Friday, January 10, 2020

How to "fix" Jude

So far on our blog journey we have discussed our diagnosis, where we plan to deliver, and how we came to Texas Children's, but we haven't said much about how doctors plan to treat Jude's defect.  In today's post, I'd like to discuss what might be in Jude's future as far as treatment is concerned. 

First, let's talk about the two broad categories of "fixes" for congenital heart disease.  I place the word fix in quotes because some of the surgeries done on children with congenital heart disease don't actually fix the defect but rather make it such that children can live with the defect.  In fact, in congenital heart disease treatment there are two broad categories of treatment -- anatomic repair and palliative treatment.

Anatomic repair means surgery truly fixes the defect.  The surgery corrects the problem and after surgery you are left with a heart that essentially looks and works like a normal heart.  These children generally live normal and healthy lives with little to no effect on their daily activities.

Palliative treatment means surgery can NOT be performed to make the heart normal.  In a palliative approach, the goal is to make changes to the heart and blood circulation such that the child can live as normal a life as possible.

If you had the choice between anatomic repair and palliative repair you would generally always prefer anatomic repair because it leads to better life quality, less complications, and less treatment down the road.

At our first cardiology appointment in Dallas we were told Jude would not be candidate for a anatomic repair given the complexity of his diagnosis.  In fact, this is one of the reasons we were referred to Texas Children's in Houston because our cardiologist in Dallas was hopeful the surgeons in Houston might be able to offer a treatment that was not available in Dallas. 

At our first visit in Houston the cardiologist confirmed she did not believe Jude would be a candidate for anatomic repair.  Despite Jude having a 4 chamber heart, both centers recommended we treat Jude with the Fontan Pathway/Single Ventricle Pathway.

Babies treated on the Fontan Pathway classically have been born with hypoplastic left heart syndrome -- Jude does not have this.  However, over the years the Fontan pathway has been used to treat children with other diagnosis that are too complicated for anatomic repair. 

The traditional Fontan Pathway involves 3 surgeries:
1. Norwood Operation
2. Bidirectional Glenn Shunt
3. Fontan Operation

Jude WILL NOT require a Norwood Operation because he has a 4 chamber heart with an aorta and pulmonary artery -- Norwood is required for babies with hypoplastic left heart syndrome.   

Therefore, on the Fontan pathway Jude will need the Glenn Shunt and the Fontan Operation.  Generally the Glenn Shunt is performed around 6 months of age and the Fontan Operation is performed anytime from 2-6 years of age.  Both are open heart surgeries.  I don't plan to discuss how the surgeries are done here -- there is a large amount of information and graphics on the internet for both if you'd like to learn more about them.

The big unknown for Jude will be how much blood flow he has to his lungs after birth due to his pulmonary stenosis.  Complicating this will be his large VSD.  Therefore, the real question after Jude is born is how low will his oxygen saturation be?  If we can keep his oxygen level above 75% (normal would be greater than 90%) he may not require any procedure after birth.  However, the doctors in Houston are worried he may not get enough blood flow to his lungs and as such would require a procedure to increase blood flow shortly after birth. 

As such, Jude's treatment plan looks like this:
1. BT Shunt or Ductus Arteriosus Stent (possible) [Birth-2 Weeks]
2. Bidirectional Glenn Shunt [6 months]
3. Fontan Operation   [2 years - 6 years]

We are all hoping we don't need surgery at birth.  Babies who need surgery at birth generally spend much longer in the hospital and have much higher rates of complications.  The Glenn and Fontan surgeries are much lower risk surgeries that generally only require several week hospital stays.

Finally, we should mention there is a small sliver of hope that Jude could get an anatomic repair.  At our cardiology appointment on December 23 our cardiologist informed us Jude's case had been presented in cardiology conference.  The chief of cardiothoracic surgery told everyone he would not totally exclude an anatomic repair but that he would be unable to know if Jude would qualify without "taking a look inside."  So, more wait and see.  For now, we are planning on Fontan and hoping for more options.

For those who want to know more about Fontan treatment and outcomes following Fontan there is a ton of information on the internet.  Keep in mind Fontan was developed in the 1970s and the technique has changed and results have gotten much better with time.  I'm not going to spend the time to go through all the steps and outcomes of Fontan here because there are already great resources on the internet that cover this.  I've listed several sites below that are good resources -- keep in mind all these sites are going to show hearts with hypoplastic left ventricles and hearts that have had Norwood surgery but Jude has neither so the pictures are not perfect.

BT Shunt:

Glenn Surgery:

Fontan Surgery:

So long until next time!


  1. Thank you for the continued updates. Sorry we could not make the send off. Our love and prayers follow all of you to Houston and Louisiana.