We are between a rock and a hard place. It seems like any decision we make has pros and cons. It's frustrating to feel like your settling, when in reality you may not be settling, but you just don't know so that's what it feels like. To make a decision that effects your son's life, in a life and death sort of way. We are praying, but peace is not coming with either decision.
Many people have asked what exactly is wrong with Levi's heart. Levi's heart diagnosis' are Double Outlet Right Ventricle (DORV), Complete Atrioventricular Canal Defect (CAVC), and Pulmonary Stenosis (PS).
With DORV, the Aorta and the Pulmonary Artery are both attached to the right ventricle. This is in contrast to the normal heart, where the Aorta attaches to the left ventricle and the Pulmonary Artery attaches to the right ventricle. Blood from the left ventricle, which would normally leave the heart through the attached aorta, now must cross an abnormal hole in the wall dividing the right and left ventricles, called a Ventricular Septal Defect (VSD), in order to leave the heart and supply the head and body with blood. In this condition, the normally separated oxygen-rich arterial blood and oxygen-poor venous blood is mixed in the right ventricle prior to leaving the heart. Also, the normally low pressure right ventricle can be subjected to increased pressure from the normally high pressure left ventricle.
The CAVC is a second hole Levi has. This large hole is in the center of the heart affecting all four chambers where they would normally be divided. When a heart is properly divided, the oxygen-rich blood from the lungs does not mix with the oxygen-poor blood from the body. A CAVC allows blood to mix and the chambers and valves to not properly route the blood.
His PS is above and below the Pulmonary Valve so not much blood can get through.
When we adopted Levi we were told he would need the Fontan due to an underdeveloped left ventricle that was not functioning. We were at peace with this. But then they found his left ventricle was developed and functioning fine. While it is smaller than the right side it is functioning. Everyone was so happy because everything lined up for Levi to have a biventricular repair.
But when the surgeon got inside Levi's heart he felt a biventricular repair was impossible.
"Looking in, there was noted to be a complete atrioventricular canal, right dominant with a somewhat smaller left ventricular cavity. However, the important feature was that the ventricular septum inserted more rightward and anterior to the aorta such that any attempt to close the VSD would bring the baffle across the left ventricular outflow tract, nearly obliterating it. It was clear that there was no way to do a 2 ventricle repair and wind up with an inadequate left ventricular outflow tract. Therefore, the decision was made to abandon the possibility of a 2 ventricle repair and proceed with a pulsatile bidirectional Glenn." (quote from surgeon's medical report)
He said his Fontan would be very good because even though his heart would work as a single ventricle, his left side is there to help the right. This is usually not the case with the Fontan. Usually the left ventricle is not functioning and the right ventricle works alone. With both sides working together the Fontan may last longer. He may not ever require a heart transplant. We just don't know.
We have been told by some to get a second opinion from a doctor in Boston who specializes in biventricular repairs. We did contact him and he was more than willing to review Levi's files. But after reading the surgeon's report we are not sure the outcome would change and we don't want to put Levi through more tests and surgeries "incase" he could have a biventricular heart. Or what if a biventricular heart would not be as safe in the long run because of his complex defect. Sometimes a biventricular repair can be done but will require more open heart surgeries down the road to replace parts that your child out grows. Ones heart and body can only handle that so many times. The outcome would still be a transplant if the heart couldn't handle the surgeries. So do you go for a Fontan that has it's own set of complications with the possibility of a transplant down the road or a biventricular repair that could require open heart surgery every few years and still end with a heart transplant?
I wish it were a cut and dry decision. It is not. We have decided to request a second opinion, hoping the doctors are of the same agreement as to what should be done. We know God knows what is best for Levi. We are glad He has gone before us. Please be praying we hear His voice. That we choose the path He has for Levi.
Romans 15:13 "I pray that God, the source of hope, will fill you completely with joy and peace because you trust in him. Then you will overflow with confident hope through the power of the Holy Spirit."