What does Hypoplastic Left Heart Syndrome (HLHS) mean? It means the whole left side of the baby's heart didn't develop correctly. The left side is the side that pumps oxygen-rich blood to the rest of the body. It includes the aortic artery as well.
How rare is it? Primary Children's hospital treats all the
HLHS cases in the intermountain west--Utah, Idaho, Wyoming, Montana,
Nevada, and parts of Arizona. They have about 20 reported cases per
year of babies born with this defect. It is extremely rare.
How serious is it? This type of defect is one of the most
serious heart defects. It is life-threatening from the baby's first
breath. Even in the best case scenarios, this baby is not looking at a
very long life.
Can it be fixed? The short answer is no, it can't be fixed.
Without immediate intervention at birth the baby will die. The
interventions are risky and essentially just buy the baby time (1-18
years) before requiring a heart transplant.
What is the 1st treatment option? The first option is
called Comfort Care. After a full-term pregnancy and normal
labor/delivery, we take the baby home on hospice care. He/she will live
anywhere from 3-22 days, usually averaging around 2 weeks.
What is the 2nd treatment option? The second option is a
series of reconstructive open heart surgeries where the blood flow
through the body is reworked/rerouted through the lungs and the right
side of the heart (the normal side). There are three surgeries (at
birth, 6 mos, and 3 yrs). This treatment has only been performed for
the last 25 years, so there is limited data. It has about 50% to 60%
survival rate to 5 years old. It does not "fix" the heart permanently.
A heart transplant will always be needed to support life.
How hard is it to get a heart transplant? Hearts are
scarce. That's the cold hard truth. Babies and children with this
condition often have a difficult time qualifying for heart transplants
because their lungs and other organs suffer serious strain and possible
damage from overwork and lack of blood flow. They often need heart AND
lung transplants, which have low success rates and and super hard to
justify even IF the possibility arises.