[Published in The Oregonian on August 16, 1999]

Six months into her pregnancy, 22-year-old Melissa Peterson learned that her unborn son had a life-threatening heart defect.

A specialist at Oregon Health Sciences University in Portland told the Roseburg woman that without an operation soon after birth, her baby boy would die.

The pediatric cardiologist offered three choices: forgo surgery and provide comfort care until death; register for a heart transplant operation and hope for a suitable donor; or send the baby soon after birth to a surgeon in San Francisco for a risky and complicated operation known as the Norwood procedure.

What the OHSU doctor neglected to say, according to Peterson, was that a rival group of cardiologists and heart surgeons across town at Legacy Emanuel Children’s Hospital regularly performs the Norwood procedure, attracting referrals from as far away as Alaska.

“I was very upset when I found out. I just think I should have been given the choice,” said Peterson, who is unmarried and supports herself on a minimum wage job at a shoe store.

Her story highlights how murky the process of getting a medical referral can be for patients, whose choices may be limited by behind-the-scenes competition among rival doctors and hospitals. And because of medical providers’ long-standing reluctance to release performance results, such as mortality and infection rates, referral choices are often based on best guesses and subjective judgments about reputation rather than objective evidence, consumer groups contend.

Yielding to pressure from consumers, some medical providers and health plans are starting to release more explicit quality data through the Internet and printed report cards. But experts such as health services researcher David Lansky think progress has been minimal.

“It remains a difficult problem for the typical person who just wants to know whether their hospital or their doctors are at the high end or the low end of the quality curve,” said Lansky, who is president of the Foundation for Accountability, a Portland-based nonprofit group that develops ways to measure and report on the quality of medical care.

Dr. Sidney Wolfe, director of Public Citizen’s Health Research Group, agrees. “What you have in most cases are self-serving ads saying, ‘We’re the best.’ The last thing they do is put up objective information,” Wolfe said.

The diagnosis Peterson faced made her predicament about as difficult as they get. The entire left side of her unborn baby’s heart, the side that pumps oxygenated blood to the body, was malformed beyond repair—a rare defect called hypoplastic left heart syndrome, which occurs less than once per 6,000 live births.

Rebuilding the heart

Before the early 1980s, virtually all babies born with the syndrome died within about a month of birth. In 1983, a surgeon named William Norwood, then at Boston Children’s Hospital, pioneered a complex, multistage operation to rebuild the heart so its right ventricle, or pumping chamber, takes over the entire job of circulating blood. The normal role of the right ventricle is to pump unoxygenated blood only to the lungs.

The first stage, named after Norwood, is extremely risky. Infants who undergo the procedure face a 40 percent to 50 percent chance of dying during and shortly after the operation at many hospitals, according to reports published in medical journals. The best programs that have published results claim mortality rates ranging from 23 percent to 32 percent.

Soon after Norwood’s accomplishment, surgeons at Loma Linda Medical Center in California showed that heart transplantation could save babies with hypoplastic left heart syndrome.

Peterson decided the risks of the Norwood approach were preferable to the helplessness of waiting for a suitable donor heart for Keyton, the name she chose for her baby boy.

But she worried about the plan outlined by doctors at OHSU. She was supposed to go to Portland to give birth at the university hospital, where pediatric cardiologists could assess Keyton and administer a hormone-like drug to stabilize his heart.

Then they would fly Keyton to the University of California at San Francisco, where a heart surgeon would perform the Norwood procedure.  Later, they would have to make two more trips to San Francisco to complete the three-stage surgery.

“I wasn’t comfortable with it at all,” Peterson said. She sought more information on the Internet, where she found a Web site devoted to parents of children with the same heart defect. In an e-mail exchange, one mother from Alaska asked Peterson why she wasn’t going to the hospital in Portland where children from Alaska had gone for the Norwood procedure.

Shocked, Peterson said she didn’t know about the other hospital. She suspected that rivalry between the two groups was the reason. In the medical business, the hazard of sending too many complicated cases to a competitor is that doing so might send a message to others that the rival group is more qualified.

Extreme differences of opinion

Dr. Mark Reller, chief of the division of pediatric cardiology at OHSU, said he and his colleagues prefer to send patients to San Francisco for the Norwood procedure, not to avoid sending them to a competitor, but because the operation is so high-risk that infants should go to a center that operates on large numbers of babies every year.

“It’s probably in our patients’ best interest to send them away,” Reller said. “Centers with bigger numbers tend to have better results.” But, he added: “It’s not a black-and-white scenario. It’s a judgment call.”

Heart specialists at Emanuel bristle at their competitor’s assessment.

“If our results on this operation were poor, I would understand, but our results are good,” said Dr. Marc Le Gras, a pediatric cardiologist who works at Emanuel.

Dr. Robert Dannenhoffer, a pediatrician from Roseburg who got involved in the case, found himself perplexed by the extreme differences in expert opinion.

“Is this best done at a handful of megacenters that do a huge number of them? Or is it best done closer to home, where travel brings less stress and financial strain on a family, but the experience is lesser? This is a very important question,” Dannenhoffer said. “The question is, who’s right?”

Dannenhoffer agrees with consumer groups that a lack of information to compare programs makes the question almost impossible to answer.

“If you are a parent, and you want to find out some logical measure between the two hospitals, I don’t think there would be any way for you to know,” he said. “It’s very hard for me as a physician to know.”

Doctors at Emanuel told Peterson that they do about five to 10 Norwood procedures a year and that 30 percent of the children don’t survive the operation. Peterson also found out that Dr. Albert Starr, a pre-eminent heart surgeon, leads the child surgery program at Emanuel, where he has performed Norwood procedures since 1991.

Le Gras said that from 1993 through 1997, Emanuel’s program performed the operation on 23 infants, seven of whom died during hospitalization, for a mortality rate of 30.4 percent. Those are the latest figures that the group has reported to the Pediatric Cardiac Care Consortium, a voluntary registry that is not open to the public and that not all cardiac programs report to.

The University of California at San Francisco declined to release its mortality rates for the Norwood procedure. Nor would the medical center say how many of the operations it does each year. Dr. Frank Hanley, chief of cardiothoracic surgery, said through a spokeswoman that he “prefers to speak to parents one-on-one about these statistics and what they mean.”

Reller, the OHSU cardiologist, couldn’t cite precise mortality figures for San Francisco’s program.

“This is not published,” he said. “I can tell you they are comparable to the best programs in the country, and the best are achieving 50 to 60 percent one-year survivals.”

He said he didn’t know the exact number of procedures at San Francisco, but he figures it is “at least 10-fold higher” than Emanuel.

Reller says survival statistics don’t tell the whole story and can be misleading to patients. Some centers, for instance, may treat patients who are sicker than average and more difficult to operate on, or statistics may not reflect recent changes in a program, such as the departure of a key surgeon.

Second-guessing

Peterson took stock of what the two groups had to say and decided to go to Emanuel in Portland. But she soon found she had to persuade her health plan to authorize the change, which dragged on so long, she wrote to her congressman, Rep. Peter DeFazio, D-Ore., who wrote a letter on her behalf.

“A lot of people were second-guessing me, even family,” Peterson said.  “It’s hard because it’s such a big decision. You’ve just got to choose and live with what you choose.”

Peterson persisted and gave birth to Keyton on Feb. 28 at Emanuel. He was a pink and healthy-looking, chubby-cheeked boy. Apparent good health is a characteristic of hypoplastic left heart syndrome that amplifies its cruel surprise when cases aren’t detected in the womb and newborns take a sudden turn for the worse.

Two days after Keyton’s birth, Starr performed the dicey Norwood operation, which involves  stopping the infant’s heart while the surgeon reroutes its main blood vessels and drastically alters its anatomy.  Keyton came through with no complications. Friday, he underwent the second-stage operation at Emanuel.

Peterson sat at Keyton’s bedside after the operation, holding his small hand and watching his bandaged chest rise and fall to the mechanical rhythm of a ventilator. “I look back and think, ‘Did I make the right decision?’ “ she said. “I’ve been wondering: ‘Is this still the right decision? Is he going to be OK?’ There is just no way of knowing.

“Whether it was the right decision or not, he’s doing well now. I’m glad that I did fight for what I thought was right. But I don’t think I should have had to fight as much as I did.”

– Joe Rojas-Burke