2011 cardiac stewardship report
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A REPORT ON OUR EFFORTS TO HELP AND HEAL: 2011
FOR CHILDREN’S
Hearts
giftofchildhood.org/supportcardiac
We are so pleased to share with you this collection of updates on the Cardiac Center at The Children’s Hospital
of Philadelphia. Your generous gifts are vital to funding the unique research, care and education detailed in
the following pages. So much of what makes CHOP the best place for children with heart conditions is only
possible because of our community of philanthropic supporters. Whether you give every month or gave a
one-time gift a year ago, you should be proud of your contribution.
We hope you will enjoy learning more about what your dollars have helped to accomplish. The Cardiac Center
offers a continuum of care for heart patients, starting with the Fetal Heart Program that diagnoses, evaluates
and manages congenital heart disease before birth. The continuum extends through the Philadelphia Adult
Congenital Heart Center, which is increasingly important as our patients live longer, healthier lives.
In between, the Cardiac Center helps children with everything from addressing elevated cholesterol levels,
to surgical correction of structural problems, to heart transplants. The follow-up for children who have had
surgery or other serious interventions involves monitoring their mental and emotional development as well
as their hearts, highlighting CHOP’s dedication to caring for the whole child.
In the following pages, you will read about a wide range of exciting research, innovative approaches to care and
even ways we help our young patients keep up their spirits. You enable all of these things to happen. Thank you
for your generosity to the Cardiac Center and the children and families we serve.
Sincerely, The Cardiac Center Executive Committee
dear friend,
THOMAS L. SPRAY, M.D.Chief, Division of Cardiothoracic Surgery
Alice Langdon Warner Endowed Chair
in Pediatric Cardiothoracic Surgery
ROBERT E. SHADDY, M.D.Chief, Division of Cardiology
Jennifer Terker Endowed Chair
in Pediatric Cardiology
SUSAN C. NICOLSON, M.D.Medical Director, Cardiac Center
Chief, Division of Cardiothoracic Anesthesia
Josephine J. Templeton Endowed Chair
in Pediatric Anesthesiology Clinical Education
THERESA R. SCHULTZ, M.B.A., R.N.Interim Director of Cardiac Nursing
MARY JO TIMLIN-HOAG R.N., B.S.N., M.P.H.Director, Cardiac Center Service Line
Kareim, 8, heart transplant patient
THANK YOUwith all my heart
Madeline had two heart surgeries before she was a year old. Her family wanted to give back.
GREAT GIFTSWhile Madeline McAndrews was still in the womb, her parents
came to The Children’s Hospital of Philadelphia and faced
frightening news.
After fetal echocardiography, their doctor, Jack Rychik, M.D.,
told them one side of their daughter’s heart was smaller than
expected. He would track the heart defect as the pregnancy
progressed to get a better idea of its severity.
Karen and Brian McAndrews went home knowing their baby
might be born with a heart with only one functioning pumping
chamber — a single ventricle. Karen went to her computer and
learned that single ventricle heart defects are among the most
severe, requiring at least three open heart surgeries. “We were so
scared,” she recalls. “It’s a very hard diagnosis, and we thought
that’s what we were facing.”
Looking back, the McAndrews feel hugely fortunate. The
Cardiac Center team helped them through the pregnancy, and
after Madeline was born on October 5, 2007, she was taken
by ambulance to CHOP.
GRATITUDE FOR GRANDDAUGHTER’S HEALTH
LEADS TO DAISY DAY GIFT
It was a happy ending — and beginning: Madeline’s heart
had grown as the pregnancy progressed, and both pumping
chambers (ventricles) were functioning. While she still had a
serious heart defect, atrio-ventricular canal, she would not need
the more radical surgeries required for single ventricle defects.
Madeline had heart surgery at 5 days and at 8 months. Today,
at 3, she is an energetic “girly-girl” who loves music and old
movies like “The Wizard of Oz.”
“She is just a little ball of fi re,” Mrs. McAndrews says. “Tons
of energy. We do dance class. We do gymnastics. And she has
to wear her red sparkly ‘Dorothy’ shoes every day.”
The family remains in the care of the Cardiac Center.
Dr. Rychik is Madeline’s primary cardiologist, and she visits
annually for checkups. “We have this wonderful little girl,”
her mother says, “and we feel indebted to CHOP.”
When Madeline’s parents and maternal grandfather, Bob
Klimasewski, heard Dr. Rychik was starting a program focused
on single ventricle patients, they knew they wanted to help
other families who faced these severe defects. The Klimasewski/
McAndrews family is the honorary chair sponsor of the 2011
Daisy Day Luncheon, which will benefi t the Single Ventricle
Survivorship Program.
“We are grateful to CHOP for the excellent care and
compassion showed to Madeline and our family during
extremely terrifying times. The outcome was beyond
expectations,” says Mr. Klimasewski. “We can’t do surgery,
advise on cardiac issues or prescribe meds. But we can assist
CHOP and, in particular, Dr. Rychik to advance his ability
to help others similarly affected like Madeline through a
meaningful contribution.”
Thirty years ago, patients with single ventricle defects didn’t
survive. Now, after surgery to alter their circulatory systems
and hearts, most survive. Many are living into their teens and
twenties. However, Dr. Rychik says, “We are realizing with
growing alarm that these patients are unlikely to have a normal
quality of life or a normal duration of life. They are experiencing
serious complications, including abnormal heart rhythms,
growth and puberty delays, liver disease, and gastrointestinal
and lung disorders.”
His program brings together physicians from different
specialties, including cardiology, pulmonology, gastroenterology,
hepatology and endocrinology, to address the complications.
“Survival alone is not adequate,” he says. “Our purpose is to
focus on this group and to acquire solutions so these children
can have long and healthy lives.” The team provides clinical
care to single ventricle patients of any age, conducts research,
and is developing screening and treatment protocols. Without
philanthropic support, their efforts wouldn’t be nearly as robust.
Big Hearts to Little Hearts, a nonprofi t group of grateful
families of Cardiac Center patients, is also a presenting
sponsor of the Daisy Day Luncheon. The group has supported
Dr. Rychik’s work in the past, including helping fund a study
that showed the drug sildenafi l improves heart function in
single ventricle patients.
The Daisy Day Luncheon 2011Park Hyatt Philadelphia at the Bellevue
Wednesday, April 27, 2011
4
Founded by the Auxiliary of
The Children’s Hospital of Philadelphia
in 1953, Daisy Day is the largest
annual volunteer fundraising
effort benefi ting Children’s
Hospital. Since its inception,
when volunteers sold daisies
on street corners throughout
the city, Daisy Day has grown
to include the prestigious Daisy Day
Luncheon and the Daisy Day Community
Campaign, which is supported by schools and
businesses across the region. To date, Daisy Day
has raised more than $12 million for patient-care
programs at Children’s Hospital.
More information at
DaisyDayLuncheon.org
6
With a Little Help: Women’s Committee Provides Grant to NeuroCardiac Care
Children with complex heart defects, particularly those who require
surgery in the fi rst months of life, are at higher risk for learning disabilities,
attention defi cit disorder and other academic, behavioral and emotional
problems than the general population. Thanks to a generous grant from the
Women’s Committee of The Children’s Hospital of Philadelphia, the
Cardiac Center’s NeuroCardiac Care Program (NCCP) will be able to help
those children and their parents get the best start and possibly avoid those
problems. The NCCP brings together specialists to provide screening and
long-term evaluation for cardiac patients who may be at risk. The unique
team includes cardiologists, neurologists, developmental pediatricians,
nurses, nutritionists, social workers, psychologists, and physical, speech
and occupational therapists. Beginning in infancy, patients visit at regular
intervals for evaluations of developmental milestones, such as crawling,
so problems may be addressed immediately.
The grant will be used for a variety of purposes, including developing
materials to educate families about ways to help their children recover
from open heart surgery, such as scar massage, tummy time and much
more. Massaging the scar promotes healing and keeps chest muscles
loose — so movement isn’t inhibited as the child grows. And tummy
play-time — to give babies a chance to work their arms and learn to
crawl — is even more essential for babies who have been hooked to
machines in intensive care and are used to being on their backs.
The Women’s Committee gift will also purchase fl oor mats, therapy balls
and other equipment, and software, questionnaires and screening tools
for neurodevelopmental issues. The NCCP will use the funds to create a
registry, tracking every patient’s progress. The database could serve as
a template for other institutions and eventually be combined with others
into a national registry to help researchers learn about and plan care for
the ever-growing group of cardiac survivors.
One of the most important uses of the Women’s Committee grant will be
a small fund for vouchers for meals, public transportation, gas, parking
and the other costs that can quickly become unmanageable for families
visiting CHOP frequently.
“It’s absolutely wonderful that philanthropy is helping to cover those costs
right now,” says Jean M. Carroll, R.N., M.S.N., NCCP program manager.
“In these economic times families are facing even greater challenges in
getting to their appointments. That people really care and have reached
out to help means the world.”
As cure rates for childhood cancers began climbing
in the 1970s, doctors soon realized survivors were
experiencing “late effects,” complications from their
disease and treatment.
Pediatric oncologists responded to ensure complications
were treated and even prevented. Helping lead the way
was The Children’s Hospital of Philadelphia, where the
fi rst program in the country to care for and track long-
term survivors of childhood cancers was founded in 1983.
Another survivorship challenge is happening right now,
in a different fi eld — pediatric cardiac care. Surgeons
began attempting new procedures in the 1970s,
but into the 1980s, most infants with serious heart
defects died. As the available treatments became more
sophisticated, survival rates began climbing signifi cantly
in the 1990s. Most babies with heart defects now
survive. Just as it did with cancer survivors, CHOP is
rising to meet the unique challenges of cardiac survivors.
“The Cardiac Center has built, and continues to build,
one of the most comprehensive cadres of programs for
cardiac survivors,” says Gil Wernovsky, M.D., director
of the NeuroCardiac Care Program at CHOP. “Our
responsibility to our patients doesn’t end with their
hospitalizations. As they grow, we’re still here for them.”
Families whose children have surgery or other
procedures at CHOP take comfort in knowing the
Cardiac Center team will be with them for years,
helping immediately if problems arise.
Children with complex heart defects, particularly
those severe enough to require surgery in the fi rst
months of life, have a higher incidence of academic,
behavioral and coordination problems than children
without complex heart defects. The NeuroCardiac
Care Program brings together a team of specialists
to provide screening and long-term evaluation for
patients who may be at risk. (See accompanying article.)
The Single Ventricle Survivorship Program, led by
Jack Rychik, M.D., is for patients born with hearts with
only one functioning pumping chamber (ventricle).
As these children are, for the fi rst time, living into their
teens and twenties, they’re experiencing a range of
complications. Rychik brings together a team from
endocrinology, gastroenterology, hepatology and other
specialties to focus on their care.
As patients outgrow pediatrics, it’s imperative that
they continue to have doctors who know their unique
physiology. The Philadelphia Adult Congenital
Heart Center, a partnership between CHOP and
Penn Medicine, provides that team of experts.
Yuli Y. Kim, M.D., who has special training in
treating adult survivors of congenital heart defects,
was recently hired as the new director of the Center.
“Right now there are more adult survivors of
congenital heart defects than there are children
under the age of 16 (with those defects),” says
Stephanie Fuller, M.D., a cardiothoracic surgeon
who operates on both pediatric and adult patients.
“An adult center sets a platform for having surgeons
and physicians who really are lifelong partners in
managing your disease. We don’t say goodbye as
soon as you turn 18. It’s a continuum of care from
specialists who are truly devoted to their patients.”
SURVIVAL IS NOT ENOUGH
A PRESSING CHALLENGE IN PEDIATRIC CARDIAC CARE
Abby, 18 months, has a complex
heart defect, double inlet left
ventricle, and is followed by
numerous teams at CHOP.
Years ago, when survival rates
for complex heart defects were
low, Abby’s grandma lost a
daughter to the same heart
defect. Today, the family rejoices
in hope for Abby’s long life.
8
For a pregnant woman, discovering that the baby she is
carrying has a heart defect is devastating. Stress can harm
the mother’s health. How might it affect the baby?
In a new study, researchers at the Fetal Heart Program at
The Children’s Hospital of Philadelphia plan to measure stress
levels after fetal diagnosis and their effect on blood flow in the
fetus, in the mother and between mother and fetus.
Prenatal diagnoses are helpful, because parents have time to
absorb the news and doctors can monitor the pregnancy and
plan to immediately stabilize the newborn, if necessary. But can
the stress of the diagnosis before birth disrupt fetal circulation,
potentially resulting in grave consequences for fetal development
and even the long-term health of the child?
The study will ask these difficult questions and may be a step
toward more carefully monitoring the mom in fetal care. The
researchers have applied for funds from the March of Dimes.
“By making a prenatal diagnosis, we think we’re helping, but is
it possible we are also causing some harm?” says Jack Rychik,
M.D., director of the Fetal Heart program and the Robert and
Dolores Harrington Endowed Chair in Pediatric Cardiology.
“This study will help us gain a better understanding of those
effects. Eventually it may also help us address the question
of how we can help modify stress. What we may find is that
when we make a prenatal diagnosis of a birth defect, we should
MOTHER’S WORRY, BABY’S HARM?
GIFT ADVANCES RESEARCH ON EFFECTS OF STRESS
CAUSED BY PRENATAL DIAGNOSES
also step up our efforts towards stress relief and perhaps refer
mothers to proven and effective stress-reduction programs such
as group counseling, or even yoga or massage therapy.”
Scientists have proved that some factors during pregnancy,
such as smoking, are harmful to the fetus. However, the effects
of maternal stress on fetal development and the child’s long-term
health is a relatively new field of study. Some research has
shown that maternal stress during pregnancy is associated with
early labor, low birth weight and, later, cognitive delays and
other developmental problems.
There has been surprisingly little research on stress in women with
high-risk pregnancies. The Fetal Heart Program study would be
one of the first of its kind in this important and emerging field.
A remarkable gift several years ago from Anne and John Bazik
made such research possible. Their gift funded two Fetal Heart
Program studies showing that women with a prenatal diagnosis
of a heart defect have higher rates of depression and anxiety
than women with a normal fetus. These results were crucial
preliminary data in applying for the March of Dimes funding
for the larger study.
“The Bazik gift clearly laid the foundation for a whole new field
of research for us,” Dr. Rychik says. “It’s a remarkable example
of how private gifts from generous and devoted individuals are
so vital to our efforts.”7
DECORATED FOR BRAVERYBeads of Courage is a program in which patients receive
a bead for any event or milestone during their stay: For every
echocardiogram, a glow-in-the-dark bead; for every needle
poke, a black bead; for every admission to the intensive care
unit, a glass star. When children show particular strength or
bravery, they receive an “act of courage” bead, a unique hand-
made glass bead donated by artists. There are dozens of beads
to mark every imaginable procedure. Depending on their
length of stay, participating kids collect anywhere from a few
dozen to a few thousand beads.
“The beads help to remind kids that this experience of being
in the hospital didn’t just happen to you — this is something
you did,” says Child Life specialist Sherry Polise, who runs the
program in the Cardiac Center. “You were brave and strong.
You got through this.”
The beads can also facilitate conversations, opening the door
for Child Life to discuss different procedures with a child. One
girl brought her beads to school for show and tell, helping her
classmates understand what she had gone through. The beads
can also give parents of an infant patient a memento to show
the child when he or she grows up.
Beads of Courage is a national non-profi t program used by
more than 60 children’s hospitals. Polise brought Beads of
Courage to CHOP in 2007, thanks to the fi nancial support of
the family and friends of Joanne and Peter Gori, who through
the establishment of the Bridgette Lauren Gori Child Life
Fund have supported the program in both cardiology and
oncology ever since.
The couple’s daughter, Bridgette, was a double lung transplant
recipient at CHOP. During her stay, Bridgette loved getting
stickers after different procedures. She collected three albums
full. “The stickers meant so much to her,” says Joanne. “I can
only imagine how much she would have loved beads.” While
Bridgette passed away in 2001 at the age of four, the Goris
continue to support the Child Life department, which gave
Bridgette so much joy.
Each year since 2003, Joanne and Peter, together with their
family, hold the JMR Charity Classic, a golf tournament to
commemorate and honor John M. Rodak, Joanne’s brother
who was killed in the World Trade Center attack the same week
that Bridgette passed away. John was Bridgette’s godfather, and
the event is another symbol of their connection. Most of the
funds raised through the tournament are donated to Bridgette’s
fund at CHOP. When children begin the Beads of Courage
program, they receive beads spelling out their name plus two
starter beads, one bearing Bridgette’s name, the other with
John’s. The two sit side-by-side.
“We wanted to donate to the Hospital in a way that directly
touched the kids’ lives,” says Joanne. “I know both Bridgette
and John would have been proud.”
Visitors to the Cardiac Center at The Children’s Hospital of Philadelphia might notice strands of colorful glass beads hanging from IV poles or sitting by the bedside. At first glance, they may seem like another cheerful decoration. But these beads are more than decorative. They tell the story of each child’s journey at CHOP.
This beautiful string of beads was created by a patient while she waited for a new heart. 10
In his fi rst years as a
pediatric cardiologist
in the late 1970s,
children born with
heart defects faced a
very diffi cult road.
Many did not make it.
“It drove all of us crazy
to stand by and see a
child die like that,” recalls
Robert J. Levy, M.D.
Over the next 30 years, a legion of scientist-physicians,
Dr. Levy among them, would rise to the many challenges
presented by their patients’ hearts. From the beginning, he
was fascinated by a particular problem: replacement valves for
children with faulty heart valves. One option is mechanical
(man-made) valves, which work well but are prone to blood
clots. Children with mechanical valves take anticoagulants
(“blood-thinners”) for their rest of their lives.
BETTER PHILANTHROPY SUSTAINS WORK OF RENOWNED RESEARCHER
Another option is valves from animals, most commonly pigs.
At the start of Levy’s career, these valves were working in adults,
with no anticoagulants required. But in children, because they
are experiencing rapid bone growth, the valves quickly became
covered in calcium. Dr. Levy saw waves of patients returning to
the hospital, often within months of surgery, severely ill with
calcifi ed valves.
Troubled by what he saw, Dr. Levy went to his lab and began
searching for answers. Today his research team at The Children’s
Hospital of Philadelphia is an international leader in the quest
for better replacement valves.
Meaningful research takes time. Refl ecting on the past decades,
Dr. Levy says, “It was really the private philanthropy that
kept us going. Because of that I was able to build my earliest
research into the major projects we have now.”
Two generous donors, families of Cardiac Center patients,
helped this progress. Richard and Ann Seidel have supported
research for more than a decade through the Erin Beatty
From a Seed, ForestsWhen a philanthropist makes a large donation to the Cardiac Center,
it is often only because of that donation that one of the many extra,
groundbreaking projects in research or care is possible. In those
cases, it is clear how the money given leads directly to expanded
services. But the hundreds of modest gifts that come into the Cardiac
Center every year also provide support for projects that could not
happen otherwise.
For several years, the Cardiac Center has been allocating money
from all of those donations made for the general support of the
Center to provide seed money to promising research projects that
would otherwise have no money to get started.
“The impact on my laboratory was quite signifi cant, as the preliminary
work that was collected with the help of the Cardiac Center was used
to secure a two-year grant from the March of Dimes Foundation,”
says Fraz Ahmed Ismat, M.D., who was provided funding for his study
of microRNA’s role in regulating the cardiac outfl ow tract. Dr. Ismat
notes that knowledge of microRNA’s role in disease is currently
advancing quickly, and that scientists suspect it plays a large part
in fetal development and congenital heart disease.
Cardiac Center donors are funding other work at the leading edge of
science. Michael Chorny, Ph.D., received funding for a study of using
magnetically guided nanoparticles to aid with the recovery of the
inner cell lining of an artery that has had a stent implanted.
“We developed biodegradable magnetic nanoparticles enabling genetic
cell modifi cation to improve their function and provide magnetically
Endowed Fund for Cardiology Research, named for their
granddaughter, a Cardiac Center patient who is now a healthy
teenager. And Adam and Christine Kibel, whose son was
treated at CHOP for an aortic valve defect, provide support
through the Kibel Fund for Cardiac Research.
Levy’s work is ongoing. Through the efforts of his team,
CHOP holds four valve-related patents. Tens of thousands of
patients have the St. Jude Medical Epic Valve®, created by
Levy. It is a porcine (pig) valve treated with a coating that slows
calcifi cation. Another of his valves, the St. Jude Medical
Trifecta Valve, is in clinical trials.
As so often happens, Levy’s research has branched off in
different directions.
Because heart valve disease is localized (in one place in the
body; not systemic), his focus has been on treatments targeting
one area. His team is now a leader in a burgeoning area of
medical research: nanoparticles as targeted delivery systems for
medicines and other therapeutics.
In a recent animal study, Dr. Levy’s team used uniform magnetic
fi elds, comparable but far lower in magnetic strength than those
present in MRI scanners, to drive iron-bearing nanoparticles to
metal stents, tiny devices used to keep narrow or injured veins
and arteries open. The nanoparticles slowly released medicine
and prevented blockages. This localized treatment could prove
helpful not only to heart patients, but also to the millions of
diabetics who suffer from poor circulation because of damaged
blood vessels.
Dr. Levy is also examining nanoparticles in another disease: the
childhood cancer neuroblastoma. Dr. Levy is collaborating with
Garrett Brodeur, M.D., an oncologist at CHOP, in a study of
nanoparticles as carriers of chemotherapy to tumors.
For Dr. Levy, the progress and the paths can be traced back to
the children who were so sick, and even died, when he was a
young doctor. Today, because of scientists like Dr. Levy, and their
supporters, the vast majority of children with heart defects live.
“It’s been revolutionary, what I’ve seen,” he says. “It’s miraculous.
We’re talking about quality of life now, instead of mortality.”
driven cell delivery to the injured arterial segment,” Chorny
explains. “Our studies showed that after the magnetic nanoparticle
treatment, the ability of cells to produce nitric oxide, an essential
element in the normal vessel homeostasis, was signifi cantly
improved. In addition, our magnetic delivery strategy enabled
effi cient cell guidance and prolonged residence at the stented
vessels, which was not achievable without the magnetic targeting.”
The results justifi ed additional studies as the technique moves
towards a possible place as a treatment for children in the Cardiac
Center in the future.
In all, from 2007 through 2009, 21 research studies were
supported by Cardiac Center donors. Through advancing basic and
clinical science in all aspects of cardiac care, donors like you are
building a better future for children with cardiac conditions.12
Nanoparticles with (bottom)and without (top) surface modifi cation
VALVES, BETTER LIVES
TEACHING ELEVATES CARE
NURSES’ PROJECTS EDUCATE COLLEAGUES AND FAMILIES
Whether it’s monitoring patients after surgery or keeping an
eye on them at home for signs of distress, caring for children
with cardiac problems often falls on two groups: nurses and
parents. Ensuring these important caregivers have all of the
knowledge, skills and resources they need has been the focus
of numerous initiatives this past year.
Cardiac Center nurses proposed, developed and carried out
six educational projects for parents and nurses last year, all
made possible by a donation from Big Hearts to Little Hearts,
a group of patient families and supporters who have raised
more than $650,000 for a variety of Cardiac Center programs
over the past seven years.
“The competence and the confidence of the nurse who is
providing the care at the bedside and beyond really heavily
relies upon education,” says Theresa Schultz, M.B.A., R.N.,
interim director of Cardiac Center nursing. “These are nurses
identifying what nurses’ needs are and then pulling together
all the expertise to meet that learning need.”
One project brought together nurses from CHOP and the
Hospital of the University of Pennsylvania to collaborate on
how best to transition childhood cardiac patients to adult
care. Another provided an opportunity for nurses to learn
about and practice procedures that are rarely needed, but are
vitally important.
Big Hearts to Little Hearts funding allowed CHOP nurses to
develop and host a pediatric cardiovascular nursing conference
in September, the contents of which were turned into online
training for nurses. As a separate project, Cardiac Center
nurses researched best practices in developmental care, in which
factors like skin-to-skin contact with a parent or different
lighting configurations can promote a child’s well-being.
While all six projects have shown positive results and will
be continued in some form in the future, some of the most
dramatic improvements seen so far were from the two
projects focused on family education. One study is testing the
Creating Opportunities for Parent Empowerment (COPE)
program with Cardiac Center families. This program educates
parents about how they can better monitor their children’s
health and participate in their care. Although the research is
not yet complete, families using COPE at the Cardiac Center
are responding positively.
In a separate project, two Cardiac Center nurses used the
Big Hearts to Little Hearts grant to produce a manual for
parents of children who are being evaluated for potential
heart transplants, helping parents distinguish normal events
from symptoms that should cause concern. Thanks to the
manual, the nurses are now receiving fewer worried phone
calls from parents.
Lynne Ramsay, president of Big Hearts to Little Hearts,
was impressed by the transplant manual and says that the
members of her organization are pleased with the effect
the nursing education projects are having.
“It is really good to know your child is in the very best
informed, capable hands,” Ramsay says, adding that she hopes
the community of donors supporting the Cardiac Center
continues to grow to provide that level of care. “Congenital
heart disease is so much more prevalent and common than
people realize. It is so grossly underfunded, and I don’t think
people are aware how many children are affected by it.”Hailee-Anne, 1 month, born with double inlet left ventricle, in the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit
14
“A FOUNDATION FOR OUR FAMILY”
DAUGHTER’S HEART TRANSPLANT INSPIRES GIFTS
Katrina Penney was only 24 hours old when her parents, Kati
and Jon, learned she would need a heart transplant. Katrina
had been diagnosed in utero with serious heart irregularities
— her parents had come to The Children’s Hospital of
Philadelphia from Bethesda, Md., specifically for the Cardiac
Center’s expertise — but Katrina’s doctors anticipated that
her problem could be corrected with surgery.
But now the doctors could see Katrina’s problem was even
more complicated than they’d realized.
Kati and Jon were shocked. In the months before Katrina’s
birth, CHOP had been mentally preparing her parents for
her surgery, showing them the Evelyn and Daniel M. Tabas
Cardiac Intensive Care Unit, the operating rooms, and the
step-down unit where she would go after surgery. The plan had
now changed: Katrina was on the list waiting for a transplant.
Katrina was fortunate — it took just nine weeks to find her
a donor heart. As Thomas L. Spray, M.D., took Katrina into
surgery, Kati and Jon sat in the lobby atrium. At 12:50 a.m.,
they heard a helicopter approach, the lights and blades casting
shadows onto the walls and floor. The donor heart, no bigger
than a walnut, had arrived.
The surgery was a success. Twelve days later, Katrina was able
to leave the Hospital. While she will remain on medication
for life to prevent rejection, Katrina is now a healthy second-
grader. She continues to return to CHOP for medical care,
but she and her sisters love stopping by even without a medical
appointment. “To them, it’s not just a building,” says Kati.
“It’s a great place to go and visit.”
Since Katrina’s surgery in 2003, the Penneys have made
it a priority to give back to other families at the Cardiac
Center, bringing thoughtful donations like food for the
family lounge, DVDs and videogames for the playroom,
and loads of toys every Christmas. With every donation,
the Penneys leave behind a letter of hope to let families
know that things do get better. Through their Penney Girls
Foundation, the family holds an annual golf tournament,
the proceeds of which have gone to support Child Life and
cardiac research at CHOP.
“We would never wish this experience on anyone, but the
result of what we’ve experienced we would never take back,”
says Kati. “It taught us so much and has been a foundation
for our family.” 1615 Katrina, 8, heart transplant patient
EVERY DAY, EXCELLENCE
THE CARDIAC CENTER’S EFFORTS TO ELEVATE
RESEARCH AND CARE NEVER STOP. HERE WE HIGHLIGHT
JUST A FEW OF THE MANY RECENT PROJECTS.
Echocardiography, ultrasound imaging of the heart, is one
of the most important and common tests in diagnosing and
monitoring heart problems in children.
To obtain ideal images with echocardiography (“echo”), the
patient must stay still. But children are not always known for
their ability to stay still. For many years, a drug called oral
chloral hydrate has been used for children who must be sedated
during echo. However, doctors became dissatisfi ed with chloral
hydrate due to unpredictable onset and offset of sedation and
concern about the safety of the drug.
A team of pediatric cardiothoracic anesthesiologists and
cardiologists at CHOP decided to examine an alternative.
In July 2007, the staff began offering sedated echo using face
mask anesthesia.
After comparing the charts of 507 patients, the team found
signifi cant advantages to face mask anesthesia. Since face mask
anesthesia takes effect more quickly than choral hydrate, and
usually wears off more quickly, visits for echo took much less
time. Also, the echo study was completed in all cases with face
mask anesthesia, as compared with a 6 percent failure rate with
chloral hydrate. The results of the study were published in the
Journal of the American Society of Echocardiographers in January.
Study co-author Susan C. Nicolson, M.D., chief of the Division
of Cardiothoracic Anesthesia, is the Josephine J. Templeton
Endowed Chair in Pediatric Anesthesiology Clinical Education.
By supporting outstanding doctors such as Nicolson, endowments
help elevate the level of care CHOP offers to children.
“Face mask anesthesia offers numerous advantages and is preferred
by both families and the medical team,” says Nicolson. “It has
become the standard for sedated echo at our institution. We
hope other institutions will examine our fi ndings and consider
making the same change.”
When a child is born with a severe heart defect, time is the
enemy. Babies can quickly get very sick, or even die, if they
don’t receive a medicine called prostaglandin or undergo
specialized treatment.
However, if doctors know about the heart defect ahead of
time and have a plan for care ready, babies are less likely
to go into shock or develop end-stage organ failure. Fetal
echocardiography (ultrasound of the heart) allows doctors to
diagnose many heart defects before birth. But predicting the
severity can be diffi cult.
A Cardiac Center team led by Michael D. Quartermain, M.D.,
is working to establish some very important standards by
using fetal echocardiography (“echo”) to predict the severity
of heart defects. Their fi ndings have appeared in the Journal
of the American Society of Echocardiography, with more
publications pending.
A team led by a cardiothoracic surgeon at CHOP has discovered,
for the fi rst time, common genetic variants that strongly raise
the risk of non-syndromic congenital heart disease (CHD).
Peter J. Gruber, M.D., Ph.D., an attending surgeon in the
Cardiac Center, and a team from Philadelphia and Michigan
found that variants in the ISL1 gene had strong associations
with CHD. The study appeared online in May in the journal
Public Library of Science One.
In Gruber’s previous research, he found that ISL1, on
chromosome 5, was crucial in regulating the development
of early cardiac progenitor cells. “In designing our study,
instead of assuming separate genes would govern each specifi c
defect, we formed the hypothesis that a common gene variant
operates early in the biological pathway of heart formation,
thus affecting multiple subtypes of congenital heart disease,”
said Gruber. “Based on previous research, we suspected that
ISL1 was a likely candidate.”
New Standard for Sedation for Echocardiography The Knowledge to Predict
The physiology of the heart changes over the course of the
pregnancy. Two fetuses with similar echo images at 18 weeks
gestation can be different at birth. One may be critically ill
because of a heart defect, while the other may have a milder
form. “We haven’t fully discovered all the markers that allow us
to reliably predict which fetuses will require immediate medical
attention after birth,” Dr. Quartermain says. “This work is
helping to push that effort forward.”
With better standards by which to predict severity, doctors can
make better plans. For example, they can decide if the baby
should be born in the Garbose Family Special Delivery Unit at
CHOP. Here, newborns can be in the care of a specially trained
cardiac care team within seconds.
“The more we know going into the delivery room, the better,”
Dr. Quartermain says. “We hope this research will help set
standards for prenatal monitoring and for delivery that will
result in better health for our patients.”
Gruber’s collaborators included his brother, Stephen B.
Gruber, M.D., Ph.D., a geneticist and epidemiologist at
the University of Michigan Medical School, and Hakon
Hakonarson, M.D., Ph.D., director of the Center for
Applied Genomics at CHOP.
Peter Gruber collected DNA samples from 300 CHOP
patients with CHD and 2,200 healthy children at the Center
for Applied Genomics. Hakonarson’s team did the initial
genotyping, looking for gene variants in the DNA of genes
in or near ISL1. The researchers found that CHD was more
common in children with certain variations in the gene.
Adding DNA from medical programs in Canada and the
Netherlands to the U.S. samples, the researchers studied genes
from a total of 1,344 children with CHD and 6,135 healthy
controls, and confi rmed in replication studies that variants in
ISL1 had strong associations with CHD.
CHOP-led Team Finds First Common Gene for Congenital Heart Disease
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34th Street and Civic Center Boulevard, Philadelphia, PA 19104-4399 • 267-426-5332
The Children’s Hospital of Philadelphia is one of only eight pediatric hospitals in the nation to be named to the prestigious U.S.News & World Report Best Children’s Hospitals Honor Roll for 2010-11.
The children you see throughout this report are our patients. It is for them, and for the many others in our care, that we relentlessly pursue the next advances in treatment and research. Your support is vital to the work of the Cardiac Center. To learn more about how you can help, please contact Robin Austin, associate vice president, at 267-426-5332 or austinr@email.chop.edu.
Thank you!
On the cover: Marisa, 7, born with heterotaxy syndrome, has been a patient of the Cardiac Center since birth. She is now part of the Single Ventricle Survivorship Program.
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