Medical visionaries aim to develop the best pediatric heart center in the world

BY CHRISTOPHER VAUGHAN

Last April, 9-year-old Robert Klein made a life-saving journey from his home on Long Island, NY, to Packard Children's Hospital, where renowned cardiac surgeon Frank Hanley, M.D., repaired a serious heart defect, which had plagued Robert since birth.

WINTER 2002 - When Robert Klein was born nine years ago, his parents got terrible news: Their son had severe heart defects, which were so extensive as to be inoperable. Surgery might well be fatal.

With strong parental support and close medical supervision, Robert nevertheless survived. "First they told us that he probably wouldn't make it past two or three, and then they told us that we shouldn't bother thinking about schools because he would never make it to school age," says Robert's mother, Charlene Klein.

At only 43 inches tall and 36 pounds, Robert is now a fourthgrader and has thrived in many ways. Although he can't play sports, he studies piano and loves to create imaginative, elaborate Lego structures. Over the years Robert managed to stay relatively healthy, and his cardiac defects never became life threatening. Until he was 9 years old.

In Robert's ninth year, his cardiologist found that the already diminished flow of blood to his lungs had been further constricted. Robert's narrowing arteries could progressively cut blood flow to his lungs, putting his life in danger. "His cardiologist told us there was only one place we could go," says Irwin Klein, Robert's father. The physician told the Kleins that their best hope lay with Frank Hanley, M.D., at the Lucile Packard Children's Hospital at Stanford.

At nearly the same time that the Kleins were making arrangements to come to Packard, Beth Klemm gave birth to a daughter, Aubrey, at a hospital near her home in the Central Coast region of California. "Right away we knew there was something wrong," she says. "Her breathing was whimpy and wheezy." Tests indicated that the baby had heart problems that kept her from pumping enough blood through her lungs. Doctors immediately flew Aubrey on a small jet to the Bay Area, and transferred her to an ambulance for the trip to Packard.

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The next day, surgeon V.Mohan Reddy, M.D., whose specialty is performing surgery on the tiniest of infants, operated on 1-day-old Aubrey. Robert Klein and Aubrey Klemm are only two of the hundreds of children who come from all over the country to Packard Children's Hospital because there they find the greatest hope for treating their grave cardiovascular disorders.

In little more than a year, Packard has built on its existing expertise and gathered some of the nation's leading experts in pediatric cardiac surgery, cardiology, anesthesiology, and cardiovascular research into one place: the Children's Heart Center. The initial success of the ongoing Campaign for Lucile Packard Children's Hospital has allowed Packard to create a nucleus of medical visionaries who aim to develop the best pediatric heart center in the world -- a place to help the sickest of children and conduct research at the healing edge of medical science.

It Takes a Team

Although heroic surgeons like Hanley and Reddy often get the most attention for helping kids like Robert, the healing process requires the work of many pediatric heart specialists at Packard. Long before any child undergoes an operation, cardiologists will use a number of technologies to understand the scope of the problem and help surgeons decide how to address it. Before performing surgery, doctors often will look at an angiogram -- a picture of a child's working heart. A catheter is threaded into a vein in the leg and up to the heart. A dye that is opaque to X-rays is squirted into the bloodstream through the catheter, and X-ray movies show exactly where the blood flows. Cardiologists can also visualize the heart using an ultrasound technology called echocardiology, an area that Packard researchers have pioneered over recent decades.

In Robert's case, extensive angiograms had been done in New York, so Packard cardiologists did not need to put him through the tests again. After reviewing the boy's records, Hanley decided that he could perform a complex series of three surgeries. During the procedures, Hanley would combine fragments of the existing, insufficient circulatory system between Robert's heart and lungs to construct a more functional vascular network.

Robert's parents didn't tell their son immediately about the operation because they wanted him to concentrate on school. They worked with a child psychologist to decide on the best way to break the news to him, and as the time grew close for the trip to California they let him know they needed to talk to him about his most recent cardiology tests.

"They weren't so good, were they?" Robert asked. No, they weren't, his parents confirmed, and they wanted to take him out to California for some more tests.

After Robert heard this he asked: "Will I need to get an operation?" It's possible, his parents told him. "Okay," Robert said, and went on with what he was doing.

"His resilience and mature acceptance of all of this continues to amaze us," Irwin Klein says.

The First of Three

Once the Kleins were at Packard Children's Hospital, Hanley explained that the first procedure, called unifocalization, would have to be done right away. Luckily there was an opening available on the operating room schedule, and the surgery could proceed. Scheduling surgery is not as easy as it used to be. The influence and reputation of the Children's Heart Center have grown, and the staff must try to accommodate a greater influx of patients. Occasionally, when no other times are available, surgeons operate at midnight or on the weekends.

As he was wheeled to surgery, Robert showed remarkable calmness. The anesthesiologist put Robert to sleep and the operation began. Anesthesia always has to be carefully monitored, but in pediatric cardiac surgery there are special issues to address, such as differences in gas absorption through the lungs in children with cardiopulmonary disorders. Unifocalization, the kind of operation Robert underwent, is especially challenging for anesthesiologists because the patient must be kept under for many hours, and during some of that time oxygen and the anesthetic must be administered through only one lung. "That's why all our anesthesiologists are specially trained in both pediatric and cardiology anesthesiology," says Chandra Ramamoorthy, M.D., who heads pediatric cardiac anesthesia services at Packard. For nearly nine hours, Hanley and his assistants performed the demanding, dangerous task of consolidating existing arteries into a few branched pathways to bring sufficient blood to and from the lungs. Irwin and Charlene Klein had expected the surgery to last only five hours, so when the surgery finally ended they were extremely anxious. Hours of worry and frustration melted away, however, when Hanley came to tell them the good news: The surgery was successful.

"He is a great guy," Irwin Klein says of Hanley. "He takes as much time as you need to feel comfortable, and explains everything in as much detail as you want until you understand."

Back to Surgery

Although the operation had gone well, Robert's recovery in the days that followed didn't go as smoothly as everyone had hoped. Too much fluid continued to drain through the lines placed into his healing chest. Ultimately, the surgeons and cardiologists agreed to bring Robert back to the catheter lab to get more tests. An angiogram revealed that the operation had, in some ways, gone too well. Such a large volume of blood was now going to Robert's lungs that they were retaining fluid.

Once the tests were back, Hanley explained the situation to the Kleins and told them that he would like to get Robert back into surgery to correct the problem. If there were any consolation in returning Robert to surgery, Hanley told them, it was that he also could perform the second operation in the series, so that Robert would require only one more, months later. This time the operation and the recovery went more quickly than expected, and in just a few days Robert was on his way back home to New York.

Robert has not yet had the final stage of the unifocalization procedure, but he has noticed an improvement. Four months after the surgery, Charlene Klein recalls, she and Robert were heading for his piano lesson, going up a flight of steps that had always left him huffing and puffing. "When he got to the top steps he said, ‘Ma, last time we were here I couldn't breathe so good, but look at me now!'"

An Unexpected Trip

For kids like Robert Klein, Packard Hospital is a last hope after a long search, but for many more families, like the Klemms, the trip to Packard is quick and unexpected. "Aubrey was born at 1 a.m. and they had her to Packard by 1 p.m." says Beth Klemm.

As with Robert Klein, the first priority was to determine what had gone wrong. Packard cardiologists used echocardiology technology to get good images of her heart. The problem was quickly apparent. Aubrey had four defects collectively called a "tetralogy of Fallot," and a missing heart valve.

Now a healthy 4-month-old, Aubrey Klemm (held by her mom, Beth) had surgery at Packard when she was just one day old, to correct a missing heart valve and other defects.

The day after Aubrey arrived at Packard, Reddy operated. Using only his hands and magnifying goggles, Reddy was working with a heart the size of the tip of his thumb, cutting and sewing until he put in a new valve and closed a hole between her two ventricles. Even more than with older children, heart surgery with such tiny babies requires special training and experience. Anesthesia must be administered exactly, and surgeons have an even smaller margin for error than they would in larger children or adults. Earlier this year, Reddy had successfully a premature corrected a heart defect in baby weighing just over one pound -- the youngest and smallest baby in the world to undergo open-heart surgery.

After surgery, Aubrey continued to need the assistance of pediatric heart specialists. In the cardiac intensive care unit, her heart began to race, beating so fast that finally she suffered cardiac arrest and had to be resuscitated. Aubrey also suffered from high blood pressure, which could damage her lungs or other organs. Cardiologists were crucial in finding medications that could control these conditions. Nurses and physicians with training in pediatric intensive care were important for monitoring Aubrey's progress and finding signs of trouble.

After just over a month, Aubrey was released to go home, but she continues coming back to Packard so that her condition and her response to medications can be monitored. "She is on the road to recovery, and she still has a way to go," says Beth Klemm, "but we feel good about how she's been treated and the expertise we've found here at Packard."