The normal human heart has four equal-sized pumping chambers; two atrium (a right and left) and two ventricles (right and left). The right side pumps blood to the lungs, and the left side pumps blood to the rest of the body.
Many children are born with chambers which are smaller than they are supposed to be; some examples are single ventricle and hypoplastic left heart syndrome, but there are many different types and subtypes. Amazingly, it has become possible over the years for many of these children to live active and healthy lives despite this serious condition. This is especially true for many children and teenagers in San Diego, as Rady Children’s Hospital-San Diego has performed hundreds of operations for these conditions over the past 30 years, including those listed below.
In most cases, some form of cardiac surgery is required in order for the child to remain healthy. Several operations are usually required. Small children (newborns and infants) who require surgery usually undergo a shunt operation (see Single Ventricle page) as a first surgery. This allows the child to grow and thrive, and return at an older age for additional work.
At age 4-6 months, infants with these conditions (including both those who have had a shunt and those who have not) will undergo a bidirectional Glenn or BDG for short. A BDG allows the child several years to further grow and thrive and live an active life.
At age 3-4 years old, one additional connection is required; this is called a Fontan operation. In a Fontan, another large vein called the inferior vena cava is connected to the pulmonary arteries. This allows more blood flow to the lungs and results in a higher oxygen level in the blood, resulting in a pinker appearance. There are different ways to complete this connection, and we have performed all of them, but we usually favor the use of a large tube of Gore-Tex (the same material rain jackets are made from). The operation requires cardiopulmonary bypass and additional procedures can be performed, if necessary, during the Fontan.
In the modern era, a Fontan is always done after a BDG and so is always a redo operation. Although redo operations are more difficult and take longer to complete, our experience has brought excellent results. From 2009-13, we performed more than 70 Fontan operations with a 98.7 percent success rate to hospital discharge.
Fontan patients sometimes have prolonged hospital stays (several weeks), but the overall success rate is very high nonetheless. they do not usually require further surgery but do require close follow-up and sometimes undergo further catheterizations and minimally invasive procedures. For those rare patients who do require further surgery (pacemaker placement, other surgeries), we also have good outcomes. In the current era, Fontan patients are routinely surviving into their teenage years and on to young adulthood.