KALEB'S HEART CONDITION

Many people have asked what heart complications Kaleb has. There is not a single website that we can point you to that shows the complication because there are many, and no one has ever seen them all in one child. I've spent some time and put some graphics together below to help explain them all to you in a short and long version.   Below is the list of the complications that Kaleb has:

-Heterotaxy

-Dextacardia
-Complete AV (Atriventricular) canal Defect
-Pulmonary Atresia (no pulmonary artery connecting heart to lung)
-Discontinous Pulmonary Artieries
-Supercardiac Total Anomalous Pulmonary Venous Return (TAPVR) to a right SVC
-Bi-Lateral SVC's (Superior Vena Cavas)
-Pulmonary vein obstruction
-Right Aortic Arch
-Asplenia - (no spleen)
-Malrotated Intestines

My Explanation: 
As you know Kaleb’s heart did not develop correctly, and his heart will need to be repaired through multiple surgeries.  This specific blog is to explain the complexity of his heart conditions.  As a good friend of mine once said, “ask Karl for the time and he’ll tell you how to build a watch.” I will provide the short and long version for everyone.  However, during this process, more people have asked me detailed questions than what I’m more accustomed to hearing.  Therefore, I’ve developed images and diagrams that will help.

Short version:  (for those who don’t want to know how to build a watch)

Kaleb’s heart is on the wrong side of the body and is pointing in the opposite direction. His heart only developed 2 chambers instead of 4.  The part that sends blood to the lungs did not develop and is obstructed.  The part that brings blood back from the lungs to the heart is in the wrong place.  In short, there is no way to get blood to the lungs without surgical intervention.

Long version: (for those wanting to know how to build a watch)

Before we begin, go get a drink and use the bathroom.  You’re about to get a crash course in the anatomy of the heart through 6 images.  Please keep in mind; I’m an architect and not a thoracic surgeon or cardiologist, so I may not be 100% correct.  However, through extensive research and conversation with the doctors, I feel pretty comfortable that I have the overall picture/story correct.  So without further a due, let’s get started.

Color code:

Each colored arrow and text is representing something. This is consistent throughout the process so that you’ll know the difference between un-oxygenated blood, oxygenated blood, and mixed blood.  Everything in green explains the defect or surgery. 
Blue = un-oxygenated blood (blue blood is bad)
Red = oxygenated blood (red blood is good)
Purple = mixed blood (blue and red make purple, a mixture of good and bad blood)
Green = what I’m trying to convey or describe. 

Image 1: Normal Fetal Anatomy:

The first image is a normal heart.  Follow the numbers and notice the colors.  I won’t go into too much detail because I’ve provided quite a bit of information on the image.  The atriums receive blood and the ventricles send blood.  Right atrium receives blood from body, while left atrium receives blood from the lungs.  Right ventricle sends blood to the lungs, and left ventricle sends blood to the body.  Remember that the ventricles are the lower part of the heart and are the pumps.

All women produce a hormone called prostaglandin that creates the PFO (Patent Foramen Oval) and PDA (Patent Ductus Arteriosus).  The mom provides oxygen to the baby through these in utero.  After a baby is born, the mom stops producing this hormone and the “bypasses”, as I will refer to them, naturally close.   

This link is a good reference on how this works. http://www.pted.org/?id=fetal1.  I only bring this up because the PDA is very important for Kaleb. 

Image 2: Dextrocardia and Heterotaxy

This section may seem a little wordy, but Heterotaxy causes multiple complications and will be a lifelong issue for Kaleb.

The first two heart complications are Dextrocardia and Heterotaxy.  Dextrocardia only means that the heart is pointing in the opposite direction http://www.pted.org/?id=dextrocardia1.  Heterotaxy is the latin word meaning wrong place http://heterotaxy.org/what_is_heterotaxy.aspx.  Because we have both conditions, Kaleb’s heart is on the opposite side of the body pointing in the opposite direction.  Basically it is flipped along the center of the chest.  Use your left hand when saying pledge of allegiance instead of your right.  Your hand represents the position and location of the heart.  There are complications with heterotaxy.  Spleen and liver seem to be the biggest concerns.  There is a 95% chance that the spleen will either be asplenia (no spleen) or polysplenia (multiple spleens that probably don’t function properly).  The spleen controls your antibodies.  Kaleb may have to take an antibiotic every day for the rest of his life.  Due to heterotaxy, Kaleb also has malrotated intestines. https://childrenshospital.org/az/Site1181/mainpageS1181P0.html This is very common in heterotaxy kids, and it has a relatively simple corrective procedure.  The procedure is referred to as the Ladd’s procedure http://emedicine.medscape.com/article/930313-treatment#a1128 and we will have this procedure very quickly after our big heart procedure.  In short, they will untwist Kaleb’s intestines, tack them in place, and he will be fine.  The biggest issue we face with Heterotaxy is arrhythmia.  Because the heart was developed in the wrong location, the electrical circuitry does not fire as correctly as it should.  The image below is basically the same as image 1 but shown flipped representing Heterotaxy and Dextrocardia.

Image 3: Complete AV Canal Defect

The image is pretty self-explanatory, but the easiest way to understand this is to think of a normal heart with 4 chambers and then cut a hole between the lower and upper chambers.  Another way to look at it is to visualize the heart looking down on it.  In a normal heart you would see two flaps on either side.  In Kaleb’s heart, you would see only one big flap. (Right image on webpage) This website shows the condition quite clearly. http://www.pted.org/?id=atrioventricularcomplete1.

Image 4: Pulmonary Atresia

Pulmonary Atresia is when the part of the heart that sends blood to the lungs did not develop.  http://www.pted.org/?id=pulmonaryatresia1.  Notice when you roll over the image on the website it shows the right ventricle being smaller.  This is not the case in Kaleb, which is great, and has given us some better surgical options!

Image 5: Pulmonary Branch Obstruction

The website that I’ve been using does not have an image for Pulmonary Branch Obstruction but it is really not necessary.  The pulmonary artery makes a “T” at the back of the heart.  This allows blood from the heart to flow to either lung.  However, this “T” is blocked in Kaleb.  Therefore, Kaleb naturally created two PDA’s.  This is amazing, and is probably one of the reasons why he is doing so well, and has not had any major lung issues.  Just think if he only developed one…..one lung would never receive blood.  According to our Cardiologist, a 3 year old could survive with only one lung, but an infant cannot. 

Image 6: Total Anomalous Pulmonary Venous Return to a Second SVC. (TAPVR)

This is probably the most complicated defect to explain.  http://www.pted.org/?id=tapvr1.  There are 2 pulmonary veins that bring blood from the lungs back to the heart, for a total of 4.  In Kaleb, these veins come together behind the heart instead.  Even more complicated, the veins transverse the back of the heart to another SVC, which is a vein that brings blood from the body to the heart.  Most TAPVR children do not have a second SVC, so this may be a little confusing when you look at the website.  The veins from the lungs never go to the heart.  Along with this, Kaleb has a mild obstruction of the pulmonary veins right at the point where they connect to the SVC.  There is nothing that any surgeon can do for obstructed pulmonary veins.  However, in Kaleb’s case, it has been a miracle and he is diagnosed with this condition but has never clinically shown any signs of it.  Our cardiologist (who does the catheterizations) has said it is obstructed, but where it is located it has not been an issue and will not be for surgery.  They will ultimately remove the obstructed part of the pulmonary vein and will not be an issue after surgery.

Image 7: Kaleb’s Heart Prior to Surgery

As you can see, it is a miracle that Kaleb is alive with so many complications.

Surgical Procedures

Kaleb’s first procedure was to install coronary stents in the PDA’s at one week of life, a Cath procedure that has never been successful (no publications) in a child his size/age.  This allowed Kaleb to have oxygenated blood flow to the lungs.  It was a very risky procedure, and he suffered severe arrhythmia after the surgery.  He recovered, thrived, and had the stents enlarged 3 months later.  He is now roughly 6 months old and is ready for his big heart repair.  I could go into a lot more detail on how we have gotten to this point, but it really is not necessary.  Jenifer and I use the term single ventricle lot, and I think I should explain the procedure to you.

Single Ventricle Repair: http://www.pted.org/?id=singleventricle1

Many of you have heard me say that we have a figure 8 circulatory system.  Blood goes from body to heart – heart to lung – lung to heart – heart to body.  The heart is the middle of the figure eight.  Through 3 surgeries the veins and arteries will be re-routed to create what I refer to as a circular circulatory system.  Blood goes from body to lungs – lungs to heart - heart to body.  No pump, just passive blood flow.  There are long-term complications with this because the blood flow in the body is not as fast as a normal heart.  The oxygenated blood has to travel further, as well, and is not as effective throughout the body.  Limited exercise and organ failure are possible as Kaleb grows. The 3 heart procedures involve the following:

BT Shunt: http://www.pted.org/?id=doubleoutlet3

Glenn: Couldn’t find any images on line.  Procedure removes shunt and connects SVC with Pulmonary Artery.

Fontann: http://www.pted.org/?id=fontan1