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Clinical update
Fixing a tiny heart
Children's cardiologists and surgeons have been pioneering increasingly
complex heart procedures since performing the first repair of a
cardiac defect 65 years ago. But in the last two years they've taken
their work to a new level, and today they can correct some deadly
heart conditions before a child ever leaves the womb. In 2001, Children's
specialists teamed with other doctors to open the tiny, blocked
aorta of a 23-week fetus who would have developed 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) fails to develop. It was the first such procedure in the
world, but last fall they faced an even more complex case.
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Pam and Chris Pomrink of Bethlehem, Penn., learned that their unborn
child not only had HLHS, but also lacked a second defect that should
have helped his heart route oxygenated blood to the body. Without
a hole in the atrial wall known as an ASD (atrial septal defect),
which allows blood to cross to the right side of the heart to be pumped
to the body, the baby's chances of survival were below 40 percent.
The Pomrinks turned to the Web and learned that Children's had
successfully treated a child without an ASD. Despite the extensive
experience of the hospital's cardiologists, the Pomrink's unborn
baby's HLHS was too advanced for them to fix in utero. So doctors
embarked upon another world-first, creating an ASD before the child
was born.
Guided by ultrasound, doctors directed a needle into Pam's abdomen,
through her uterus, and gently into the baby's heart. Cardiologist-in-Chief
Jim Lock, MD, created a small perforation in the atrium and widened
it to the proper size with a tiny balloon catheter. The success
was visible immediately, as the new blood flow showed up on the
baby's echocardiogram.
Jake Pomrink was born Nov. 5, and two days later underwent the
first of the three surgeries to repair his HLHS. Because of his
fetal procedure, he survived and went into surgery a stronger, healthier
baby.
Second bionic ear gives depth to
sound
Sarah Novick, 5, of Windham, Maine, is one of just a few children
in the world with two cochlear implants, a device that can bring
a world of sound to people who are born deaf. The sound-capturing
device, which is surgically implanted behind a patient's ear,
uses an electrical lead to stimulate the auditory nerve, mimicking
the body's own sensory mechanism. Doctors and family hope that
Sarah, who got her first cochlear implant four years ago, will
now hear the world more naturally. The device should allow her
to make sense out of noisy environments such as her mainstream
classroom, and distinguish where individual sounds are coming
from. As the first Children's patient with two implants, Sarah's
story will be of much interest to future patient families facing
deafness. –CM
Vision of the future
Cataracts are often considered an adult problem, but the condition
affects one in 2,000 children as well. In kids, early intervention
is the key to preventing blindness and preserving cognitive development.
After pediatric cataracts are removed, artificial lenses are
so crucial to a child's recovery that they are typically implanted
surgically. But this procedure is widely avoided in children under
6 because of the challenges it poses. However, led by David
Hunter, MD, PhD, chair of Ophthalmology,
Children's experts are using advanced surgical techniques to implant
lenses and save the vision of patients as young as 6 months old.
Children's ophthalmologists use special software in the operating
room to predict a child's eye growth in future years. That way
they can choose the best possible lens so that it may never have
to be replaced even when the eye grows.
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