Gray's Anatomy

James' current official diagnosis (as of January 24, 2013) is Double Inlet Left Ventricle (DILV), with transposition of the great arteries (Aorta and Pulmonary Arteries) complete Ventricular and partial Atrial Septal Deviation (VSD & ASD), and a variation on tricuspid atresia, where his valve simply isn't operating as it should. These are a series of very rare but related congenital heart defects.

Simply put, his heart didn't develop properly, and instead of having 4 effective chambers of the heart, James has 3. Normally, in a 4-chamber heart, non-oxygenated blood returns from the body to be pumped from the heart to the lungs, becoming oxygenated, and the oxygenated blood is then pumped from the lungs through the heart and back out to the body. In a 3 chamber heart like James', all the blood gets mixed up together, so this essentially makes it close to impossible for his lungs to oxygenate his blood and his heart to get that blood to the body.

Although this is a permanent condition, with a series of required surgeries over the course of his early years, he has very good chances of being able to live a fairly normal, active, healthy life. As complicated as it can seem, a broken heart really is very simple. Here are two very different ways to think about this. We hope they both help.

Keep It Simple

If medical jargon ain't your thing, or when it just seems to be getting too complicated to make sense of, what we find most helpful, and perhaps most important to remember, is that, aside from whatever he might feel recovering from the various surgical things the docs will do, our baby won't feel any of his heart issues on a day-to-day basis. And, even after he completes the barrage of surgeries needed to make his system work effectively, he'll go years without any inclination of what the pros are saying. If he can do it, we can do it too :)  

Embrace Modern Medicine

If, on the other hand, you find solace in knowing the inner workings of our bodies, the remainder of this page shows the medical information we've received from our doctors and found online since the initial day of diagnosis (February 14th, 2012). If you find the scientific regalia helpful in navigating what is happening, we'll keep posting as much information as we receive and find in an effort to keep you as informed as we are, and maybe so we won't have to explain this too many times :)




Current Diagnosis: Double Inlet Left Ventricle

Since our initial diagnosis of DORV (double outlet right ventricle) during one of our anatomical prenatal ultrasounds (this one at Brooklyn Hospital on January 31st, 2012), to our most current diagnosis of DILV, James has undergone 2 surgeries: a BT-Shunt procedure on June 23, 2012, and his Bi-Directional Glenn procedure on January 24, 2012. The third surgery, the Fontan procedure, completes what was started with the Glenn.

Surgeries 1,2, &3

The BT-Shunt (this was surgery 1) essentially creates an artificial pathway for de-oxygenated blood to get from the heart to the lungs. Dr. Chen, his surgeon, called his BT-Shunt one of the best from 2012. When done at an early age, children will usually outgrow this shunt in 4-8 months, at which time low blood-oxygen saturation levels (sats) will lead to the second stage of surgery. 

Since the heart's connection to the lungs via the pulmonary artery (PA) is not viable with his condition of DILV, the Bi-Directional Glenn essentially creates a new "natural" pathway for blood to get to the lungs. It does this by allowing blood to passively flow to the lungs on its way back from the upper body via the superior vena cava (SVC). Literally, this surgery disconnects the SVC from the heart and connects the SVC to the right PA. This negates the need for the heart to do what it can't already do (pump blood to the lungs), and allows it to focus on simply pumping blood to the body. James had the Glenn Procedure in 2013 (that was surgery 2) and is spending a few years now letting his body adapt to the new circulation system before moving on the the next surgery.

What is the Glenn-Fontan?

When James is fully recovered from the Glenn and is beginning to need more oxygenated blood to his lower extremities as he grows, the Fontan procedure completes the new circuit by connecting the inferior vena cava (IVC) to the left PA. The Fontan is this part 2 of the Glenn/Fontan duo: redirecting blood flow through the body is a major change, so it’s done in two stages and two surgeries a few years apart. In this way, the heart will no longer try to pump any blood to the lungs, but instead only focus on pumping blood to the body. James will have this in spring/summer of 2015.

More Info

DILV is very rare and not much information is online. However, it is a form of SVD – single ventricular defect – which is has a lot of info available. Several other forms of SVDS are more researched, but the anatomy and the treatment are nearly identical. So, if you’re looking to better understand DILV, here are some links to help.

SVD: Single Ventricular Defects

DORV/Double Outlet Right Ventricle

HLHS: An SVD very similar to DILV with more information online

Complications & other stuff

James has been very lucky to not encounter complications from either of the two surgeries thus far, or illnesses in between. He struggled a lot with weight gain and had a feeding tube, discussed in blog posts here and here and here, and is on a high-calorie Pediasure 1.5 diet now, which is working great. He gets Speech Therapy, Physical Therapy, and Occupational Therapy for developmental delays, and this extra attention has helped him progress almost as normal. Our blog post about why he gets therapy and what it does is here.