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Samantha was BORN with a condition called Heterotaxy that caused several heart defects which she has undergone 3 open heart operations to correct. The first was the BT shunt. The second, the Glen. The third was the Fontan. ALL of these operations were successful...
Samantha NOW has developed this condition/disease of Pulmonary Vein Stenosis. There are several degrees of the severity of this disease and Samantha's is one of the most aggressive and severe. This disease is extremely dangerous and does not have good survival odds. Samantha's anatomy is very unique and therefore, there is no previous case EVER like Samantha's to draw data from. The only option for Samantha was surgical intervention by way of the Sutureless Vein Repair. The repairs odds of longterm success are 30%. What that means is that after 5 years, only 1 out of three patients with this repair are still alive.
If this vein repair does not work, the only other option is a Heart AND Lung transplant. This disease removes any possibility of a Heart Only transplant. Heart-Lung transplants are extremely rare. Only 4 Hospitals in the country are capable of doing this and the closest is Philly. We have been advised that we should not actively persue getting Sami on the transplant list until there are signs that the desease is reoccuring. If Samantha does not have reoccurrence of the vein stenosis, and it does not spread beyond the repair, she will not be placed on the list. If things worsen and she gets on the list, it is at least a 2 year wait before you get to the organs.....if you get them. And given how young Samantha is, it is even more rare.
During Samantha's 4th operation, in order to get to the Pulmonary Veins to repair them, the surgeon DID have to reverse what they did during the surgery in January - the Fontan. The reason is that the "Fontan circuit" literally sat directly in front of where the 4 pulmonary veins connect to the heart.... To clarify, if they were able to work around the Fontan and do the Pulmonary Vein repair, they would have. The success of the repair did not rely on the Fontan being reversed. It was just simply in they way. And, the repair was more important, and actually life saving for Samantha to be taken back to the "Glen" stage and have the repair vrs keeping the Fontan and not having the repair. Also, they felt that taking down the Fontan, repairing the veins, then redoing the Fontan all in one shot was just too many major reconstructions for one surgery and it would have made her recovery longer and/or harder.
We will be redoing the Fontan surgery at some point in the future, unless the need for a transplant happens first. Right now we will be watching to see how her Pulmonary Veins react to this repair. The thought process is that we will let her go as long as she is doing well. If her Pulmonary veins are still doing well in 2 or 3 years, then we will assess the need for the Fontan. Or, if she starts to "get blue" before then, we will assess the need for doing the Fontan.
The plan right now is more or less the standard recovery plan. There are many hurdles for Samantha to overcome on the road to recovery. She has ALWAYS amazed us with her strength and speed at recovering from these operation. The good news about being back to the Glen, is that there is much less pressure in the heart and lungs and therefore, the fluid production should decrease much more quickly and therefore the chest tubes will hopefully come out in a much shorter time frame. Time will hopefully prove this correct.