Infant Heart, the Size of a Walnut, Rebuilt and Running

Surgery Corrects Unique Defect That Routed Blood Through the Brain in a Bizarre Circuit

Sometime in the first seven weeks of life in the womb, Zachary Davis's heart got the wrong instructions.

The intricate folding, splitting and sprouting that produces the heart's four chambers and a tree of major blood vessels did not follow the normal genetic plan. The heart he was born with on Oct. 20 was not the engineering marvel that can take people through nine decades.

Still, Zachary's heart was a marvel in its own right. It shared many features of known congenital malformations. But it also had something not previously recorded in the annals of medicine. His coronary arteries, which normally deliver oxygen and nutrients directly to the heart muscle, were instead supplied by a bizarre circuit routed through his brain. It was as if an important package were being sent from one house in Washington to another down the street via . . . Baltimore.

"It usually takes lot for us to say, 'That's amazing,' " Richard A. Jonas, a heart surgeon at Children's National Medical Center, said last week. "We see a lot of unusual things. But this was out there."

This exotic misassembly was good enough to get the baby through gestation. It would not be good enough to get him through life. A week after Zachary was born, Jonas took his heart apart, added missing pieces and reconstructed it to something close to its original specifications. It took about four hours and included a 25-minute period when the infant was packed in ice, with no blood circulating.

One day soon, Zachary and his mother, Jessica Davis, will go home to La Follette, Tenn., a town of 8,000 on the Kentucky border. On this Thanksgiving holiday, however, the baby is still learning to eat enough to enable him to grow. He will need at least two more operations on his heart as he gets bigger. But doctors expect him to have a normal life.

"There are only a handful of people in the world who can take a problem like this, think it through, do a complete repair, and have the child turn out so well," Mary T. Donofrio, Children's director of fetal cardiology, said of Jonas. "He actually did three operations, and [Zachary] had no leftover heart defects."

Jonas, 54, a native of Australia, went to Children's last year after 20 years in Boston, where he was a professor at the Harvard Medical School. He is an advocate of "early primary repair" -- fixing heart malformations right after birth in a single operation. The team being assembled around him is turning Washington into a referral center for ultra-complicated pediatric heart surgery. Last week, he operated on an infant flown in from Abu Dhabi.

Early primary repair is difficult, even daring. How Zachary would tolerate a one-stop solution to his many heart defects, including the unique one, was hard to predict.

"What did I anticipate? It was sort of hard to have an anticipation," the surgeon said.

A Broken Arch

Jessica Davis is a 17-year-old high school senior. Zachary is her first child. The baby's father at this point is not in the picture, she says.

She went to school until April, when she left and began studying for her General Educational Development (GED) certificate at home. She hopes to return after Christmas and graduate with her class. She would like to be a cosmetologist.