Children's
fetal cardiac center
Fetal intervention established
as viable treatment for defects
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Children’s cardiologists and fetal care specialists performed
the first fetal cardiac intervention for a hypoplastic left
heart patient with an intact atrial septum. Doctors created
an atrial septal defect (fig. 1) to open bloodflow within
the heart (fig. 2), improving the infant’s outlook.
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Children’s Hospital Boston has been a leader in pediatric
cardiac care since 1938, when Robert Gross, MD, performed the
world’s first successful surgical repair of a congenital
cardiovascular defect, a patent ductus arteriosus. Today, that
tradition is upheld by Children’s cardiologists, led by Cardiologist-in-Chief
Jim
Lock, MD, and clinicians from the Advanced
Fetal Care Center (AFCC), led by pediatric and fetal surgeon
Rusty
Jennings, MD. Over the last 18 months, these teams have
established fetal cardiac intervention as a viable treatment for
fetuses with life-threatening cardiac defects.
In Sept. 2001, Children’s interventional cardiologists,
echocardiographers and fetal surgeons teamed with high-risk obstetricians
and fetal ultrasonographers from Boston’s Brigham and Women’s
Hospital (BWH) to perform the world’s first successful intervention
in a fetus with critical aortic stenosis and early signs of left
ventricular hypoplasia.
Based on experience with other fetuses, all indications were
that the baby, now named Jack, would go on to develop full blown
hypoplastic left heart syndrome (HLHS). At 23 weeks,
Jack’s mother underwent a percutaneous procedure
in which Jack’s severely narrowed aortic valve was opened
with a balloon catheter.
The needle that delivered the catheter was introduced through
the mother’s abdomen and into her uterus, and was then slipped
between Jack’s ribs and into his heart. The 3-mm balloon
was inflated, increasing blood flow through the valve and enabling
the ventricle to develop and grow. Ten weeks later when Jack was
born, he required no additional cardiac intervention, although
according to echocardiographer Wayne
Tworetzky, MD, who follows Jack in the cardiology clinic,
Jack may require another aortic valve dilation when he gets older.
Since March 2000, Children’s has performed 12 fetal cardiac
catheterizations to address a number of serious congenital heart
defects, including aortic valve stenosis and atresia, pulmonary
valve atresia with hypoplastic right heart syndrome, and HLHS
with an intact atrial septum. These advances complement Children’s
rapidly growing Advanced Fetal Care Center, one of only four comprehensive
fetal care centers in the United States and the only one in New
England.
The AFCC brings together specialists in fetal surgery, fetal
imaging and diagnosis, neonatal care, genetics, anesthesiology,
cardiology and cardiovascular surgery, otolaryngology, urology,
and neurology to provide services ranging from state-of-the-art
fetal diagnosis to fetal catheter interventions to fetal surgery
and exit-to-ECMO procedures.
In the fetal cardiac procedures performed to date, Children’s
has partnered with neighboring BWH, where a high-risk obstetrical
team headed by Louise
Wilkins-Haug, MD, director of Maternal and Fetal Medicine,
manages the mother’s care. During the fetal procedures Dr.
Wilkins-Haug’s role as the obstetrician includes rotating
the fetus into position—either by massaging the mother’s
abdomen or massaging the uterus directly—so that Children’s
cardiologists can access the tiny target in the fetus’s heart.
Under the guidance of Carol
Benson, MD, a specialist in obstetrical ultrasound, Dr.
Wilkins-Huag then places the needle through the uterus and into
the fetus’s chest and heart, where Children’s cardiologists
take over.
In Sept. 2002, Dr. Lock and the team performed a fetal cardiac
catheterization to create
an atrial septal defect (ASD) in a 31-week fetus with HLHS
(figures 1 and 2). While
HLHS diagnosis carries a mortality risk below 15 percent at Children’s,
babies born with HLHS and an intact septum—where there is
no route for pulmonary venous return to leave the left atrium—are
at the highest risk, with a survival rate around 40 percent. At
31 weeks, the development of the baby’s heart was too adced
for cardiologists to improve left ventricular size, but Lock and
Mary
van der Velde, MD, director of the Fetal Echocardiography
Program, believed an ASD would improve the baby’s lung development
during his remaining weeks in utero, making him less cyanotic
at birth and improving his long term outcome.
The baby, now named Jacob, was stable when he was born at 39
weeks and underwent a Norwood procedure at 2 days of age. His
surgery and recovery were uneventful, and his family returned
to their home outside Philadelphia just days later with their
healthy baby.
