RUNNING HEAD: Diagnosis Report #10 1

Diagnosis Report #10: Hypoplastic Left Heart Syndrome

Erin Kisch

Concordia University
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Hypoplastic Left Heart Syndrome is a congenital heart defect that can become critical

under certain circumstances. Because this condition cannot be completely cured, it certainly has

several psychosocial results. Coping strategies can be helpful for the diagnosed child but also for

the family.

Hypoplastic Left Heart Syndrome (HLHS) is a fairly rare condition which can be

detected even before a baby is born using fetal echocardiograms, an ultrasound for the heart

(Hypoplastic left heart syndrome). This condition occurs when the left ventricle and several other

structures of the heart are underdeveloped forcing the right side of the heart to pump blood to the

lungs and to the rest of the body. The extra work the heart is doing can make the infant short of

breath or have tinges of blue in the fingers and toes. Unfortunately, there is no known cause of

HLHS (Facts about hypoplastic left heart syndrome, 2016). While some medications can help

strengthen the heart muscle, surgery is required to prolong the prognosis of the infant. There is a

three-surgery process which the infant must undergo. First, surgeons will make new connections

between the left and right sides of the heart. Second, connections are made between the veins

coming from the upper body and the pulmonary artery so that oxygen-poor blood can go straight

to the lungs. Third, connections are made between the veins coming from the lower body and

pulmonary artery. In this way, oxygenated blood does not mix with oxygen-poor blood.

Although this series of open-heart surgeries, which is complete by the infant’s second or third

birthday, can sometimes fix the issue, a heart transplant may be needed later on.

As one could imagine, the serious interventions needed to correct HLHS can have lasting

psychosocial implications on the child and family. Although the child will be able to participate

in typical exercise and play after the completion of the surgeries, beforehand, the infant or

toddler can become fatigued easily. Even feeding can be difficult for newborns. Also, the long
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days spent in the hospital obtaining heart catheterizations and recovering from heart surgery can

slow development in children and increase stress in families. In addition, surgery will not cure

the child from HLHS; regular doctor visits and medications will still be necessary to keep an eye

on the function of their heart.

Thankfully, there are coping strategies which families can make use of to help them get

through these struggles. The most sensitive time during this long journey is during infancy. This

is when two of the three major surgeries are taking place. Piaget states that infants use their

senses to explore and learn about their environment. If an infant must lay flat and still after heart

surgery, their ability to explore is taken away. Parents can help stimulate their infant’s

development by reading stories, playing music, or holding up colorful toys for the infant to see.

Allowing babies to hold the finger of a parent or having the parent rub an arm or leg can be very

soothing for the infant as well.

The last surgery takes place during toddlerhood. Toddlers’ psychosocial development

centers around trying new things on their own and sustaining a feeling of autonomy as Erikson

describes in his theory. If a child is becoming fatigued quickly and often needed a parent’s

assistance to complete a task, the toddler can begin to doubt their own abilities. It may be helpful

for parents to find challenging quiet activities for the toddler to discover. Simple wood puzzles,

puzzles with various types of latches, coloring, and naming pictures of objects are all excellent

ways to exercise a toddler’s cognitive and fine motor develop and increase autonomy and self-

confidence.

By the time a child has reached school-age, the surgeries will be over, but frequent doctor

visits or heart catheterizations may still be occurring. Being taken out of school or spending

weekends to accommodate these visits may become frustrating. This is an age-appropriate time
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to explain more details about the child’s heart condition. Since Piaget lists school-aged children

as learning through concrete observations, learning should be through physical activities.

Looking at pictures of a healthy heart next to their heart, children can spot the differences. For a

medical play session, a child could cut out a paper heart, attach strings or pipe cleaners to

represent how the surgeries connected different blood vessels, place the heart inside the doll’s

chest, and close up the scar with a sticker, medical tape, or other method. These are ways to help

concrete thinkers understand their diagnosis.

The main focus of adolescents is independence. Erikson describes teens as going through

a time of discovering their own self image and identity. This identity is unique to them and

drives their wishes for the future. Having a heart disorder may feel like an obstacle that cannot be

passed. It is important to teach youth that, while their medical journey is an important part of

making them unique, it does not have to hold them back. Giving some control to the teen will

help them achieve this self-reliance. Allowing adolescents to take over the responsibility of

taking medications or asking their doctor questions can be ways that a teen takes ownership of

their diagnosis. By helping the teen transition from child to adult, parents are helping the teen

learn coping strategies.

Overall, although HLHS can be a fatal congenital heart defect, it can also be a diagnosis

that an adult grows up with and overcomes. Helping children learn about their diagnosis from an

early age can help them expand their knowledge at an age-appropriate pace.
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Reference

Facts about hypoplastic left heart syndrome. (2016). Retrieved from

https://www.cdc.gov/ncbddd/heartdefects/hlhs.html

Hypoplastic left heart syndrome. (n.d.). Retrieved from

https://www.mottchildren.org/conditions-treatments/ped-heart/conditions/hypoplastic-

left-heart-syndrome

Stories: living with hypoplastic left heart syndrome. (2017). Retrieved from

https://www.cdc.gov/ncbddd/birthdefects/stories/hlhs.html