Differences part 2

Because Alexander’s heart is complicated and I am wordy, I couldn’t quite keep the discussion about his heart in one post.  Drawing his heart, his anatomy, his condition has become very important to me.  I remember those first days when I was searching the web for “heterotaxy syndrome” and how little information would pop up.  I recall that most the articles were pitifully short and lacking details, or so in-depth I felt I needed a medical dictionary just to wade through the first paragraph.  Little more than a year later, that has changed for the better, and I want to continue the trend.  I want families to be able to understand Alexander’s diagnoses, and perhaps that would help them understand the ones they have been presented. 

Two Right Atria

The right atrium and the left atrium are structurally different.  They are intended to match with their corresponding ventricle.  For instance, the electrical impulses that tell the heart to beat originate in the right, and the left side of the heart is higher pressure system.  In Alexander’s heart, the atrium on the left side of his body is structured like a right atrium.  Many doctors say this shouldn’t cause a problem; if he had two left, he might need a pacemaker.  However, it is something of concern to me.  Alexander is unusual that he will have a two ventricle repair.  Not many heterotaxy-RAI patients are so lucky.  But what is luck for his ventricles, there really is a lack of information on how well his left-sided atrium will hold up.

Atrial Septal Defect (ASD) and Ventricular Septal Defect (VSD)

The separating wall between the left and right side of the heart is known as the septum.  It is solid and keeps the blue blood from mixing with the red.  In utero, there is a small hole (foramen ovale) between the left and right atrium, but like the ductus arteriosis (DA) it closes soon after birth.  In Alexander’s case, it was larger than normal and did not close on its own.  Alexander also had a moderate sized hole between his left and right ventricles.  Sometimes these defects close on their own, but Alexander’s were considered to be too large for that to happen.

Left-sided Inferior Vena Cava (IVC)

There have been many times when I have been baffled by Alexander’s anatomy, but none surprises me as much as this defect.  The IVC is a right-sided structure.  It brings all the deoxygenated blood back to the heart from the lower part of the body.  It forms from the two iliac veins (one from each leg) and travels just right of the spine to the right atrium.  All of the lower body’s veins flow into it as it climbs upward.  Before entering the atrium, the hepatic veins from the liver flow into the IVC.

Alexander’s IVC is much different.  He has a true left-IVC.  It is to the left of the Aorta and spine, and both of the iliac veins flow into it.  It continues past the renal (kidney) level and flows along what is referred to as a hemi-azygos continuation.  It then flows into the left side of the heart with no interruptions.  Alexander’s hepatic vein connects directly to the right atrium and his azygos vein connects to the right atrium instead of the SVC.  If you look at the vascular structure of Alexander’s heart with little regard for labels, you can see how much the left mirrors the right.  While this anomaly by itself may not cause issue, the flow from the IVC must be routed back to the proper side of the heart and any surgeon that may operate on him need to know of his abnormal vasculature to avoid mistakes.

Partial Anomalous Pulmonary Venous Return (PAPVR)

The pulmonary veins bring oxygen rich blood back to the heart to be delivered to the body.  Each pulmonary vein (there are typically four, but can be more) independently attaches to the left atrium.

In Alexander’s anatomy, none of his pulmonary veins attach like they typically should.  However, three of four veins do drain into the left atrium.  His right lower pulmonary vein (RLPV) and left lower pulmonary vein (LLPV) come together in what is known as a confluence before draining into the left atrium.  The left upper pulmonary vein (LUPV) drains into the L-SVC which drains to the left atrium.  The right upper pulmonary vein (RUPV) is the problematic one.  It drains into the R-SVC which drains into the right atrium.  In a heart with all of its flow being normal, this would reduce the oxygenated blood around 25%.  It also causes an increased workload on the right ventricle by adding additional blood volume.

Alexander arrived with a special kind of heart.  He has certainly had his cardiac team on their toes learning about his system.  But Alexander has other anomalies, too.  The effects of heterotaxy are far and wide on his tiny system, as is usual with those with this condition.  His body is like a tiny puzzle, every piece must be scrutinized and fit together exactly right to function well. 

I hope that in my next post I can describe heterotaxy on more of a broad scale, helping others understand the scope of this terrible condition.

***I am not a nurse or doctor, nor have I received any formal medical training.  Any and all medical related information on Drawing Heart is the product of a mother’s desperation to understand and advocate for her child.  This blog is the culmination of countless hours of independent research and medical consultation and is meant only to communicate my understanding of Alexander’s condition.  It is not intended as medical advice.  As always, seek the advice of a qualified medical professional to explain your specific diagnosis.***

Differences

Even with no knowledge of heart conditions, it’s easy to see Alexander’s heart is significantly different from a normal one.  It was created uniquely, just inside his tiny body and no where else.  However, it has and continues to work for him.  He’s a special heterotaxy-RAI guy that’s fortunate enough to have two ventricles.  The following is how I perceive his differences.  Please remember, I’m just a mom devouring medical documents, not a trained medical professional.

Dextrocardia (Incomplete)

Dextrocardia is when the heart is on the opposite side of the body.  Normally the human heart is located just on the left side of the body with the apex the furthest point left.  Oddly enough, the apex is not at the top of the heart, but the very bottom.  In complete dextrocardia, the heart is just on the right side, with the apex furthest right.  It would look something like this:

Alexander’s dextrocardia is incomplete.  His heart is completely midline, straight as you are looking at him, with the apex of his heart near the tip of his sternum.  

While in itself, this is not a problem, it has many effects.  It makes surgery more difficult, as the parts and structures surgeons are familiar with are not where they normally are, and creates new problems when trying to re-route heart plumbing.  It makes it more difficult to get accurate readings with ECG (electrocardiogram), it makes Echos (echocardiograms/ultrasound of the heart) harder to read.  Most of all, it’s indicative of other structural differences of the heart.

dextro-Transposition of the Great Arteries (d-TGA)

d-TGA generally happens when the heart is forming, near the 24^th day of pregnancy.  Instead of twisting one last time, the Aorta and Pulmonary Artery remain parallel to one another.  While mother is carrying the baby, it has no effect, as the mother oxygenates the blood (baby doesn’t breathe) and the Aorta and Pulmonary Artery are connected by the Ductus Arteriosis (DA).  For such a simple problem, it devastates the circulatory system.  

  

At birth, a mother’s hormones stop being passed to baby and the DA begins to close.  The body gets less and less oxygen.   In d-TGA, instead of operating as an entire system, the heart operates as two closed loops.  The blue blood enters the heart on the right side and is immediately sent back out to the body through the Aorta.  On the left, blood rich in oxygen from the lungs enters the left and is sent directly back to the lungs.  The result is the body’s tissues are never oxygenated and the lungs receive blood at high pressure.   Without surgery, death is near certain by one year of age.

While Alexander was born with this condition, because of his other defects, d-TGA was not able to leave his body with no oxygen.

Dual Superior Vena Cava (SVC)

Because of Alexander’s heterotaxy, many structures that normally occur on the right are copied onto the left.  The SVC is the major vessel that drains all of the blood from the upper part of the body (from the heart up) back to the heart so it can be re-oxygenated.  It connects to the right atrium.  

Alexander also has one on his left side.  It connects into the left atrium much like the other side.  There was one small bridging vessel between the right and left SVCs.  The left SVC is problematic because it would return blue blood to the side of the heart that deals with red.  Instead of being able to head to the lungs and exchange its carbon dioxide for oxygen, it would simply mix with red blood and head out to the body.

Those are three of Alexander’s heart defects; he has five others.  Like all of his heterotaxy brothers and sisters, his anatomy is complicated.  In the interest of keeping blog sizes manageable, I’ll save those for later.

***I am not a nurse or doctor, nor have I received any formal medical training.  Any and all medical related information on Alexander’s Fight is the product of a mother’s desperation to understand and advocate for her child.  This series of notes is the culmination of countless hours of independent research and medical consultation and is meant only to communicate my understanding of Alexander’s condition.  It is not intended as medical advice.  As always, seek the advice of a qualified medical professional to explain your specific diagnosis.***

A Normal Heart

When I began my journey understanding Alexander's heart, I realized that I must know what a regular heart does and looks like.  Only then, could I appreciate the special structures, the innovative compensation, and unique organ my son possesses in his chest.  It simply amazes me how a body can learn to deal with adversity and just how strong the body's instinct to survive is.

The heart is a miraculous machine.  It’s a pump that circulates blood throughout our bodies.  It delivers oxygen and nutrients to our tissues and disposes of the carbon dioxide and other waste.  It pumps approximately 60 times per minute, every minute of every day.  In a 50 year lifespan that’s approximately 1,576,800,000 times.  Yes, about one and a half BILLION times.

In a normal heart, blood enters from the body through veins on the right side.  Its oxygen stores are depleted and it’s blue.  It’s received in the right atrium, passes through the tricuspid valve into the right ventricle so it can head to the lungs.  When the right ventricle squeezes, it leaves through the pulmonary valve in the pulmonary artery.

The pulmonary artery branches and delivers blood to both lungs.  Here, the carbon dioxide waste is traded for oxygen and the blood is red again.  It returns to the heart through the pulmonary veins (there are usually 4 of these, but more can occur naturally) into the left atrium.  When the heart relaxes, the oxygenated blood passes through the bicuspid (or mitral) valve into the left ventricle.  With the next pump of the heart, the blood is pushed out of the aorta, through the aortic valve where it travels to the body to deliver its oxygen and nutrients.

This is a very elegant solution that supports every system in our body.  And every year 1 out of every 100 children born will have one that is less than perfect.  Congenital Heart Disease (CHD) claims more lives than all of childhood cancers combined and is the leading cause of death in children.  Our children need us to understand how this miraculous muscle works.

Alexander has no less than eight CHDs.  Less than 10 years ago, his diagnosis (Heterotaxy-Right Atrial Isomerism) had 80% mortality before the first birthday.  While the numbers are improving today, they still are frighteningly poor.  It is a constant worry, but odds we plan on defeating.

This is Alexander's heart beside a normal one.  It's obvious that it's different, but it's still strong.  His specific heart diagnoses are:

Dextrocardia (not complete)
d-TGA (dextro-Transposition of the Great Arteries)
Dual SVC
two right atria
ASD (Atrial Septal Defect)
VSD (Ventricular Septal Defect)
left-sided IVC
PAPVR (Partial Anomalous Pulmonary Venous Return)

Among children born with Heterotaxy-RAI, he is lucky.  His left ventricle is full size and fully functioning.  He lacked defects involving his pulmonary and aortic valves and it's hopeful that we can straighten out his circulatory system without compromising his quality of life.  We are hopeful and he is strong.

***I am not a nurse or doctor, nor have I received any formal medical training.  Any and all medical related information on Drawing Heart is the product of a mother’s desperation to understand and advocate for her child.  This blog is the culmination of countless hours of independent research and medical consultation and is meant only to communicate my understanding of Alexander’s condition.  It is not intended as medical advice.  As always, seek the advice of a qualified medical professional to explain your specific diagnosis.***

Drawing Heart

***I am not a nurse or doctor, nor have I received any formal medical training.  Any and all medical related information on Drawing Heart Project is the product of a mother’s desperation to understand and advocate for her child.  This blog is the culmination of countless hours of independent research and medical consultation and is meant only to communicate my understanding of Alexander’s condition.  It is not intended as medical advice.  As always, seek the advice of a qualified medical professional to explain your specific diagnosis.***

 

From the moment Alexander was born he was making doctors ask questions.  Because of fetal imaging we knew he would be born with d-TGA (dextro-Transposition of the Great Arteries).  Traditionally, these infants have been called “blue babies” as the circulation did not allow oxygen into their blood and to their bodies.  Alexander was born pink and continued to be.  Doctors knew something else must be occurring for him to be so well oxygenated.

Within hours, Alexander was the main attraction in the NICU/CICU.  Every doctor came to look at him, tons of imaging was done, nurses were constantly puzzled about his true condition.  We had one doctor that consistently drew pictures for the other staff, trying to communicate Alexander’s entire heart status.  Terminology was constantly thrown out, and Matt and I weren’t nearly as educated on the topic as we are now.

At eight days old, they prepared to take him into surgery for the first time.  The cardiac surgeon talked to us about his conditions and the surgical plan when he spoke the words that inspired this project.

“He’s unique; no other like him.  You won’t find a picture of his heart in a book or online.  He’s as rare as they come.”

That upset me.  How could I, a “normal” parent, understand when the doctors are not only delivering diagnoses that I’ve never heard of, made up of multiple words that each need a google search, but have no real illustrations to go with it?  How could I grasp Alexander’s entire condition “blindly?”

The answer was a long amount of time, a ton of research and, eventually, sketching his anatomy.  It brought a great amount of confidence understanding his condition and being able to communicate it.  I’ve found myself sketching the same heart pictures over and over, refining them, explaining his special heart to the newest doctor.

That brings me to now, and the project that’s known as “Drawing Heart” on my hard disk.  I realized that if I had trouble understanding Alexander’s condition, others must have as well.  I decided that I would draw the structures and conditions that have become so familiar to me in hope that others would be able to better understand by looking and reading my own interpretations.  My goal is to raise awareness and understanding for all things heterotaxy.

For now I’ll just leave my drawing of Alexander’s special heart when he was born(so now a picture of it can be found online!), but I will explain it in a later post.  I hope “Drawing Heart” brings attention and increases understanding.