His heart…

 

Our boy  was born with a previously undiagnosed congenital heart condition. Well, actually four – transposition of the great arteries (TGA), Ventricular septal defect (VSD), Pulmonary stenosis (PS) and sub-pulmonary stenosis.

TGA

Transposition of the great arteries (TGA) is when the two major vessels that carry blood away from the heart — the aorta and the pulmonary artery — are switched (transposed). This means that oxygenated blood is pumped around the lungs and de-oxygenated blood is pumped around the body. Essentially, instead of the plumbing creating a flow in a figure eight, Albie has two parallel circuits going on. TGA is not compatible with life unless there is a hole somewhere in the heart. Thankfully for us, that is exactly what Wrigglesworth also had.

VSD

The ventricular septal defect (VSD)is a hole in the wall between the right and left ventricles of the heart. Blue blood enters the right heart and is pumped out into the lungs. The red blood returns to the left heart from the lungs, but as it comes into the high-pressured left ventricle, some of the blood meant to be pumped out to the body is forced across into the right ventricle. There the extra blood is pumped out to the lungs. This means that the lungs see more blood volume than they should, and the body sees less oxygen rich blood than it should.

This hole in the heart was actually a great thing for our mister as it meant that at birth some oxygenated blood got around his body and some deoxygenated blood got into his lungs which stopped him from reaching critically low oxygen saturation levels.

PS and SPS

Pulmonary stenosis and sub-pulmonary stenosis simply means that his pulmonary valve, and the artery itself are much, much narrower than they should be. The valve is thick, and does not open properly. This restricts the blood flow through this part of his heart a great deal. In his open heart surgery, they will replace his pulmonary valve completely with a transplanted conduit.

What happens with all of this?

He has had four operations so far. These haven’t corrected any of his heart problems, but have just been gap-stop measures so that he can get big enough for the full open heart corrective surgery – this is called a Rastelli operation.

I wrote about what the Rastelli operation is here when Albie got very close to having it done last Christmas.

Once he has had his Rastelli repair, he will be a great deal better. He will need pulmonary valve replacements (via open heart surgery) as he grows up.