Hospital Gives Little Hearts a Chance to Focus on Loving

Cherie Crane doesn’t need a cardiologist to tell her what her heart is for. Like most 3-year-olds, she knows it’s made to love people with.

But because Cherie is among the unlucky 2% of children born with heart defects, she’s had to learn earlier than most that the heart also is a fallible organ, and that when it isn’t working right she can’t jump off the planters in the garden of her North Hollywood home or roughhouse with her black Labrador, Killer.

Cherie ended up in the heart center at Childrens Hospital of Los Angeles when she was 3 days old. There, with the use of techniques such as cineangiography (high-speed motion pictures of the heart in action), pediatric cardiac specialist Dr. Masato Takahashi was able to diagnose the problem in Cherie’s heart, which was then no bigger than a walnut.

For adults, heart surgery often provides only temporary improvement in a heart deteriorating from years of wear and tear, said Takahashi, one of a team of six cardiac specialists at Childrens Hospital who work under the direction of Dr. Arno Hohn. However, children’s hearts--even those as seriously malformed as Cherie Crane’s--can often be restored so that they function perfectly.

“The majority of defects we see here are treatable,” said Takahashi, who is married to a high school biology teacher, Marcia. The couple have two teen-age daughters, Yuki and Rumi.

Spectacular Recoveries

“The children can be very ill when they come in, but once the problem is corrected, their recovery is spectacular,” he said. “If you do the right thing, the child will go back to school, be with normal kids, play sports, go to college. . . .”

Patty Crane, 30, thought there was something out of the ordinary about her daughter’s constant crying as a newborn. Her husband, 35-year-old rock musician Stephen Crane, was concerned only with getting mother and baby home from the hospital and tried not to think about the possibility that something was wrong. But instead of being discharged three days after her birth, Cherie was scheduled to see Takahashi at Childrens Hospital.

When Takahashi examined her, as he later recalled for the hospital’s in-house publication: “Her (Cherie’s) kidneys and lungs were failing, her liver was enlarged and she had an excess of fluid. The baby was in heart failure, her color was poor and her fingernails were blue. We did an EKG and echocardiogram and found she had a rather primitive form of endocardial cushion defect, also known as atrioventricular septal defect with single atrium. Her heart had not developed beyond that of a 30-day gestation embryo.”

Because she was too tiny and her condition too critical for surgery, Takahashi put Cherie on medication to control the heart failure. When she was 2 months old, her life was again in danger, and this time the surgery could not wait. A team of surgeons used a single Dacron patch to create the missing wall between the two upper chambers of Cherie’s heart, as well as a missing half of the wall between the lower chambers. The baby’s own tissue was used to construct a mitral valve.

‘Her Chances Were None’

But the reconstructed mitral valve continued to leak. At five months, Cherie became the youngest baby to receive an artificial mitral valve at Childrens Hospital. When doctors finally told Patty Crane she could take her daughter home after the second surgery, Crane said she was unprepared. She had never really expected that Cherie would live to come home.

“Ever since she was born her chances were none,” Patty Crane said. “They (doctors) prepared us that she would not make it.”

Cherie Crane’s heart, now about the size of small orange, recently began to exhibit symptoms resembling those of heart failure. The blond-haired girl was once again back in Dr. Takahashi’s office.

The heart center at Childrens is no longer an alien or frightening place for Patty and Stephen Crane. (Cherie is their only child.) They have come to have faith in what Patty Crane called the “remarkably wonderful people” here who repair children’s hearts.

“I just become more and more determined every time something happens to Cherie--I’m not going to let her go,” said Patty Crane, 30. “She’s incredibly strong, incredibly. She wouldn’t be here today if there weren’t some reason. . . .”

Pinpointing a heart defect and deciding what to do about it is only one element of Takahashi’s work. He also must consider the anxiety it causes a child when this most symbolic of all body parts breaks down.

“In one way or another, the disease is bound to cause some psychological problems,” Takahashi said. To alleviate the worries of an older child, Takahashi engages the heart patient in a frank and private discussion. Many children who have known an adult who died of a heart attack assume that their own bad heart is a death sentence, according to patient activity specialist Sheri Szeles.

Those children who must have their physical activity restricted due to heart problems are bound to feel different from their peers, Takahashi said.

Some Ignore Advice

“Some become isolated and angry, others become overachievers. Some teen-agers, in particular, ignore our advice and go ahead and play contact sports, ride motorcycles. . . .

“Communicating with a small child (about medical matters) is difficult,” Takahashi said. The doctor encourages toddlers such as Cherie to play with the take-apart heart model on his desk. Young patients are introduced to potentially traumatic procedures such as heart catheterization by patient activity specialists, such as Szeles, who familiarize them with the equipment to be used the day before their scheduled procedure. Szeles said that even babies seem to benefit from pre-exposure to the doctors’ masks and medical machinery.

Most heart irregularities referred to Takahashi are congenital. Up until the late ‘60s, rheumatic fever accounted for many cases of heart disease in children, but today the doctors at Childrens see only one or two rheumatic fever-related cases a year.

A disease discovered in the ‘70s, Kawasaki syndrome, is the newest culprit in childhood heart disease. Takahashi said the Los Angeles area this past winter experienced an outbreak of the disease, which causes an aneurysm, or ballooned area in the coronary artery. (Takahashi has just begun a six-month sabbatical at Children’s Hospital of Boston where he will be researching Kawasaki syndrome.)

Problems Have Many Sources

Occasionally, trauma plays a role in heart problems for children. For instance, several years ago Takahashi saw a 9-year-old patient who had had a soccer ball kicked point-blank into his chest, causing heart damage.

Still other children develop heart disease as a result of drugs used to treat other illnesses.

“Unfortunately, some of the most effective drugs for cancer treatment are toxic to the heart,” Takahashi said.

Typically, a patient is referred for a heart murmur detected by a pediatrician or school nurse. The heart lab takes a detailed history of the patient. An electrocardiogram reveals the rhythm of the heart and shows whether there is enlargement of a particular chamber. Abnormal blood flow to the lungs, an indication of a heart problem, can be detected on a chest X-ray.

About 20% of those children referred to the heart center turn out to have normal hearts, Takahashi said. The rest have some form of heart disease, but in a number of cases the murmur is “innocent” or functional and the child is returned home with instructions to the parents not to restrict their child’s play, Takahashi said.

Difficult Decisions

Once it is determined that a child has a more serious defect, the doctor must perform what Takahashi says constitutes the true art of medicine. He has to decide whether to let the defective heart go on beating as is, or to operate. Since tinkering with the heart involves risk, it can be a life-or-death choice.

To help in the decision, a more detailed portrait of the heart can be obtained through ultrasound. This exam, the echocardiogram, gives doctors a clear picture of all four valves and all four chambers of the heart.

If surgery is needed right away, Takahashi said he “nails down a diagnosis” by performing a cardiac catheterization. During this procedure, dye is injected in the heart through a catheter; the heart is then X-rayed on 35mm movie film in a process called cineangiography. The cineangiography equipment in the cardiac catheterization lab at Childrens Hospital is specially designed to capture the rapid beating of a child’s heart, which averages 120 beats a minute compared to an adult’s 60-80 beats per minute.

Chief technologist Gary Ritchie talked Cherie Crane through the painful part of her recent heart catheterization--the shot of Xylocaine in her leg where the doctor would insert a catheter.

Used to discomfort and hospital procedures, Cherie took the injection like a pro. She watched cartoons on a bedside monitor in the high-tech cardiac catheterization laboratory. In a few minutes, she was asleep.

Ritchie has observed that the procedure, although relatively painless, can be frightening for children or adolescents. When the doctor injects dye through the catheter and into the patient’s heart, the child sometimes can feel a sensation of heat radiating through his or her chest. The warmth may be accompanied by a peculiar taste in the mouth. If the catheter bumps a heart wall, Ritchie said the patient may notice a flutter in his or her chest.

“It can make some of the kids apprehensive,” he said.

‘Hypnotic’ Help

Ritchie got the idea of installing video equipment in the lab from watching the effect television has on his own children: “You can call their name (when the TV is on) and they won’t even hear you. It’s hypnotic.”

Takahashi threaded the catheter through Cherie’s femoral vein into her inferior vena cava, one of two large veins that bring blood into the heart. She awoke from her cartoon-induced slumber (patients also are given a sedative) just as the catheter reached her heart.

Ritchie sat behind a glass barrier monitoring Cherie’s heart rate. There is a very small possibility of rhythm disturbance or even perforation of the heart during a diagnostic heart catheterization, Takahashi said. When the catheter is used to perform a therapeutic procedure, such as a balloon angioplasty (in which a valve is stretched by enlarging a tiny balloon at the end of the catheter) the risk is slightly greater.

While Cherie alternated between sleep and cartoon watching, Takahashi took blood samples from the chambers of the girl’s heart. By comparing blood oxygen levels in the various chambers, he could determine that blood was not shunting between the left and right sides of her heart. (If it had been, a different defect would have been indicated.)

Evaluating the Pictures

The image of Cherie’s heart pulsed on screens visible to Takahashi and Ritchie. The picture showed two catheters in the heart. One entered through the vein to the right side of the heart; the arterial catheter entered the left side.

After about 45 minutes, the catheters were removed. The only remnants of the exam were two small holes in Cherie’s upper leg, which would not require stitches. Technician Annie Meade applied pressure to stop the bleeding while Cherie sipped ice water from a straw and watched “Alice in Wonderland.”

Takahashi was still wearing his blue scrub suit and clogs when he met with Cherie’s parents several minutes later.

“The problem is more complicated than I anticipated,” Takahashi said. He had performed the catheterization expecting to confirm that Cherie had outgrown the mitral heart valve implanted when she was 5 months old. She was indeed due for a new valve, the catheterization showed, but an additional obstruction had developed in the exit portion of the left ventricle.

May Be Last Surgery

Takahashi told the Cranes that Cherie would require surgery as soon as he could consult with a physician and arrive at the best way to solve the tricky problem. (The doctors later elected to postpone the operation for a few months, giving Cherie’s heart a chance to grow.)

Once again, dealing only with what Takahashi calls “shadows” (X-ray images), cardiac specialists had made a guess as to how long a small heart could safely function in its malformed state.

If the doctors judged correctly, the next open-heart surgery--Cherie’s third--may be the last one she’ll require, Takahashi said. Then her parents won’t have to worry any more when she dances energetically to rock videos on television, and she’ll have a good chance of living a normal life.