The Fontan

Our 4 year old child, Corey, is scheduled for the Fontan open heart surgery on Tuesday, April 7th.  Many people probably don't know why Corey needs this surgery, why it's happening now, or what comes next. So I have decided to write information about the surgery in interview format.  There are links in dark grey below to sites that provide more details.

Let's start with the basics...

Why does Corey need heart surgery?

Corey has a congenital heart defect called tricuspid atresia.



Kids with tricuspid atresia lack a tricuspid valve, and therefore, lack a functioning right ventricle. In other words, Corey has just one functioning heart pump instead of two. Kids with tricuspid atresia typically require almost immediate intervention. Corey required emergency heart surgery when he was barely two weeks old. For six months, until his second surgery, Corey's blood circulation was in a tenuous position. Because Corey has holes between the left and right sides of the heart (required during development to allow blood to flow at all), much of the oxygen-poor blood returning to his heart to pick up oxygen was being pumped back to the body before passing through the lungs. Because of this, his oxygen saturation levels were low; he didn't have much energy to do important things like eat.

The long-term fix to Corey's situation was a two-step process to re-route all blood returning to the heart from the body directly to the lungs via the pulmonary artery – in other words, to bypass the right side of the heart completely. This fix would stop the mixing of oxygen-poor blood returning from the body and oxygen-rich blood returning from the lungs.

The first step of this two-step process is an open heart surgery called the Glenn surgery, which Corey had done when he was 6 months old. The Glenn procedure re-routes the blood from the head and arms directly to the pulmonary artery, bypassing the right heart. The next step is the Fontan procedure.  Before the Fontan can be done, a cardiac catheterization is required. Corey had his cath yesterday.

What is a cardiac catheterization, and what was discovered during Corey's 3/31 cath?

Corey's cardiac catheterization was done for two reasons. First, blood pressures were taken on both sides of the heart to learn whether Corey's circulation is such that it will allow for the post-Fontan procedure circulation. Second, the pulmonary arteries were looked at in detail to determine whether the stenosis (narrowing) Corey has is great enough to require the placement of a metal stent to open it. The pulmonary stenosis is a necessary by-product of Corey's second heart surgery, the Glenn, that he had in the summer of 2005 when he was 6 months old.

Both questions were answered in our favor: Corey's blood pressures are very favorable to allow for the Fontan surgery, and the stenosis is not great enough to require the placement of a stent.

So now that the catheterization was a success, we are able to move on to the open heart surgery Corey will have next week, the Fontan.

What is the Fontan procedure?



The Fontan procedure is an open heart surgery that Corey will have this spring, currently scheduled for April 7. (Another good overview of the Fontan can be found on Wikipedia.) The Fontan procedure re-routes the blood from the lower part of the body directly to the pulmonary arteries, bypassing the right side of the heart. The pressure and flow created by the left side of the heart will push the blood through the entire circuit on its own, without any help from the right side of the heart.

Why is Corey having this surgery now that he's older?

The doctors at Johns Hopkins like to wait until the kids are between 3-5 to perform the Fontan surgery.

The surgery cannot be performed right after the Glenn surgery because the blood pressures in babies is too high to handle the Fontan circulation.

Additionally, a Gortex conduit will most likely be used to go around the right side of the heart directly to the pulmonary artery. This conduit needs to be big enough to last Corey into adulthood without being too big to handle the current blood pressures in Corey's body.

However, Corey's left ventricle is doing a lot of unnecessary work right now to keep his oxygen saturation levels high enough since it has been pumping oxygen-poor blood back to his body for more than 4 years. If we wait too long, then this will wear out Corey's left ventricle prematurely.

So after the Fontan, Corey's heart will be fixed?

Not exactly. Corey's surgeries have been palliative, meaning that all three are intended to reduce the affects of his heart defect - the surgeries are performed as a workaround, not a fix. Since the tricuspid valve never formed, the right ventricle did not properly form. There is currently no way to grow a new ventricle. However, kids like Corey have a great chance to live long, fulfilling lives, far into adulthood.

How long will Corey be in the hospital?

Kids who did well with the previous surgeries tend to do well with the Fontan. Corey was in the hospital about a week for both previous surgeries so this bodes well for his Fontan stay. We were told during the cath that Corey's stay will most likely be in the range of 7-10 days. For the first 3-4 days after the surgery, Corey will be in pediatric ICU. After his pain is being handled and he is well enough, he will go to the Children's floor for 4-7 days until he is well enough to come home.

So after the surgery Corey will just heal and recover?

Basically, yes, but if the previous surgeries are any indication, unexpected surprises are likely along the way to a full recovery.  Corey's two previous surgeries went extremely well but there were several moments of great concern during the recovery period in the hospital for both.

Several factors determine the length of hospital stay post-Fontan surgery. Chest tubes will likely be inserted into Corey's abdomen to drain any fluid collecting around his lungs. This is fairly common after these heart surgeries. After the Glenn, Corey had a single chest tube for a few days. Another main factor in determining the length of stay is how Corey's heart and body adjust to the new blood circulation. The post-Fontan circulation will be a big shock to Corey's heart and it will need to make adjustments to adapt.

Feel free to ask additional questions you might have about the process Corey will be going through. It's tough to know exactly what questions people have. I'd be glad to answer any as well as I can.

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