Many people have asked about Blaise's heart, and how it's developed, what the surgeons are doing and what the future holds. I'll do my best to explain what we know and what we've learned. Trust me, I definitely did not pay enough attention in my high school anatomy classes, but have totally relearned the heart structure. We studied up well in advance on what we found out through the ECHO's done in utero, were given "maps" of his special heart by the doctor that they drew out for us and also maps of plans for surgery. After Blaise had his heart cath done last Thursday, more developments were discovered- most specifically the structure of his coronary arteries, which further complicated matters.
Mike and I have realized that Blaise's heart structure does indeed fall on the most severe end of the spectrum when it comes to heart defects. We are often asked what the plan is to "fix" his heart. Unfortunately, there is no way to actually "fix" his heart, but instead go through a series of palliative surgeries which allows his heart to function in a different manner than a normal heart.
For those science buffs- here is an explanation of his heart structure: In a typical heart blood flows through the right ventricle to the pulmonary artery, and on to the lungs. In Blaise's heart, the valves in his pulmonary artery did not develop, thus causing the blood to enter his right ventricle and then not have anywhere to go. The strong pressure of this blood entering and not being able to leave caused coronary arteries to form between his left and right ventricle, and the coronary arteries are what flow blood to the heart muscles to make it pump. Thus, Blaise's right ventricle is coronary artery dependent. Because of this, the surgeons are not able to open up any of the valves in his pulmonary artery, because this change in pressure for his coronary arteries would be fatal.
The plan then, is to redirect the blood flow in his heart so that oxygen rich blood can reach his lungs through a different method. This is done through a three step, open heart surgery process.
Step 1: BT SHUNT: COMPLETE! Blaise's first round of surgery was to put a very small vortex shunt in his heart, directly allowing oxygen rich blood to reach his lungs. This shunt, however, does not grow with Blaise, and is only big enough to last until he is about 4-6 months old. They are unable to put in a larger shunt, because too much oxygen rich blood at this point is also a bad thing. To date, this first step of surgery has been largely successful, and one of the heart surgeons said this morning that the first 72 hours are the most critical so we've passed that point. However, the next six months are just as critical. This shunt can clot at any time, which would be fatal. This is why we will be giving him two shots of blood thinner each day, tracking every single one of his feeds, his weight gains every day and his STAT levels every day from home. They have learned this is the best way to see if their are any "signs" of the shunt not working.
Step 2: Glenn: In this process at 4-6 months Blaise will undergo open heart surgery and also be put on bypass. With his coronary artery situation, going on bypass and this surgery will be very serious. Here, the surgeons re-hook up the superior vena cava directly to pulmonary artery leading to the lungs, alleviating the need of the BT Shunt.
Step 3: Fontan: This is done at about 2 years of age or when he reaches 30 pounds. In it he will again go on bypass and the surgeons will finish connecting more of his heart directly to the lungs, finishing off what was started in the Glenn.
End Result: Blaise will function as a single ventricle heart, where most adults are a two ventricle. This surgery process has only been around for about 20 years, so long-term statistics are hard to determine. In addition, his coronary arteries also play a big role, as the pressures will need to remain the same in his heart.
The good news is God must have known what he was doing when creating Blaise's heart because so far, Blaise's heart seems to be working just fine for him! The doctors and surgeons think he is doing great, and we couldn't be more proud of him and how he is doing. We are thankful everyday for this wonderful gift and continue to give praise to God for all he has done for us. The many prayers being said daily are so uplifting and encouraging and have definitely been heard!