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.***