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Pam and Jacob Pomrink
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 hris
Pomrink made the desperate phone call late on a Saturday night.
An internist at a suburban Philadelphia hospital, Chris was researching
heart disease—not for one of his patients, but for his unborn
son. Chris had learned that the baby, two months from being born,
had a life-threatening heart problem called hypoplastic
left heart syndrome (HLHS), a congenital
defect where the left ventricle (which pumps oxygenated blood
to the body) does not develop.
Survival with HLHS is dicey to begin with, but the unborn child
faced another hurdle: he did not have a second defect that actually
would have helped his heart route oxygenated blood to the body.
The missing defect, a hole in the atrial wall known as an ASD
(atrial septal defect), would have allowed blood to cross to
the right side of the heart where it could be pumped to the body
by the right ventricle; without an ASD, the baby’s chances
of survival were below 40 percent.
Cardiologists at their local hospital proposed waiting until birth
and then rushing the newborn from delivery to the operating room.
The risk was high, and for Chris and his wife Pam, the thought of
open heart surgery on their newborn was devastating. They felt there
must be another option.
Chris searched the Internet and finally pulled up an article
about a Children’s Hospital Boston patient with HLHS who was
born without an ASD. The Children’s approach was to rush the
baby to the cardiac catheterization lab at birth to create an ASD,
then give the baby several days to stabilize and get stronger, improving
the chances of surviving the big surgery ahead. Chris wanted to
talk to that child’s cardiologist, Mary
van der Velde, MD, an expert in echocardiography, who asked
him to send his son’s diagnostic information. In the back of
her mind, van der Velde was thinking that the unborn child might
be a candidate for a fetal procedure.
Three weeks later, Pam Pomrink, 31 weeks pregnant, and her husband
were in Boston consulting with Children’s cardiologists and
the high-risk obstetrical team at Brigham and Women’s Hospital
(BWH). Although Children’s cardiologists have performed 12
fetal
cardiac catheterization procedures in the last 18 months, what
Jim
Lock, MD, chief of Cardiology, van der Velde, and specialists
from the Advanced Fetal Care Center team were proposing had never
been attempted. The development of the baby’s heart was too
advanced for them to fix the HLHS in a fetal procedure, but Lock
and van der Velde believed that if they could create an ASD for
the baby before he was born, it would improve his lung development.
With the new pathway from the left to the right of his heart, his
condition would be less critical at birth.
 On
Sept. 27, Pam was in an operating room at BWH surrounded by a crowd
of high-risk obstetricians, fetal ultrasonographers, echocardiograhpers,
anesthesiologists, nurses and the interventional cardiology team,
led by Lock. Pam was placed under general anesthesia, which also
anesthetized her baby. Louise Wilkins-Haug, MD, director of Maternal
and Fetal Medicine at BWH, massaged Pam’s abdomen to rotate
and move the baby into a position that would allow Lock access to
the septum, the wall between the chambers in the baby’s tiny
heart where Lock would created the ASD. With an ultrasound to guide
her, Wilkins-Haug directed a needle into Pam’s abdomen, through
the uterus, and gently through the baby’s chest and into the
right atrium. Lock then took over to perforate the septum. With
the needle in place, he slid the cardiac catheter through needle
and inflated the balloon on the catheter to stretch the hole to
the proper size. The successful redirection of the blood flow was
immediately visible on the baby’s echocardiogram.
On Nov. 5, Jake Pomrink was born, weighing nearly seven pounds
and letting out a full cry as he was lifted from his mother’s
uterus in a cesarean section. Two days later, he underwent the first
of the three surgeries needed to repair his HLHS, but his parents
and doctors are confident that he went into that surgery, a stronger,
healthier baby due to his fetal procedure.
Now back in Bethlehem, Penn., Pam is settled into a routine with
Jake and his brother Luke, 2. “He’s just like a normal,
healthy baby,” says Pam. “If he didn’t have the surgical
scar, you would never know what we’ve been through.” Jake
still has two surgeries ahead of him, and the Pomrinks will bring
him to Children’s Hospital Boston, where he is a mini-celebrity
for his role as another Children’s “first.”—BA
Related links:
What
is a congenital heart defect?
What
is hypoplastic left heart syndrome?
What
is an atrial septal defect?
Fetal echocardiography
contributes to success in HLHS
Children’s,
BWH doctors perform a fetal first
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