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Welcome to MUSC Childrens Hospital.

Hypoplastic Left Heart Syndrome (HLHS)


HLHS is a complex heart defect. The exact cause of HLHS is unknown. In HLHS,
the structures on the left side of the heart are small and underdeveloped. Because
the structures are underdeveloped, the left side of the heart is unable to pump
blood to the body. Without treatment, HLHS is fatal.
The following heart structures are small in HLHS:
mitral valve
left ventricle
aortic valve
aortic arch

Blood flow to the


body is supplied by
the ductus arteriosus

HLHS is not correctable but some


babies can be treated with a series
of heart surgeries. If you and your
babys doctor agree the surgeries
should be performed, they will
years of life. The surgeries do not

Aorta is reduced in
size (diameter)

Left ventricle
cavity is small

Mitral valve
closed or atretic

Instead, the surgeries re-route the


hearts circulation so that a child
can live with HLHS.

Norwood operation or Hybrid procedure. The second stage (Bidirectional Glenn

For consultation or referral, call MUSC Meduline at


843-876-CARD (2273) or toll-free at 1-800-922-5250.

Reprinted with permission


www.heart.org

Notes:

Notes:

Pediatric Cardiac Intensive Care Unit (PCICU)

Step-Down Unit (8D)

Your baby will spend several weeks in the PCICU after birth. The PCICU is a
14-bed intensive care unit (ICU) that specializes in treating infants and children
with heart problems. The PCICU medical team and staff are experts in caring
for children with heart defects.

When your child is medically stable and ready to leave the PCICU, he or
she will be moved to 8D. 8D is a specialty step-down unit for infants and
children with heart problems. 8D has 14 private rooms with showers and
in-room sleeping. Parents and caretakers are encouraged to stay and sleep
in the room with their baby on 8D. You will receive more details about 8D
once your baby is transferred there.

The PCICU is an open unit, which means family presence is welcome any time
of the day or night. There is a limit of 2 visitors at the bedside at any given time.
You will receive more details about family presence at the bedside and unit
guidelines when your baby arrives to the PCICU.

number for 8D is (843) 792-9280.

The telephone number for the PCICU is (843) 792-3806.

Medical Team
Your babys medical team will be large. The medical team will include many
doctors, nurses, therapists, dietitians, pharmacists and other staff members.

that trains and educates doctors, nurses, medical students and other health
medical knowledge and treatments, technology and patient care.
Your babys staff will change with each shift. There are two shifts per day. The
team will be written on a sign next to your babys bed. Please ask a staff member
if you are unsure who your babys medical team members are.
Physicians
experience and training.
Attending Physicians (MD or DO)
medical training. The attending physician supervises and leads the medical team.
Fellows (MD or DO)
Licensed medical doctors who have completed their general (e.g. pediatric)
The attending physicians supervise fellows. Pediatric cardiology fellows are
important members of the PCICU and 8D teams. There is always an on-call
pediatric cardiology fellow who sleeps in the hospital.

Residents (MD or DO)


Licensed medical doctors who have completed medical school and are
currently receiving their general (e.g. pediatric) training. Residents are

Nurses
There are many different roles of nurses.
Nurse Managers
Each unit has a nurse manager who is the supervisor. He or she oversees the
general operations, policies, and care environment for that unit. They are available

Medical Students
school as part of their early training to becoming a doctor. They do not have

Charge Nurses
designated shift. The charge nurse rotates with every shift.

Other Licensed Professional Providers


There are other licensed medical providers who write medical orders,
prescribe medications, and provide medical advice. These providers practice
medicine under the supervision of a physician.
Nurse Practitioners (PNP or FNP)
training. They are able to order tests, interpret lab results, write medical
orders, prescribe medications, and perform medical procedures with a
physicians supervision.

Registered Nurses (RN)


Nurses who provide the direct bedside care for patients. Nurses provide medical
care under the direction of physicians, nurse practitioners, and physician assistants.
The bedside nurse rotates with every shift.
Nurse Educators
Registered nurses whose primary role is to provide ongoing education and
training for the bedside nursing staff.
Nurse Case Managers
Registered nurses who help with planning for hospital discharge. They work
closely with social workers, home health services, and medical supply companies.

Physician Assistants (PA)


Licensed providers who practice medicine as part of a healthcare team under
a physicians supervision. They are able to order tests, interpret lab results,
write medical orders, prescribe medications, perform medical procedures,
and assist with surgeries.

For consultation or referral, call MUSC Meduline at


843-876-CARD (2273) or toll-free at 1-800-922-5250.

Other Medical Team Members


Anesthesia Team
pain management during procedures and surgeries.
Child Life Specialists
Specialists who help children and families cope with illness. They use play
and child friendly activities to prepare children for medical procedures.
Doctors of Pharmacy (PharmD)
medications. They also monitor medication side effects, allergies, and
drug interactions.
Dietitians
children.
Guest Services Representatives
Individuals who help families during their hospital stay. They staff the
PCICU waiting room, provide information about hotel discounts, provide
hospital tours, and general information about available resources.
Lactation Consultants
They help manage a variety of lactation concerns including: low milk supply,
engorgement and mastitis. They also can assist with breast pump rentals.

Occupational Therapists
skills and sensory skills. They provide therapeutic support for daily activities
including eating by mouth.
Physical Therapists
Providers who evaluate and help children with movement, muscle strength,
gait, posture and walking, running, jumping and balance.
Radiology Technicians
tests as directed by the health care team.
Respiratory Therapists
Providers who take care of a childs airway, including ventilation,
tracheostomy care, suctioning and breathing treatments.
Social Workers
Professionals who help families cope with being in the hospital. They
provide help with family or social challenges. They can provide short term

Speech and language pathologists


Providers who evaluate and help children with communication skills
including forming sounds and words. They also help with assessing a babys
oral and feeding skills after cardiac surgery.
Volunteers
Individuals who help children and families by providing support to the
nursing and child life staff.

For or
consultation
orMUSC
referral,Meduline
call MUSC
For consultation
referral, call
at Meduline
at 843-792-2200
or toll-free
at 1-800-922-5250.
843-876-CARD
(2273) or toll-free
at 1-800-922-5250.

What to Expect
Day of Delivery
Your babys delivery will be carefully planned.
On your delivery day, your medical team will include the following:

Transfer from Stabilization Unit to the PCICU


Once your baby arrives to the PCICU, the following routine studies
will be performed:

Obstetrician

Neonatologist

Neonatal nurse
Labor and delivery nurse
Respiratory therapist

he/she will be examined, measured, and weighed. Special catheters will be


placed in the umbilical vessels. The catheters will be used for monitoring,

Echocardiogram (a type of ultrasound) to evaluate your babys heart


Lab work
Chest X-Ray
EKG

Over the course of several days, your babys cardiac diagnosis will be
will be made.

comfortably at birth. However, if your baby has breathing trouble, he/


she may receive oxygen in their nose. Occasionally, a baby may need a
ventilator (breathing machine) to help them breathe. Once your baby is
stable, he/she is transferred to the PCICU.

Day of Surgery
The day before surgery, the surgeon will have a meeting with you to discuss
the operation, possible risks and complications, and to obtain your consent.
Your baby will go to the operating room very early on the day of surgery,
updates about the progress of surgery. You can expect your baby to be in
the operating room all morning and most of the afternoon.

During the weekday, there is a guest services representative available to


help with lodging, transportation and other needs. The waiting room has
free Internet access, computers, television, a private consultation room, and
a private lactation room for breastfeeding and pumping mothers. Please

When surgery is completed, your baby will return to the PCICU. The
The surgeon will discuss with you your babys surgery and condition. Once
your baby has been transferred from the operating room to the PCICU,
members can return to the bedside.

ventilator
multiple monitoring catheters
chest tubes
foley catheter
temporary pacing wires
continuous intravenous (IV) medications

Special Medical Issues

Swallowing problems

Infants with HLHS are at risk for special medical issues during their hospital
course. These medical issues include:

surgery. With time (weeks to months), the swallowing function usually

Vocal cord dysfunction


Swallowing problems
Feeding problems
Chylous effusion

Vocal cord dysfunction


Vocal cord dysfunction may occur in some HLHS babies. There is an
important nerve (recurrent laryngeal nerve) that can be stretched or injured
during the reconstruction of the aorta. The recurrent laryngeal nerve affects
vocal cord function and if the nerve becomes injured, your baby may have a

feeds into the stomach, your baby will have a special swallowing study with
the speech therapist. Both the occupational and speech therapists will work
with your baby to recover and improve swallowing function.
Feeding problems
Because HLHS babies are at risk for swallowing problems, aspiration, and
tiring with feeding by mouth, many babies require a gastrostomy tube
is a feeding tube that is placed directly into the stomach by a surgeon. If

most HLHS babies become better feeders as they grow older, bigger, and
off the ventilator, a pediatric otolaryngologist (ear, nose throat doctor) will
evaluate the vocal cord function using a small bedside scope and camera.
8

year of age.

Chylous effusions
Chylous effusions are a milky, high fat drainage from the chest tubes. It
occurs when the lymphatic system in the body has been damaged or disrupted. Heart surgery increases the risk for having chylous effusions. If your
baby develops chylous effusions after his/her surgery, the medical team
will have to change your babys feedings to either skimmed breast milk or a
special low fat formula.

after surgery. However, every baby is different and there are many things
tor, infection problems, wound healing problems, unstable cardiac function,
and heart rhythm problems.
When your baby goes to 8D, he/she may have a feeding tube, temporary
pacing wires, or chest tubes.

When is my baby ready to leave the PCICU?


You and the medical team will work together to determine when your baby
when the following goals have been achieved:

Breathing comfortably without a ventilator


Stable cardiac function
Weaned off all continuous IV medications
Removal of monitoring catheters
Tolerating feeding into the stomach

For consultation or referral, call MUSC Meduline at


843-876-CARD (2273) or toll-free at 1-800-922-5250.

Transfer to 8D
Transfer to 8D is an exciting time for families. 8D is a very important time
experience stressful.

trained to take care of children with heart defects. The 8D rooms have sofas
and private bathrooms. Parents are encouraged to sleep in the room with
their baby so they can learn their babys needs and continue developing
emotional bonds.
On 8D, an important focus for a HLHS baby will be learning to eat by
mouth. 8D is also when the transition from the hospital to home begins.
about how to provide the babys daily needs such as feeding, changing
diapers, giving medications and calming the baby when he or she is upset.
Parent education and teaching on the following topics will be given:

Holding and bathing your baby after heart surgery


Wound care
Giving heart medications to your baby
Warning signs that lung or heart function may be worsening

Feeding your baby by mouth

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For consultation or referral, call MUSC Meduline at


843-876-CARD (2273) or toll-free at 1-800-922-5250.

Interstage Monitoring Program

and second stage HLHS surgeries. During this time HLHS babies are very
fragile. They have a higher risk for poor growth, sickness, serious breathProgram is a special program to help your baby grow, prevent sudden unexpected death, and support you.

Peripherally Inserted Central


Catheter (PICC) Line

nurse practitioner and a dietitian.


in either the arm or leg. It can be used to deliver medications and also to draw
blood samples for lab work. The PICC line is placed by a nurse or doctor who
babys diagnosis and hospital course. You will leave the hospital with a home
pulse oximeter and an infant scale. Every week the nurse practitioner will call
you to answer any questions or concerns about your baby.

consent. Children are given sedation for the PICC line placement. The PICC
line is usually removed on the day of hospital discharge.

tioner will spend several days with you to give you training on the equipment and the special medical needs of your HLHS baby. The Interstage

Congenital Heart Defect Resources

takes care of your baby after you leave the hospital.

Heart Program of SC

Heart Catheterization

Cove Point Foundation


Emerson Rose Heart Foundation
Little Hearts

www.musckids.org/heart/
www.heart.org
www.pted.org
www.emersonroseheartfoundation.org
www.littlehearts.org
www.mendedlittlehearts.org

Facebook
tion is performed for many reasons including:
To measure the pressures in the heart and lungs.
To take pictures of the veins entering the heart and the arteries
leaving the heart.
To open up narrowed areas in the heart, veins, or arteries.

Palmetto Hearts (state support group for parents)


Sisters by heart (national support group for parents)
HLHSkids (family initiated support group)

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Cardiac Glossary
Arterial Blood Gas
levels of oxygen and carbon dioxide in the blood.
Arterial Line
that blood pressure can be monitored continuously.
Artery
Cardiopulmonary bypass
used in the operating room during heart surgery to support the
heart and lungs.

Extubation Removal of the breathing tube and ventilator.

Sternotomy The incision through the sternum.

Foley Catheter
drain urine.

Umbilical Lines - Catheters that are placed into the vein


and artery in the umbilical stump after birth. They are used to
deliver medications, draw lab work, and continuously monitor
blood pressure.

Gastrostomy tube
the stomach by a pediatric surgeon.
Informed Consent The process of obtaining written

Vapotherm
the nose and helps with breathing.
Ventilator
lung support through a breathing tube (endotracheal tube).

Catheterization Lab
Intravenous or IV
Intubation - Placement of a breathing tube and ventilator.

Chest Tube

Nasal Cannula
to give oxygen directly into the nose.

are described below. However, some children with HLHS


have special circumstances and do not follow the typical

Chest X-Ray
of the chest to examine the bones, heart, and lungs.

Nasogastric Tube
through the nose and into the stomach. It is used for feeding and
giving oral medications.

Stage I
The goals of Stage I surgery include:

Echocardiogram
the heart structure and function.

Oxygen Saturation - The measurement of oxygen that is


detected in the blood and delivered to the body.

Effusion
the lungs (pleural) or heart (pericardial).

Pacing Wires - Small temporary pacemaker wires that are


placed in the operating room during heart surgery.

ECG
heart rhythm.

Peripheral IV
vein to give medications.

Endotracheal tube
mouth and throat and connects the patient to a ventilator.

Right Atrial
Line
the heart by the surgeon during heart surgery to continuously
monitor the pressure in the right atrium.

Extracorporeal Membranous Oxygenation


heart-lung bypass machine that is used to support patients who
have sudden lung or cardiac failure.
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HLHS Stages of Surgery

Central Venous Line

Sternum The breastbone on the front of the chest. The ribs


are attached to the sternum.

and left atrium) so that blood can return freely from the lungs
into the right heart.

choice a baby. Your babys doctors will discuss with you which
Stage I surgery is recommended for your baby. Both the Hybrid
procedure and Norwood operation are usually performed

Notes:

Notes:

Norwood Operation
The Norwood operation requires cardiopulmonary
(heart-lung) bypass.

Stage III
chambers of the heart) is small, a balloon or stent may be
important because it allows oxygen rich blood from the lungs
to reach the right heart and eventually out to the body.

Fontan
The Fontan is the Stage III surgery. This surgery is usually
cardiopulmonary (heart-lung) bypass.

Stage II
Bidirectional Glenn
The Bidirectional Glenn is the Stage II surgery. This surgery is
Glenn surgery requires cardiopulmonary (heart-lung) bypass.

The Norwood operation includes the following:


the heart (right and left atria).
The Fontan operation includes the following:
then directly connected to the right pulmonary artery using a
conduit (a tube).

left pulmonary arteries. The disconnected main pulmonary

The Bidirectional Glenn includes the following:


Hybrid Procedure
The Hybrid procedure does not require cardiopulmonary (heart-

then directly connected to the right pulmonary artery. This

do the Hybrid procedure together. The Hybrid procedure


includes the following:

heart and go directly to the pulmonary artery. This reduces


the workload on the right heart.

right heart.

completely bypasses the right heart and drains directly into


the lungs. The right ventricle pumps blood exclusively to
the body.
______________________________________________

be removed.

be reconstructed.

Reprinted with permission


www.heart.org

We would like to thank the Emerson Rose Heart Foundation


for their continued support and generosity.

For consultation or referral, call MUSC


Meduline at 843-876-CARD (2273) or
toll-free at 1-800-922-5250.

M U S C k i d s . c o m C H A N G I N G W H AT S P O S S I B L E F O R C H I L D R E N .