He Was In The Right Place. He Had A Bypass After An Aneurysm. Then Came A New Heart. A Lot Of Luck And Finesse Saves Boy, 3, In Heart Crisis

Posted: September 21, 1995

Three-year-old John Kendall Hauth was in his room at Children's Hospital of Philadelphia, about to go for lunch at McDonald's in the lobby, when his eyes suddenly rolled to one side and he began making a strange, high-pitched sound.

His mother tore into the hallway, screaming for help.

Rodney Finalle, a senior resident who just minutes before had examined Kendall, ran to the boy's bed and placed his stethoscope on his chest.

He heard nothing. Not a heartbeat. Not the sounds of breathing.

Scooping Kendall off the bed, he rushed to a specially equipped treatment room down the hall.

A major blood vessel feeding the boy's heart had burst.

Kendall would have died in minutes if he had been anywhere but inside one of the most sophisticated pediatric hospitals in the world. Saving his life would require the instantaneous response of a pediatric resuscitation team, coronary bypass surgery rarely done on children, a largely experimental heart- lung machine, and a heart transplant - all in less than five days.

Fortunately, Kendall was in such a hospital.

The rush to save his life would undoubtedly become part of the hospital's folklore, a reminder that medicine is a bit of science, a bit of art and often a whole lot of luck.

"If at any phase . . . something hadn't worked," said Gil Wernovsky, the medical director of the cardiac intensive-care unit, "he wouldn't be here right now."

*

Finalle reached the treatment room in a matter of seconds and placed an oxygen mask over Kendall's face as the alarm sounded on the hospital's public address system.

"Code Blue, 5 West," a voice said almost matter-of-factly. "Code Blue, 5 West." A patient had stopped breathing and help was needed on 5 West.

Within seconds, the treatment room was swarming with more than 20 doctors and nurses. After rushing down a flight of stairs in response to the call, Wernovsky found a little blond, blue-eyed boy who was "white as a sheet," with no heart rate, no blood pressure, no palpable pulse.

Kendall, of East Stroudsburg, Pa., was admitted by Children's Hospital two days earlier, Aug. 28, after developing a persistent fever and swollen lymph nodes in his neck.

His parents - Lynn, a homemaker, and John, a sports trainer at East Stroudsburg University - had taken him to Children's Hospital after doctors at Pocono Medical Center diagnosed possible Kawasaki disease, a perplexing illness named after the doctor in Japan who, in 1967, first observed the syndrome in children.

It annually strikes an estimated 9 out of 100,000 U.S. children, more often Asian and black children. The cause is unknown though the list of suspects includes streptococci bacteria, some sort of virus and even house dust mites.

The disease pushes the immune system into overdrive, provoking a severe inflammatory response. Fever can persist for one or two weeks. Other symptoms include conjunctivitis, dryness and cracking of the lips, swelling and reddening of the hands and feet, and a rash that looks like measles. Eventually, the tips of the fingers and toes peel.

Tests at Children's showed that Kendall had one of the most ominous complications of the disease, a giant aneurysm in the artery supplying blood to the muscle in the front and left side of his heart. The artery had ballooned to nearly 10 times its usual diameter.

Doctors gave him the standard drug treatment and monitored the aneurysm.

As the CPR efforts continued in the tiny treatment room, an ultrasound machine was rolled in to give doctors a view of the boy's heart. They assumed that a blood clot had formed in the aneurysm, causing a heart attack.

Instead, they were shocked to see the sac surrounding his heart filled with blood. The aneurysm had burst.

Delivering oxygen through a breathing tube, doctors restored Kendall's heartbeat with chest compressions and adrenalin. But they feared he needed immediate surgery.

Cardiothoracic surgeon J. William Gaynor, who was having lunch in his office two floors below, was summoned.

Confirming the need for surgery, Gaynor rushed to the operating suite and started scrubbing for the procedure while the operating room was made ready.

The boy's heart stopped again as he was being draped for surgery. This time the doctors couldn't get it pumping again. Afraid of cerebral damage from blood deprivation, anesthesiologist Susan Nicolson packed Kendall's head in ice, decreasing the brain's need for oxygenated blood.

Gaynor cut into the boy's chest and split the breastbone to reach his heart. The boy was hooked up to a heart-bypass machine to move oxygenated blood through his body.

Gaynor found an artery so badly damaged that it couldn't be repaired. The only hope was a coronary bypass, a procedure normally done on adults with clogged arteries.

Gaynor had never done such an operation on a child.

Assisted by Thomas L. Spray, the chief of cardiothoracic surgery, Gaynor took two five-inch pieces of blood vessel from Kendall's groin and chest and used them to reroute his circulation around the damaged artery.

The surgery took six hours. Kendall was taken to the intensive-care unit.

The 12 hours after surgery did not go well for Kendall.

Three times his heart started beating irregularly and had to be jolted with an electric shock to restore normal rhythm.

It was becoming clear that the heart was too badly damaged to keep him alive.

Doctors decided to take another extraordinary step. They hooked Kendall to an extracorporeal membrane oxygenator (ECMO), which is essentially an external artificial heart and lung. The machine was developed for use in newborns, but Children's Hospital had begun experimenting with it in older children.

The ECMO, however, could sustain life for only a short period.

Aug. 31, Kendall was placed on a nationwide list for a heart transplant.

As the Hauths kept vigil at their son's bedside, Wernovsky tapped into a medical bulletin board on the Internet called "PediHeart" and asked, "Has anyone done cardiac transplantation in the early phases of Kawasaki disease?"

Wernovsky received several computer replies, and the reports were not encouraging.

No one knew of any child with Kawasaki disease who had survived a ruptured aneurysm. Neither did anyone know of a case in which a child had received a heart transplant while still in the acute phase of Kawasaki disease.

Three days after Kendall was listed for a heart transplant, John and Lynn Hauth were summoned from the parents' lounge.

Walking into their son's room, they were greeted by Nancy D. Bridges, the medical director for heart and lung transplants.

A possible heart donor had been found, a child who had died in New York.

Pregnant with her third child, and exhausted from this ordeal with Kendall, Lynn Hauth was relieved by the news. But at the same time, she was overwhelmed by sadness.

"I was so sad," she recalled later. "You feel bad for a family who lost a child, but it's giving life to your child."

A cardiothoracic surgeon and two cardiac nurses flew to New York, retrieved the heart, and, by 11:30 that night, the transplant operation was under way.

The heart was a perfect fit and began beating moments after Gaynor sewed it into the child's chest.

Kendall's recuperation went without incident. It is now 18 days since surgery, and there are no signs of organ rejection. Kendall is scheduled to go home today.

Kendall will have to take immune-suppression drugs every day for the rest of his life to keep his heart from being rejected.

The long-term prognosis is unclear because doctors have limited experience with pediatric heart transplants. Bridges said the two-year survival rate for pediatric heart transplants is 80 percent.

Kendall was confused and angry when he woke up from surgery. The last thing he remembered, he was headed for lunch at McDonald's.

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