Savannah has a condition called Heterotaxy Syndrome that consists of multiple complications that stem from her body’s inability to properly orient a left and right side. Plainly, some of her internal organs are not completely formed and are in the wrong place.

The most severe complication is with her heart. She has dextrocardia. In basic terms, it means that her heart formed on the wrong side of her body. The heart is normally located in the left chest. In Savannah’s case, her heart is essentially flipped 180 degrees over a nose to navel axis resulting in her heart being on the right side of her chest and flipped over.

In the process of forming on the wrong side of her body, her heart did not develop properly. Most people have four well defined chambers in their heart. Savannah is missing key parts of that four chamber heart structure. There are also issues with the arteries and blood vessels going to and from her heart.

Adding to her condition, Savannah’s spleen is missing its main artery. Doctors don’t even really see a splenic structure, so it’s hard telling what, if anything, is actually acting as a spleen. This is of grave concern due to the fact that the spleen is instrumental in building and maintaining the immune system. Without a properly functioning spleen, Savannah’s little body could a difficult time healing itself and fighting infection. Obviously, this is a problem knowing that there will be significant surgeries needed just for her to survive. In coming weeks, her immuniologist will begin evaluating her level of spleen function.

Savannah also has a narrowing of her airway, subglottal stenosis. This should be something that will continue to grow with her, hopefully not requiring surgical correction.

We also know that Savannah has malrotation of the bowel. This is a common complication associated with heterotaxy syndrome. This may require surgical correction at some point. The concern is that her bowels will get twisted and blocked as she continues to grow. So far, she is still passing waste through her bowels.

In addition, Savannah has a malformed liver. This too is not quite in the right spot. Her liver is not necessarily that big of a deal, because it seems to be functioning properly. Also, Savannah’s kidneys are a bit smaller than normal, but they function just fine.

Ultimately, what does all this mean? Frankly, the doctors are optimistic, but there is no certainty.

Heart Cath Loop shows Savannah's DextrocardiaThe first months of her life have been medically scrupulous — monitoring, home health nurse visits, weekly cardiologist checks, etc. Now Savannah has a fully septated four-chamber heart.

In May 2015, Savannah had her complex biventricular repair at Boston Children’s Hospital in Boston, Massachusetts. They are arguably the best center in the world for this sort of reconstructive heart procedure. In the coming years, we will still have to travel to Boston several times for follow up appointments and procedures.

Outside of continuous monitoring, our hope is for Savannah to be able to live a rather normal life after her surgeries. Sadly, this is just a hope. There is no way to predict an outcome due to the minimal amount of research and the complexity of her condition. The doctors won’t give an official statistical chance of survival.

Heterotaxy Syndrome occurs in only 1 in 250,000 children.