Information on HLHS

Hypoplastic Left Heart Syndrome (HLHS) is a rare and complex congenital heart disease.  It occurs when parts of the left side of the heart (mitral valve, left ventricle, aortic valve, and aorta) do not develop completely.  The left side of the heart is the main pumping chamber to deliver oxygenated blood to the body.  In HLHS the severely underdeveloped left side is insufficient to deliver blood to the body.  If left untreated, the condition is 100% fatal.  There is no issue during gestation as Beth is doing all the work now to supply oxygen to Shawn via the umbilical cord and his lungs are not currently functional in the womb.  Please see the image and video below to get a feel for the anatomy of a HLHS heart. 

A video from the Children's Hospital of Philadelphia:

 

  There is no known cause of HLHS.  It affects 1 in 10,000 babies and is more common in males than females.   It is sometimes (about 10%) accompanied with other developmental birth defects but that is not evident or is it a concern in our case.  Something in our favor!  HLHS is treated with a series of three staged and highly intensive open heart surgeries as listed below.

Stage 1 - Norwood procedure @ 1 week old

Stage 2 - Glenn procedure @ 4-6 months old

Stage 3 - Fontan procedure @ 2-4 years old

Now these procedures do not cure HLHS.  Shawn will have HLHS for the rest of his life.  These procedures only work to reconstruct the heart and circulatory system to manage with only one pumping chamber.  

If successful these kids live a somewhat normal life.  They go to school, play sports, hang out with friends, and even attend college someday.  That's not to say that after the three surgeries Shawn's story will end in roses and happy ending.  He will never be able to run a marathon, be a power lifter, or any other physically extensive things.  The road will be perilous and potential for complications, infections, and other developmental problems will always be around every corner.  He may need subsequent surgeries to clean up scar tissue or a pacemaker or even a full heart transplant are possible down the road.  These procedures were developed in the mid-80s so there is limited knowledge on the outlook of survivors as they mature into adulthood but we are anxious to find out as we continue this journey together. 

Some useful links:

Boston Children's Hospital: HLHS
Children's Hospital of Philidelphia: HLHS