Half a heart,twice the fight

1. Introduction

A. Whats hypoplastic left heart syndrome?

2. Symptoms and Diagnose

3. Disadvantages of surgeries

4. Three Surgeries

A. Norwood Surgery
B .Glenn Surgery
C. Fontan Surgery

5. Who suffers?

6. Conclusion

Alejandro Banegas
Kelsey Fender
English Research Paper
November 15, 2016
Half a heart,twice the fight

Hypoplastic left heart syndrome (HLHS) is a severe congenital (birth) heart defect that occurs
during the first 8 weeks or pregnancy, in which the left ventricle is underdeveloped. Hypoplastic
left heart syndrome is often fatal killing 95% of the child that born with it. Approximately 4,000
babies are born each year in the United States with HLHS, that equates to 1 out of 125 kids.
Were fortunate to live in a time where medical technology continuously advances. The
percentage of children born with HLHS living into adulthood is higher. Doctors have been able
to diagnose HLHS while the baby is still in the womb. In a normal heart, the left side has the job
of pumping oxygenated blood into the aorta. The large artery that carries blood to the body. In a
child with HLHS: the mitral valve,which separates the chambers of the heart, is too small or
completely closed. The left ventricle is very small or not develop at all and the aortic valve ,
which separates the left ventricle and the aorta, is too small or completely closed.

The syndrome can be diagnose hours or days before birth,with fetal echocardiogram
( ultrasound). Diagnosing the syndrome giving birth is crucial in to have a delivery plan ready
for the when baby is born. The following are symptoms of hypoplastic left heart syndrome that

may be presented at birth or several days later,: blue or purple tint to lips, skin and nail,difficulty
in breathing, difficulty feeding, lethargy (sleepy or unresponsive). Children with HLHS who do
not receive immediate medical care die within two weeks. Were fortunate, that today babies can
be treated with a series of operations that fix the function of the left side of the heart of the left
ventricle. Heart transplant is another choice for the infants with HLHS however there may be a
need to take life long.

There are many disadvantages from a person having HLHS including the daily restrictions of
physical activity due to decreased heart function and oxygenation levels being lowered. There
are several complicating issues that possible arise in these individuals including liver disease,
feeding problems with malnutrition requiring feeding tubes, protein losing enteropathy, plastic
bronchitis, and several other more rare complications related to HLHS. The children as
mentioned above have many social issues related to multiple hospitalizations and doctors
appointments, as well as limitations in playing with other children and playing sports. There have
also been associated with higher rate of anxiety and developmental disorders associated with
HLHS patients.

A child with hypoplastic left heart syndrome has to go through three open heart surgeries in the
matter of three years. HLHS has an extremely high mortality rate, 95%, leaving only a 5%
window for survival.

After birth, a child suffering with hypoplastic left heart syndrome will have to go through three
hard and pretty delicate surgeries, timed to coincide with the development of the lungs. The first
surgery they need to go through is the Norwood procedure. This surgery reroutes the blood flow

because of the problem with the left ventricle of the hearth. and is performed shortly after birth
(typically 10 days or less). The surgeon that performs the surgery turns the right ventricle the
non-defective ventricle-into the mainworkhouse ventricle. They connect the only working
ventricle so that it can pump blood to both lungs and the body. The main pulmonary artery and
the aorta are surgically connected with a shunt or tube, connecting a branch of the aorta to the
pulmonary artery to supply blood to the lungs.The aorta is a vital part of the heart: oxygenated
blood is supplied to all the parts of the body through the aorta. After the operation, the aorta
pumps a mixture of red and blue blood out to the lungs and throughout all the body. Since mixed
blood is pumped around the body the oxygen levels in the blood are lower than normal. As a
result, the baby has a dusty or blue lips and they turn more blue when the baby cries, but this
does not cause the infant pain. Most of the children are able to go home after ten days or two
weeks after the Norwood surgery is completed. They often go home on a couple of heart
medications.

The second operation, the Glenns surgery, is usually performed about six months after the
Norwood. Its performed to divert half of the blood to the lung when circulation through to the
lung no longer needs as much pressure from the right ventricle. On the Glenns surgery the shunt
to the pulmonary artery is disconnected and the right pulmonary artery is directly connected to
the superior vena cava. This is the vein that brings deoxygenated blood from the upper part of the
body to the heart. This process sends half of the deoxygenated blood directly to the lungs without
going through this the ventricle (www.pediatricct.surgery.ucsf.edu). After operation, the
deoxygenated blood from the upper part of the body goes to the lungs without passing through
the heart, (www.chop.edu). This makes it easier for the right ventricle because the blood flow it

can go directly without to the lungs without passing through the heart. The third surgery is not
performed until the HLHS patient in between 18 month and three years of age.

The third and last operation is called the Fontan procedure all three surgeries are named after
the doctor or surgeons that discovered them. In the Fontan surgery the doctors remove the patch
that was placed on the upper right chamber. They connect the pulmonary artery and the vessel
(the inferior vena cava) returning oxygen-poor-blood from the bottom part of the body to the
heart. This allows the rest of the blood coming back from the body to the blood to circulate back
again. A wall called the baffle is built in the upper right chamber. This guides the blue blood
coming from the lower part of body into the blood vessels that go to the lungs. Once this
procedure is complete, blood with low oxygen level and blood with high oxygen dont mix in the
heart and the infant is not longer going to look bluish or purplish( mottchildren.org). After
surgery, infants will return to ICU (intensive unit care) to be closely watched during recovery.
The chest of the patient may be left open, and covered with sterile drapes. The open chest
prevents compression of the heart by the rib cage. The chest is the closed several until the
swelling has gone down and the heart has a chance to adjust to the new circulation.

One of the worst part of this condition is not only impact on the individual with HLHS, but
also the impact on the families. There are many long hospitalizations from a week to a month to
months to years depending on the childs status and possible complications. In all these cases,
there are many times of uncertainty of possible outcomes related to their surgeries and risk of
possible death. The children spend a great deal of their early lives in hospital beds with IV lines
and tubes coming out of their bodies and spend significant time at doctor's appointments causing
fear and anxiety about health care and death.

A child with hypoplastic left heart syndrome has to go through three open heart surgeries in the
matter of three years. HLHS has an extremely high mortality rate, 95%, leaving only a 5%
window for survival.