I’ve had a draft of this post before Hudson was even born… I just never got around to completing it until now….
–Chris
Three-stage surgical procedure
A three-stage surgical procedure is an option for some babies and was for ours. These operations have commonalities for every case but there are specific things that are unique for every case. These operations reconstruct our baby’s heart so that the single pumping chamber can work for the rest of his life. Hudson had the first operation at 7days old. The second operation is performed around 4-6 months and the third operation is performed around 18 to 48 months. After the operation is complete, it allows separation of the blue blood (blood without oxygen) from the red blood (blood with oxygen).
Stage one operation
The first stage is called the Norwood Procedure. Because the left ventricle cannot pump blood adequately to the body, the Norwood procedure allows the right ventricle which normally just pumps blood to the body, to now pump blood to both the lungs and the body. The procedure involves reconstructing the aortic arch (the main blood vessel supplying blood to the body) and inserting a tube (Sano Modification) that connects the aorta to the pulmonary artery (the blood vessel which supplies blood to the lungs). Most babies coming from this operation will still look bluish after this first-stage operation. In Hudson’s case, he does look pale and/or a tinge of blue at times.
Stage two operation
The second operation, called the bidirectional Glenn procedure, reduces the work of the right ventricle by allowing it to pump blood only to the body, and allowing most of the blood to flow directly from the body into the lungs. After this operation is performed, all the blood coming from the upper body is sent to the lungs so more blood with oxygen is coming back from the lungs to the left side of the heart. Hudson’s case is slightly unique here in the sense that instead of one superior vena cava (SVC) coming form his upper body into the right side of the heart, he has 2; one coming down from the left and one coming down from the right. The “bidirectional Glenn” procedure now becomes bilateral and bidirectional meaning that they will have to tie in both SVC’s into his pulmunary artery (artery to the lungs). Thinking like an Engineer, this almost seems like a better way to go – Left SVC to the left lung and right SVC to the right lung. But of course, in reality… it just means there is more “rearranging” that has to go on in his little body. A few people have told me that as a man, going through the 2nd and 3rd operations are a little tougher on the dad than the first one because of being able to interact more with their child. I constantly have the thought of how I’d feel about Hayley who is coming up on 2yrs old and how I’d feel about her going in….
Stage three operation
The third and final operation, called the Fontan procedure, allows the rest of the blood coming back from the body to go to the lungs. Now there is no mixing of blood with oxygen and blood without oxygen in his heart. After this operation, he will no longer look bluish/pale. With more oxygen-rich blood going to the body, his general health and growth will improve.
Long-term follow-up is needed to monitor the function of the reconstructed heart and blood vessels. We’ll have many follow-up checkups with the cardiac team at BC Childrens including echocardiography, an ultrasound to check the heart pump function and the blood vessel as the child grows and develops. Two heart catheterizations are typically performed over the first 2 years, usually just before each of the next 2 surgical stages, after the initial Norwood procedure.
In a heart catheterization, soft, thin plastic tubes (catheters) are placed in the large blood vessels in the leg and threaded carefully to the heart. The catheters are used to take more accurate pressure measurements inside the heart and to inject contrast or dye so pictures of the heart can be taken. Overall, they say this is a very safe test and children can go home the same day.
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I found a couple cool 3D models of a normal human heart for reference of the different arteries and veins I talk about above. I really want to model Hudson’s Heart at each stage sometime in the future. Try clicking on the model below and rotating around and zoom in/out.
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