Questions & Answers

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.

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