Learning Major Surgery On Tiny Hearts -- Hospital Grapples With Consequences Of More Ambitious Cardiac Program

One of the most devastating birth defects known to modern medicine involves the complex plumbing of the heart. The defect is so severe, so disabling to a tiny newborn, that the infant dies within a month without immediate surgery.

The major operation to correct the defect, called hypoplastic left heart syndrome, is performed at more than 75 facilities around the country, including Children's Hospital & Medical Center in Seattle. The Norwood procedure, as the surgery is called, is one of a series of operations performed on these young patients.

Last year, nearly two-thirds of the babies who had the surgery - 13 of 20 - at Children's died, leaving some hospital staff members disheartened and angry.

Experts who reviewed Children's performance say the hospital's record over time is about average and reflective of any hospital attempting the relatively new surgery. The procedure also represents less than one-tenth of the heart operations performed at Children's.

Still, Children's overall 52 percent mortality rate for the Norwood is about double that of hospitals with considerable experience performing the surgery - a stark and painful indicator of its steep learning curve.

How Children's implemented the Norwood and other procedures, and the experiences it has undergone in doing so, are a new chapter in the life of the hospital. It's a story of how a major hospital acquires exciting new techniques, only to be confronted with

sometimes disappointing results, a sobering learning experience and forced introspection.

Alternative is transplant

Hypoplastic left heart syndrome is marked by several conditions: an underdeveloped left ventricle, the heart's major pumping chamber; a narrow or constricted aorta, the major artery taking blood to the body; and a very narrow or closed valve at the opening of the aorta. Blood flow to the body is inadequate - including to the coronary arteries, which supply the heart muscle with oxygenated blood.

About 1,500 to 2,000 babies are born with the syndrome in the United States each year. A few get heart transplants, but hearts are available for only about 300 such surgeries a year worldwide.

The other choice is the Norwood procedure and two follow-up operations. First performed in the early 1980s by Dr. William Norwood at Boston Children's Hospital, the procedure is the most difficult operation of the series - done on a heart the size of a small plum, an aorta as thick as pencil lead, and a pulmonary artery the diameter of a pencil.

The second surgery follows at 3 to 6 months, and the third is performed at age 1 1/2 to 2. Both help to decrease the blood volume and pressure in the atria, the top chambers of the restructured heart.

Because the procedure is so new, not much is known about how well children fare over many years. Physicians say that generally the children keep up with their peers in physical activity in the early years. As they get older, endurance may decrease, but they are still able to maintain a moderate activity level.

The Norwood procedure was first performed at Children's in the mid-1980s, on about 15 children. But all those infants died, and the hospital stopped doing the procedure.

A more ambitious program

Since 1991, Children's heart program has had a major overhaul.

Until then, surgeons repaired relatively simple defects with one-stage operations during infancy. The results usually were good and the death rate was low. Children with complex defects had surgery to relieve symptoms and buy time until a more complete repair could be done years later. The initial operations usually were successful, but uncorrected blood circulation problems often led to debilitation or death in late childhood or adolescence.

In 1991, Children's restructured its cardiac program to surgically correct the most difficult defects as early as possible in life. An area of the hospital was remodeled to become the new Heart Center, and Dr. Paul Herndon, a veteran Seattle cardiologist, was hired to direct it.

In 1993, Children's recruited Dr. Mark Lupinetti, a pediatric heart surgeon with an excellent reputation at the University of Michigan. He leads the surgical team in performing the more aggressive, early surgeries. Chances of death with this approach are higher, but experts say there are fewer re-operations, better quality of life and better odds for long-term survival.

For the first two years Lupinetti and his team performed the Norwoods - late 1993 through 1995 - the mortality rate for 24 procedures was 42 percent, better than the 55 percent rate of the Pediatric Cardiac Care Consortium, a group of 39 similar centers around the country.

But then came a downswing in 1996, when 20 of the center's three-year total of 48 Norwoods were performed. Thirteen died within 30 days of the surgery, meaning the surgical team had a mortality rate of 65 percent.

Deaths alarmed staff

Children's is a nationally respected specialty hospital, and its staffers are unaccustomed to seeing infants die during, or just after, surgery. So some were alarmed at what happened last year, and demanded immediate improvement.

"If we don't do something about this, we're part of it," said one staff member, who, with others, thinks the hospital didn't act fast enough to assess whether something was seriously wrong with the cardiac surgery program.

The staff rumblings came in different forms. Two anonymous letters were sent to the hospital's medical director, Dr. John Neff. Several anonymous letters and telephone calls, some of them angry and emotional, were received by The Seattle Times.

Reacting to these criticisms, the hospital conducted two reviews of the cardiac surgery program earlier than originally planned.

The three physicians from outside Children's who reviewed the program found no major problem that would have caused the high mortality rate in 1996. Nor did an internal review.

"It's not a great result, but the mortality is 100 percent if you let the children go without the surgery," said Dr. Thomas Spray, chief of cardiothoracic surgery at The Children's Hospital of Philadelphia and a veteran at performing the Norwood procedure. "To me, it means the center is evolving to do more complex procedures with more difficult patients."

For five complex heart surgeries - excluding the Norwood procedure - Children's has an overall mortality rate a little better than that of the Pediatric Cardiac Care Consortium. From January through October 1996, Children's performed 354 operations and lost 22 babies, for a 6.2 percent mortality rate. The rate for the consortium is 8.1 percent, excluding Norwoods. Officials defend record

Children's Hospital officials stress that the three-year, 52 percent mortality rate for the Norwood is roughly the same as the average 55 percent for the consortium. And they say the 65 percent death rate in 1996 occurred mainly because 11 of the 13 who died had other medical problems besides hypoplastic left heart syndrome, putting them at higher risk in the first place.

"There was no consistent pattern to death and no operative failure," said Neff. "The preoperative risks contributed" to the deaths.

The outside reviewers agreed. "These were patients in a higher risk spectrum," said Dr. Peter Laussen of Boston Children's Hospital.

Lupinetti and other staff members said the record for the entire three years, not just one year, must be considered. "Whenever you select a short period of time in considering something as complicated as this, you can get a false picture," the surgeon said.

Since the beginning of this year, two of four infants have survived the surgery.

"If I had thought something was not going well technically, I would have stopped the procedures," said Neff, the medical director. He said, however, that the surgical team "absolutely" needs to do better and that he expects improvement in time.

"We won't be satisfied with our Norwood results until we have 100 percent survival," said Neff.

Neff also expressed great concern about the distress among some staff members: "There's no question that we face the challenge of improving communication internally in order to build a more cohesive cardiology program."

Complaints about surgeon

At least two staff members have complained that Lupinetti works too slowly in the operating room. That could pose a danger. The surgery involves suspending the infant's blood circulation while the surgeon joins delicate cardiac vessels. If the time for that goes much beyond an hour, the child can suffer damage.

However, all three outside reviewers said they did not find Lupinetti's speed to be a factor in the deaths. They also said they were confident his speed would increase with experience.

"All the top surgeons have to go through a learning curve," said Boston's Laussen. "I think this one year (1996) . . . will not translate into every year. Experience and time will be very important factors."

At medical centers where surgical teams have had years of experience with the Norwood, the mortality rate for the surgery is about 20 percent to 30 percent. These include Boston Children's, Philadelphia Children's, Columbia University, the Cleveland Clinic and Texas Children's Hospital and the University of Michigan. Each center generally performs 20 to 40 of the procedures a year.

Surgeons at those hospitals agreed it takes time to get up to such levels of survival with the Norwood.

"It's not just the surgeon. It's the whole shebang. You need a good anesthesiologist, cardiologist, nurses and others," said Dr. Roger Mee, chief of pediatric and congenital heart surgery at the Cleveland Clinic.

Few choices for parents

That learning curve is just part of the difficult reality for parents of a child born with hypoplastic left heart syndrome. With little opportunity of obtaining a donor heart for a transplant, their choice is the Norwood procedure, with its high mortality rate and approximate $57,000 cost, or allowing the child to die.

"If there is any reasonable chance (for survival), even 5 percent, most families will take that chance," said Neff. "For a lot of families, it may be even less than 1 percent.

"It's awfully hard to lose a child. Families grieve forever. They have this question, `Did I do everything I could for my child?' If they think they didn't, then that will forever be a part of their grief."

Parents of most children with the defect say that the Norwood procedure and its follow-up surgeries are really their only choice.

"If there was any chance he could survive, it was up to us to give him that chance," said Port Orchard resident Debbie Stojack, whose 17-month-old son, James, is doing well after having a Norwood in September 1995. "Doctors don't know everything, and it may be that, down the road, there would be even better prospects for improving his life."

Edie Spears' son, Michael Odom, had a difficult time at first after he had the surgery in late November. His heart had trouble restarting, and he had to be returned temporarily to the heart-lung machine. His diaphragm was paralyzed for three weeks after the operation, and he had to have breathing assistance.

"It seemed that for every three steps forward, he would take a step back," said Spears of Salem, Ore.

But Michael is doing very well now. He's gaining weight. And he's behaving like a normal 3-month-old baby, said a relieved Spears.

"He would have died right away if we hadn't done this," said Spears. "Just letting him die was not a choice. If we could give him a chance, we wanted to give him a chance."

------------------------- Surgery for a small heart -------------------------

Children born with hypoplastic left heart syndrome have an underdeveloped heart chamber, artery valve and major artery, preventing adequate blood flow to the body and heart muscle. The child dies without surgery.

Norwood procedure

The major operation to correct the defect, this procedure joins the main pulmonary artery (pencil-thick), which normally takes blood to the lungs, to the underdeveloped aorta (pencil-lead thick), which takes blood to the body. The new artery is enlarged with a tissue patch. An artificial blood vessel, or shunt, takes some blood from the aorta into the left and right pulmonary arteries. The wall between the atria, the upper chambers, is removed.

----------------------------------------- Norwood procedures at Children's Hospital -----------------------------------------

Figures for deaths consist of those that occur within 30 days of the surgery. Beyond that time, deaths are not suspected to have any link to the operation itself.

Year Operations Deaths Mortality

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1993 4 2 50%

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1994 12 7 58%

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1995 8 1 13%

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1996 20 13 65%

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1997 4 2 50%

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Total 48 25 52%

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