Change of heart // Vessel switch first in
By REBECCA L. MARTIN
6/13/1990
Doctors
initially thought the heart of Lynell Penrose's
just-born baby girl was fine.
But hours later, Mrs. Penrose and her husband, Ernie, learned
the two large vessels of Rebecca's heart had been transposed
during her development.
The heart beat normally, but the transposed vessels kept
oxygen-poor blood circulating through her body, bypassing
the lungs and depriving her of needed oxygen.
Dr. Richard Ranne corrected the congenital defect one
week
later, when Rebecca underwent the first arterial switch
operation performed in Tulsa, St. Francis Hospital officials
said.
A child with transposed vessels can be kept alive until
about age 2 with temporary measures, but "without additional
treatment, will not survive," Ranne said.
Before Ranne came to
City
taken to
no other surgeon in
Rebecca went home eight days after the operation.
"We were told to treat her like any other two-week-old
baby," said Penrose, who lost his job as a restaurant
manager
in December and has taken other work to pay the bills.
The couple and their four children live in a duplex in
Arrow
burped on her mother's lap as the couple talked about the
ordeal.
Nine-year-old Stephen was at school, but Hilary, 5, and
Megan, 3, had the run of the household.
The first indication of a problem with Rebecca was a steady
blue tint to the baby's skin, which showed the tissue was
not receiving enough oxygen.
Mrs. Penrose, 29, was recovering from a Caesarean section
and saw Rebecca briefly before the child was whisked down
the hall to St. Francis'
She said that when her family doctor explained the seriousness
of the defect and the need for an operation, "I fell apart."
"I probably would have gotten more hysterical if I hadn't
been able to hold her before surgery," she said.
Penrose, 30, said not knowing what was wrong with the baby
was the hardest part for him.
"I was upset, but I didn't want to show it," he said,
because his wife was taking it so hard.
Rebecca was born at 9:16 a.m. April 26 and by 1 that afternoon,
the 9-pound, 7-ounce baby was undergoing catheterization
so doctors could get more specific information about her
heart anatomy, Ranne said.
At the same time as the catheterization, another procedure
was performed to assure Rebecca's initial survival, Ranne
said. He created a hole in the tissue that separates the
two chambers of the heart so oxygenated blood could mix
with the oxygen-poor blood and she would get some additional
oxygen to her tissues.
Ranne explained that usually the pulmonary artery,
which
sends blood to the lungs, and the aorta, which sends blood
to the body, spiral slightly so they end up attached to
the correct heart chambers.
That spiral does not occur in about 25 per 100,000 live
births, Ranne said.
The arterial switch operation, which has been perfected
in the last decade, involves separating the two vessels
from the heart, sliding the aorta behind the pulmonary artery
and then reattaching each one to the correct chamber.
Because the coronary arteries, which supply the heart muscle
with blood, must be taken off the pulmonary artery and reimplanted
in the correct vessel (the aorta), the pulmonary artery
must undergo major reconstruction, Ranne said.
He used tissue from the sac the heart sits in, called the
pericardium, to repair the pulmonary artery.
"It's our way of trying to create what she did not do
developmentally,"
Ranne said.
He learned the technique while on a fellowship at Boston
Children's Hospital, which specializes in congenital heart
defect surgery. He expects to average one arterial switch
operation a month at St. Francis.
Before the arterial switch operation was perfected, the
only other option was a procedure that did not cure the
condition, Ranne said.
Children normally had to wait until they were 1 year old
before it could be done, but up to 15 percent of those children
had major complications or died during the waiting period,
he said.
"The trend in modern cardiac surgery is to do the repairs
at a younger and younger age," Ranne said.
Ideally, the new operation is performed seven days after
birth. It requires a special operating technique called
hypothermic circulatory arrest.
For children weighing 8 kilograms (about 17 pounds) or less,
doctors slowly chill the child's body tissues until the
child reaches deep hypothermia - between 16 to 20 degrees
Celsius.
During this state of hypothermia, the body does not need
blood circulating to stay alive.
The mortality rate during the operation is 5 to 10 percent,
Ranne said.
He said that with the corrective surgery, Rebecca should
be able to lead a normal life.
Her parents said Rebecca already is acting like a typical
baby in at least one respect: appetite.
She had lost about a pound in the two weeks after her birth,
but she is now back up to 9 pounds.
Not surprising, Penrose said, "with all the
formula she
drinks."