Stephen's Diagnosis

Continuing to Shine the Light on CHD

Stephen was born with a life-threatening Congenital Heart Defect called 

Double Inlet Left Ventricle (DILV)

What is double inlet left ventricle?

Double inlet left ventricle is a congenital defect (the baby is born with it) in which the left and right atria (plural for atrium) of the heart are connected to the same ventricle (the left ventricle) leaving the right ventricle hypoplastic (small).

The human heart has four chambers: the left atrium and right atrium (plural: atria) at the top, and the left and right ventricles on the bottom. Blood circulates through the atria and the ventricles to and from the rest of the body via arteries (which carry blood away from the heart) and veins (which carry blood to the heart).

In a normal heart, blood that needs oxygen enters the right atrium and right ventricle of the heart through the vena cavas. From there, it travels to the lungs via the pulmonary artery to pick up oxygen. 

Blood that has picked up oxygen from the lungs comes into the left atrium. From there, it goes into the left ventricle. The left ventricle pumps this oxygenated blood through the aorta to the rest of the body.

Babies with double inlet left ventricle have an intact left ventricle (the pumping chamber of the heart that pumps blood to the body) and a small right ventricle (the pumping chamber that pumps blood to the lungs). Both atria supply blood to the left ventricle. As a result, oxygen-rich blood and oxygen-poor blood are combined. This mixture of blood flows to the baby’s lungs and body. 

Along with DILV Stephen has...

Ventricular Septical Defect (VDS) A hole between the 2 ventricles

Hypoplastic Right Ventricle (HRV) A small, unusable right ventricle

Levo-Transposition of the Great Arteries  (L-TGA):  The pulmonary and aorta are transposed as well as the right ventricle and left ventricle (his anatomical right ventricle is on the left side of his heart, and his left is on his right side.)  This actually made his first diagnosis Hypoplastic Left Heart Syndrome (HLHS) since it looked as if the left side of his heart was small.  It was after my first fetal echocardiogram that the cardiologist at Rady's gave the correct diagnosis.  I remember him coming in and asking what we thought he had....we said HLHS.  He said..."Well it's even more complicated than that." Of course it was!

Coarctation of the aorta (CoA): A narrowing of the Aorta.  (Not all DILV patients have this...Stephen does.  It is not shown in diagram below..because they don't have a diagram with Stephen's full condition.  This CoA contributed to his need of a more risky first open heart surgery.) 

This diagram below is closest I can find to Stephen's heart...though each child's heart is truly unique!

It is a miracle he is alive isn't it!  There is still no known cause of these single ventricle defects, though the whole process of growing a human is pretty miraculous!  The heart forms at just 6 weeks and when Stephen's did things didn't go according to God's design for the human heart.  Doctors have come up with a set of 3 palliative surgeries to gives babies like Stephen a shot at life...and for that we are grateful.  

I will later cover each of the 3 surgeries...it is truly incredible what they did.  While he looks like a pretty normal kiddo on the outside...he is "plumbed" completely differently on the inside!  But these surgeries are not enough.  They unfortunately don't last forever and cause other potential life-threatening problems.  That is why I am so passionate about advocacy, awareness, funding and research...because one day I pray that there is even more hope for these precious babies here on earth.

*most information on DILV was pulled from Cleveland Clinic