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

Stopping the Blood

Some of the surgery Jonas would do to fix Zachary's heart could be performed while the baby was on a heart-lung machine that pumps and oxygenates blood. But not the most important tasks.

That's because the machine needs to have an aorta to pump blood into, and during much of the operation Zachary's aorta was going to be in pieces. Jonas was going to have to cut it open, graft on a piece of vessel from an organ donor, and sew the reconstructed arch to its proper attachments.

During that time the infant would have no circulation. He would be cooled in ice to below 60 degrees Fahrenheit, a state of "deep hypothermia" that slows metabolism and reduces the body's demand for oxygen.

A newborn's heart is about the size of a large walnut. The operation is done with the surgeon wearing jeweler's magnifiers attached to glasses. How long a baby can be kept safely in "circulatory arrest" varies, but it is generally not more than 45 minutes. With each minute beyond a time limit that the surgeon cannot pinpoint exactly lies brain damage, a lifetime of lost potential, unhappiness and death.

There was no part of the repair of Zachary's heart that Jonas had not done many, many times. The outcome hinged on preparation, timing, speed, accuracy and imponderables -- including the chance that Zachary's unique feature might somehow affect his odds.

Jonas sketched out the repair, determined the order of his work and kept a running total of how long it would take. (He prefers to do this the night before, explaining that "you do literally sleep on it; you run through the steps in your sleep.") There were 10 steps, the first seven requiring circulatory arrest.

"What is difficult about an operation like this is coordinating all those modules," Jonas said. "You do not have an unlimited amount of time." The right order of work is not always obvious. In this operation, for example, he sewed one end of a donated vessel as Step 6, and the other end as Step 10. The price of doing things in the wrong or inefficient order can be very high.

"You can paint yourself into a corner. You can literally get in a position where you can't get there anymore because you did things in the wrong sequence."

Planning, though, only goes so far. "Early primary repair" requires fast, near-perfect work against deadlines that nature imposes. Some of the work can be very difficult.

For example, to create a normal two-ventricle heart in Zachary, Jonas had to cut a slit in one wall of the tiny organ. Through it he poked a piece of tissue, which he then installed as an interior ceiling for the right ventricle. In effect, he sewed a floppy object the size of a quarter to something inside a space the size of a ping-pong ball through a hole a half-inch long. It took him about 30 minutes.

"I have literally done that thousands of times," he said by way of explaining it was not so unusual. Pushed, he added, "It wasn't easy the first thousand times."

He gives a lot of credit to people who tell him what to expect -- non-surgical specialists such as Donofrio.