When any of my children become ill I always take to the internet and learn all I can.  The night we found out about Silas’ diagnosis I could not wait to have a quiet moment to research what HLHS really meant.  I soon found out that it was not something I would learn and be able to comprehend in a matter of an hour or even two.  I am still trying to get down the anatomy of the heart, what each part does, and how this condition will impact our little boy.  I thought I would write a brief description of what HLHS is for those who want to understand.

One thousand babies are born each year with HLHS and 2/3 of those babies are boys.

Hypoplastic Left Heart Syndromee is a severe congenital heart defect in which the left side of the heart is underdeveloped or not at all.  In a normal heart, the heart’s left side has the job of pumping oxygenated blood into the aorta, the large artery that carries blood to the body.  In a child with HLHS:

• The mitral valve, which separates the two left chambers of the heart, is too small or completely closed(atretic)
• The left ventricle(the lower, pumping chamber) is very small
• The aortic valve, which separates the left ventricle and the aorta, is too small or completely closed(atretic)

In addition to the most common form of HLHS there are a number of complex conditions with variations in the structures described above.

Treatment for HLHS varies depending on each childs exact diagnosis and how that child’s body reacts to each procedure.  Typically a child with HLHS will undergo 3 reconstructive open heart surgeries to redirect the  oxygen-rich blood(red) and oxygen-poor blood(blue).  After these surgeries:

• The right side of the heart will do what is usually the job of the left side of the heart-pumping oxygenated blood to the body.
• The deoxygenated blood will flow from the veins to the lungs without passing through the heart.

The series of three reconstructive operations to repair HLHS-The Norwood, Glenn, and Fontan procedures-is known as staged reconstruction. http://www.chop.edu/conditions

The outlook for children with HLHS has improved dramatically. While HLHS was once uniformly fatal, our experience so far indicates that the majority of children will now reach adulthood. Some children will need other catheter-based or surgical procedures, such as a pacemaker for abnormal heart rhythms. Others may need a heart transplant. Heart function, as well as the function of other organs that may be impacted by abnormalities in the circulation, will be monitored over the long term.  Overall, it is expected that these children will have a good quality of life: have friends, play, and go to school just like other children. http://www.mottchildren.org