If infections at bay. Although, the child survived

If HLHS is not treated within the
first days of a child’s life, it can be fatal. With surgical intervention a
child has about a 70-90% survival rate. Some will have learning disabilities and
some physical limitations. If the child had a heart transplant, they will need
anti-rejection medication for the rest of their lives and may have to take
certain antibiotics to keep infections at bay. Although, the child survived having
a heart transplant or the norwood procedure done, there life long risks that come
with HLHS. Children who are born with HLHS have increased risk of pulmonary
embolisms, blood clotting disorders, and an increased risk of strokes. “With
more people surviving the staged surgeries, more information is now being
gathered about the long-term experiences of people born with HLHS” (Genetic and
Rare Diseases Information Center, 2017). Close monitoring of the child for the
rest of his/her life is mandatory. Females who were born with this heart defect,
if wanting to become a mother, she has a very good chance at being able to
carry 1 or more children. With all genetic diseases the mother/ father would
need to take into consideration that they could pass this gene onto their future
children. If a mother has a child already HLHS her subsequent child also have a
high risk of being born with HLHS.

The patient’s prognosis (expected
outcome)

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While HLHS is rare, there are
medical procedures to help. There is currently a handful of options available
for the treatment for HLHS. The Norwood procedure is a 3-step surgical process,
in the first step, which done shortly after birth, the cardiac surgeon will make
the right ventricle into the main ventricle while pumping blood to lungs and to
the body, while cutting off the pulmonary arteries and placing a shunt between
the main pulmonary and the aorta valve. The second step or the bi- directional
glenn operation is done about six months after the first step, the surgeon will
take out the shunt and connect the pulmonary artery right to the superior vena
cava.  This procedure is intended to help
with sending deoxygenated blood to the lungs while avoiding the ventricle. The third
step or the fontan operation, is done around 18-36 months after the second step.
This procedure is intended to connect the inferior vena cava to the pulmonary artery,
and to create a channel to the outside of the heart and to direct blood into
the pulmonary artery. After this procedure is finished deoxygenated blood
should be flowing through the lungs freely. Other procedures that can be done
is a hybrid procedure, when babies are too small to be able to do undergo the Norwood
procedure they can do the hybrid procedure which is where the surgeons will do
surgery and catheterization. In the procedure the surgeon will go in and make
the foramen oval wider and place a stent in the ductus arteriosus to keep if
from collapsing, next the surgeon will place band around pulmonary arteries to
control the blood flow to the lungs. With procedure it will allow smaller
babies to be able to grow big enough to get the Norwood procedure done. The
next available option, if there is significant damage to the heart and the
surgeons cannot repair it, is a heart transplant. “One of the most common
indications for infant heart transplantation had been hypoplastic left heart
syndrome, which occurs in about one in 6000 live births” (Emedicine, 2018).

Treatments available for the
disease:

When a child that is born with
HLHS, they can have no symptoms to having every symptom. Some of HLHS symptoms
include rapid and shallow breathing, bluish skin color, cold to the touch, fast
heart rate, poor breastfeeding, and a weak pulse. In some cases, a child was
born with HLHS and showed no symptoms, in others they had bluish coloration
around the heart and tongue. During the physical examination of the child a
doctor or the nurse that is assisting in the care of the care will notice the
symptoms, upon these findings they may also find that the child’s liver is
swollen, and the child is lethargic. “the pulse at various locations, wrist,
groin, and others, may be very weak” (MedLinePlus, 2017). In some cases, the
heart has an abnormal beat, or sounds abnormal. Babies that are born with HLHS
can also go into shock, due to the poor circulation in the heart. Some signs of
the child going into shock are dilated pupils, rapid but weak pulse, and
abnormal breathing.

 Signs and symptoms of the disease:

 

HLHS has certain set of
characteristics, the aortic and the mitral valve can be too small, narrow or
not formed at all, and narrowing of the aorta artery. In most cases the septum
is in intact. HLHS comes in different severity, ranging from small or
underdeveloped valves to valves that were not formed at all. With HLHS, it is
accompanied with other heart defects such as endocardial fibroelastosis or the
muscular layer of the heart chambers thickens, the arteries on the right side
of the heart with larger than normal due to over working, in many cases there
are also too small of blood vessels and arteries as well. The heart,
ventricles, and the chambers are in their right spots.

Pathophysiology of the disease:

The causes of HLHS are limited. It
could be genetics, or at the time of the child’s DNA being put together there
was a malfunction causing HLHS. Doctors have a limited knowledge on this type of
defect. What doctors do know, is that it is present when the heart starts forming
and beating in the early weeks of pregnancy, and chromosomal make-up could be
the culprit. “HLHS is the result of under-development of sections of the fetal
heart during the first eight weeks of pregnancy” (Boston Childrens Hospital,
2018). HLHS is also more common in baby boys than baby girls. HLHS and many
other congenital heart defects the direct cause it largely unknown.

Causes of the disease:

In every baby, in the womb and at
the time of birth, they are born with extra openings on the heart called the
patent ductus arteriosus and the patent foramen ovale. These help the babies
heart with getting the most of oxygenated blood to the brain. The patent ductus
arteriosus sits between the aorta and the pulmonary arties, redirecting the
blood flow, because babies in the womb do need to supply their lungs with
oxygenated blood yet. In a normal baby, the paten ductus arteriosus will close
shortly after birth. “However, when the baby is born, the blood must receive
oxygen in the lungs and this hole is supposed to close” (American Heart
Association, 2018). The Paten foramen oval is very similar to the paten ductus
arteriosus, except it is located in the septum of the heart. This opening in
the septum will allow the blood that the babies get from the placenta to enter
the heart and be able to travel to the right and the left side of the baby’s
heart, supplying the oxygenated blood from the placenta to the heart as quick
as possible. When these openings do not close after birth, the baby will be
born with a congenital heart defect. A rare defect is hypoplastic left heart
syndrome. This is when the patent ductus arteriosus and the patent foramen
ovale are too small or closed at birth the heart will overwork.  In some cases, the baby’s ventricles are
fused as one, or the septum is dotted with holes, causing improper circulation
at birth. HLHS can affect different organs in the child’s body such as the
lungs, liver, brain, kidneys and the vascular system. The hearts main role in
the body is to supply the body as whole with oxygenated blood, and to be able
to pump nutrients to other vital organs. When children are born with HLHS,
their vital organs are being depraved the nutrients and the oxygen that they
need to work properly.

General information about the
disease:

Every year in the United States
there are roughly 960 babies born with a rare congenital heart defect.
Hypoplastic Left Heart Syndrome or HLHS affects the babies heart causing the
left ventricle to be closed or be too small, same with the major arteries as
well. When the patent ductus arteriosus and the patent foramen ovale to close
causing the babies heart to over work, and the other major organs suffer from
not getting enough oxygenated blood.

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