Mentor Interview

Half a heart,twice the battle

1.What do you know about Hypoplastic left heart syndrome?
Hypoplastic Left Heart Syndrome is a congenital heart defect involving the left side of the heart
being undeveloped and essentially non-functional. While the fetus is in utero it survives off of
the function of the PDA (patent ductus arteriosus) which allows for blood to bypass the lungs.
After the infant is born within three days the PDA closes and with no functional left side of their
heart, the infant will drop its oxygen saturation levels leading to eventual death of the child. Most
of these cases are found through ultrasound during the approximate 22 week testing when the
fetus is looked at for heart anatomy and function, although in some cases the diagnosis is missed
which can lead to bad outcomes around the time of delivery or shortly thereafter. In most states
in the USA, the infant is put on pulse oximetry until they are released home from the hospital
which helps identify cases missed in utero. There are a series of three palliative/corrective
surgeries for these infants to move forward in their lives. The first surgery is called the Norwood
which is typically performed with the first two weeks of life where the pulmonary artery which
sends blood to the lungs for oxygenation gets converted into the aorta which is the vessel that
pumps blood to the entire body. There is a shunt put in place from the right ventricle, which is
now essentially been converted into a left ventricle, to allow for the blood to be oxygenated
without the need of the pulmonary artery. The infants live at an oxygen saturation of 75%-85%
(with normal being 100%) until their second surgery. The second surgery called the Glenn is
performed at approximately 4 months old which converts the SVC (superior vena cava) directly
into the lungs allowing for passive flow (not needing the heart to pump this oxygenated blood to
the lungs). After this surgery, the infants are typically put on a combination of medications
including diuretics to keep the excess fluid out of the lungs and extremities, ACE inhibitors to
help with heart function, and baby Aspirin to prevent clotting. The third surgery is called the
Fontan which takes the IVC (inferior vena cava) and connects it directly to the lungs bypassing
the heart. The outcome of these three surgeries is now the right ventricle is setup like a left
ventricle pumping the blood to the body and all the return blood (unoxygenated) directly
passively flows through the lungs taking increased workload off the heart.

Do you know someone with this syndrome? If you please list name and age below.
Hughes Patrick Chisholm- my son. Age: 5yo dob. 5/1/2011

What do you think is the worst part of having this condition?

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.

Can you think of any disadvantages of having HLHS?

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. These 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 higher rate of anxiety and developmental disorders associated with HLHS
patients.

Whats something you think I should talk about on my paper?

If I were you, I would describe the diagnosis with some possible visual aids to help describe not
only the disorder, but also what the three surgeries are trying to accomplish. Then, it would be
cool to talk about Hughes story specifically (Carrie would be a great person to contact to get
more info about his story as she has journaled many of the details through the years since he has
been born). In summary, you could bring back to HLHS as a whole and how more awareness is
needed for the this disorder and all other CHD (congenital heart defects) with so many people
being born with them and with modern medicine this population is living longer better lives with
continued research needed.

Do you think there is a lack of awareness to the HLHS syndrome?

Absolutely there is a big issue with lack of awareness for HLHS syndrome as well as other
CHD diseases. There many organizations involved with bringing more awareness to HLHS
(Carrie could give you a good list) and various children's hospitals. Social media has played a
bigger role as well with parents, families, and friends playing a role in advocacy.