So here is the low-down on our boy’s heart. But before I get going on this, I want to tell you that this is just one part of our story. Its a big part, no doubt about it, but his heart condition does not define Albie. It just draws us closer to what really matters in this life – and that my friends is love, love, love.
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.
Each of them on their own is quite treatable. But together, they add up to a much bigger deal.
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.
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, and his artery doesn’t allow enough blood through either. In his open heart surgery, they will replace his pulmonary valve, and part of his artery completely with a transplanted conduit. This will need to be replaced as he grows.
Albie had a balloon septostomy, a failed cath and a BT shunt placed all within his first six weeks of life. These didn’t correct any of his heart problems, but were just gap-stop measures so that he could get big enough for the full open heart corrective surgery – a Rastelli operation.
I wrote about what the Rastelli operation is here when Albie got very close to having it done around Christmas 2011.
Albie had his Rastelli operation on 14 August 2012. He had a 16mm Hancock conduit placed to replace part of his pulmonary artery and his pulmonary valve. The rest of his heart was patched up. He was recovering really well from the operation until the next morning when a routine monitoring line was removed from the outside of his heart. This caused a cardiac arrest and his heart went into severe dysfunction. His medical records read that he had what is called a ‘Pulseless Electrical Activity Arrest’. Basically they did not know if his heart would ever work again. An ECMO machine did the job of his heart (and lungs) for him for four long days and nights – the most frightening of our lives. I am holding my breath right now as I type.
Albie pushed through it all – with astounding tenacity. He suffered some small brain hemorrhages from the arrest and his time on ECMO, so its likely there will be some developmental delays. But he is rocking it nonetheless.
Hopefully his Hancock valve will last for around eight years. For some kids it only lasts one year. Its just a wait and see game. As is all of this.And then we’ll do it all again. I am keeping such thoughts far from this thankful mind.
Oh – and Albie had a nasal-gastric tube from April 2011 till after his Rastelli operation in August 2012 – 16 months. He needed it because his blood circulation was crazy inefficient and he burned up lots and lots of calories.
Butterflies, from the Cardiology Department at the Royal Children’s Hospital, Melbourne, Australia.