Monday, October 24, 2011


Between visits with various doctors and reading our 'homework' (material from said docs), we have a lot of information on hand regarding our sweet baby's heart.  Depending on how you look at it, there's some good news and some bad, but overall we have hope.  I've tried to break it down to the nitty gritty so as to avoid a final exam type of explanation.  Here's to hoping that I haven't butchered it too much.  Feel free to ask any questions, as I know we've had a million and one so far.

Hypoplastic Left Heart Syndrome is a congenital heart defect.  Most of the structures on the left side of the heart are small and underdeveloped.  This is a problem because this side of the heart pumps blood to the body which means the body can't get the appropriate amount of blood needed to support life.  A baby left untreated will become very sick after birth and if left untreated will not survive.

Essentially the 3-step surgical process will take a patient with a single functioning ventricle and create a heart that is able to support her long term.  The first surgery is performed in the first week of life, depending on the baby's specific anatomy.  The goal is to create a way to increase blood flow to the lungs via a "shunt."  This is often the most stressful surgery for a number of reasons.  Technically it is challenging because of the small size of the baby and her heart.  Too, newborns are physiologically more fragile than older children.  Some babies may require surgery on birth day while others are stable enough to wait until day 4 or 5.

The second surgery (a bidirectional Glenn shunt) is performed at around 6 months.  Again, timing is based around baby's specific condition, ie oxygen saturation levels, etc.  After her first surgery, her body will be at around 80-85% but by the time of her second surgery, she will be at around 70ish%.  Healthy heart people operate at 100%.  This operation allows for the passive return of blue (deoxygenated) blood, coming back from the upper part of the body, directly into the lungs. This is the first step at in lessening the workload on the single ventricle (right).  It significantly reduces the amount of blue blood that the single ventricle has to pump.

The third surgery (Fontan procedure) occurs when the baby is around 3 or 4 years of age.  During this operation, the heart achieves a normal workload.  In addition, the heart is appropriately septated.  In other words, the red (oxygenated) and blue sides of the heart are separated from each other and there isn't further mixing of blood.  Most patients have normal or near-normal oxygen saturation.  It also seals off any open communication between the blue and red sides of circulation.  In this way it significantly lessens the long-term potential complication of blood clots passing from either side.

She will also have at least 2 other surgeries - heart catheterizations - which are performed before each major operation to 'check out' the heart so there are no surprises during surgery.

The long-term results of this surgical process are still unknown as the operation has only been done commonly for the last 15 years or so.  The oldest surviving patients are in their mid 20s.  Whether this form of heart circulation will last for a lifetime is yet to be determined.  However even in this short time, enhancements have been made to the actual surgeries.  We are very blessed to be in the year 2011 and having options like this surgical process versus being sent home to wait.

Most children will reach adulthood in good health.  Some children will need other procedures such as a pacemaker and some will need a heart transplant at some point in the future.  Children are restricted from competitive sports and demanding physical activities but can otherwise do all the things that children enjoy doing, as well as enjoying a good quality of life.  So we don't have an Olympian on our hands, but that's okay...Mommy's not either.

1 comment:

  1. Ha! Either is Aunt Christy. Don't worry baby girl...we have plenty of fun things to teach you!!