Colorado Fetal Care Center 2017 Overview and Outcomes CIMFH_160133591_2017-07_CFCC 2017 Overview and Out | Page 24

Institute, who would take care of Harlowe
after birth.
PATIENT EXPERIENCE
Caring for a Rare Form of Heart
Block, Before and After Birth
Mariel Vigil had known for years she had
an autoimmune condition, though she
had always been able to easily manage
her symptoms. That’s not uncommon
for Sjogren’s syndrome, a disorder that
primarily affects moisture-producing
glands like tear ducts.
“It never even crossed my mind that there
would be complications with my pregnancy
because of it,” she says.
For most of her pregnancy, there weren’t.
The 20-week ultrasound went well. Mariel
found out she was having a girl. She’d name
her Harlowe.
But at a follow-up appointment a week
later, Harlowe’s heartbeat had dropped
to half its previous rate. Mariel’s
obstetrician immediately rushed her to
fetal cardiologist Bettina Cuneo, MD, at
Children’s Hospital Colorado.
“It happens in a small percentage of women,”
says Dr. Cuneo. Sjogren’s antibodies — agents
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programmed to attack substances the body
recognizes as threats — cross into the fetal
bloodstream and attack the electrical system
of the unborn baby’s heart.
“Because the top chambers of the heart can’t
communicate with the bottom chambers —
a condition called heart block — the fetal
heart rate can fall from 150 to 40 or 60 beats
per minute,” she says. “That’s one reason
more than 20 percent of these babies will die
before they’re born.”
Even among the 3 percent of women with
Sjrogen’s syndrome, heart block is rare. Still,
as one of the nation’s foremost experts on
fetal arrhythmias, Dr. Cuneo had experience
with the condition.
Dr. Cuneo immediately started Mariel on a
combination of therapies to bring down the
inflammation and speed Harlowe’s heart
rate. She also introduced Mariel to Kathryn
Collins, MD, director of the Arrhythmia
Center at the Children’s Colorado Heart
Meanwhile, Dr. Cuneo and her team saw
Mariel every week. “Our goal was to deliver
at 37 weeks, when her lungs would be
mature and she would be big enough for a
pacemaker,” says Dr. Cuneo. “Thirty-two
weeks was a big milestone, since by that
time all the major organs except the lungs
are functioning normally, but we hoped for
more time.”
By 33 weeks, though, despite the
medications, Harlowe’s heart rate had
dropped lower than ever. It was time.
Mariel delivered via cesarean section at the
Colorado Fetal Care Center at Children’s
Colorado.
Harlowe weighed 4 lbs. — just big enough
for pediatric cardiothoracic surgeon Max
Mitchell, MD, to implant a pacemaker.
Harlowe underwent the surgery at just four
hours old. All she had to do was go from the
delivery suite to the operating room, one
floor down. Dr. Collins and the intensive care
team took over from there.
“The transition went seamlessly,” says
Dr. Cuneo.
Stopping Sjrogen’s
In a situation like Mariel’s, if doctors can act
within the first 24 hours that the Sjogren’s
antibodies cross the placenta, they can quell
the inflammation and stop the damage.
The problem, Dr. Cuneo says, is that many
mothers don’t know they’re carrying the
antibodies, and even if they do, many
obstetricians don’t know the antibodies pose
a danger.
In a successful international study, Dr. Cuneo
and colleagues gave fetal heart monitors to
120 mothers with the Sjrogen’s syndrome
antibody and asked them to check their
baby’s heart rate twice a day at home. Two
mothers caught the inflammation in its
early stages, and both babies were born with
healthy hearts.
A simple blood test can identify Sjrogen’s
syndrome antibodies, and while the condition
is rare, Dr. Cuneo thinks the cost of universal
testing is worthwhile. With testing and
monitoring for mothers who test positive,
doctors could stop cases like Harlowe’s
before they start.
Journal of Perinatology (March 2017)
“We were in the neonatal intensive care unit
for 11 weeks after that,” says Mariel. “During
all that time we were mostly focused on
Harlowe’s breathing and eating and weight
gain. Looking back, we hardly thought about
her heart. And I think that shows how well it
all went.”
Now, at just over 2 years old, Harlowe is still
doing well. “She’s feisty,” Mariel says. “I don’t
think anyone would ever guess she had a
problem with her heart.”
Bettina Cuneo, MD, fetal cardiologist
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