(Joshua - 4 months pre-Glenn)While there are many congenital heart defects, Joshua unfortunately has one of the most severe, if not the most severe. He has hypoplastic left heart syndrome, or HLHS. I still remember when the doctor who diagnosed him uttered those words & thinking to myself, "huh? Was that English?" Then a moment later I realized that incredibly long name would become a regular in our vocabulary. I don't often think back to the day when we found out because it was really tough. The short version goes something like this; I got a phone call from genetics the morning after our 20 week ultrasound. The woman wanted us to come back in immediately for more testing because it "appeared that a portion of the baby's heart looked smaller than the other side."
HLHS is as simple as that (if that is simple). Joshua's left heart is severely underdeveloped. Therefore, his heart required surgery at birth in order to support life. Other options included transplant, pallative care and termination. We chose to have Joshua undergo the Norwood procedure, as we felt that it offered the best shot at life. The Norwood procedure consists of 3 stage surgical intervention. The Norwood at birth, the Glenn at a few months old (Joshua was 4 months) and the Fontan during the toddler years (or there about). The result of the procedure allows the heart to function with a single ventricle; the right ventricle in Joshua's case. It's actually pretty fascinating the way his body functions right now. The blood flows passively to the lungs from his upper body & then goes to the heart where the right ventricle then pumps the oxygenated blood to the body. After he has his Fontan the blood flow from his lower body will also be directed to the lungs & then to the heart, allowing him to function with higher O2 levels than his current state, which is at around 80-85%. If you know Joshua, you may have noticed that he looks a little bit blue. After he has his Fontan his O2 levels should be in the high 90's, and hopefully his lips will be pinker!