve, allowing blood under high pressure to seep back into the heart. Thanks to this innovation Zachary is likely to live a long, healthy life.

"I used to hate doing [the Fontan]," says Jonas, a surgeon at Children's since 1983, "because I could never predict how well a kid was going to do. Now the odds are extremely good."

AFTERMATH

With all three surgeries safely behind him, Zachary approaches his third birthday a happy, healthy little boy. He's no longer the baby of the family since the arrival of little sister Natalie in April of last year. Sometimes their mother marvels at her good fortune.

"We were really lucky with him," she says. "Fifteen years ago, kids like Zachary had to go through a lot more."

"Boston's more aggressive treatment plan certainly paid off," says Rob says proudly of his 32-pound toddler. "Zach is larger than most kids his age and can out-wrestle his older sister."

How has the ordeal changed the Bartholets?

"It teaches you not to take anything for granted," says Rob. "But I'd like to say it didn't change me. I can't say I love my wife and kids any more than I would have had this not happened."

He reconsiders.

Today, Zach is a healthy toddler

"I do have a new respect for my wife," Robs says. "She showed incredible strength."

Karen, however, modestly insists no superhuman strength got her through her pregnancy and Zachary's three surgeries.

"People ask, 'How do you do it?' she says. "You just do."

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