I met with my cardiothoracic surgeon this morning and am processing things but wanted to get it down here so I wouldn’t forget anything later on. There’s a lot to process.
What I have is called Raghib’s Syndrome and it’s quite rare. I have a variation of this which makes it even rarer in that I do not seem to have the atrial-septal defect usually seen in the syndrome. I do not have a coronary sinus (no worries, I don’t feel like less of a woman without a coronary sinus.)
Because I don’t seem to have the atrial-septal defect…
I may not need open heart surgery, after all.
My doc consulted with another surgeon and it seems as if I may have a narrow vessel which can take on the additional load of blood flow if they simply block off the extra superior vena cava on the left side. I may have some residual swelling on my left side due to the blood trying to work its way through a new pathway, but my body should adjust to this and even develop new vessels to help.
The next step for me is an echocardiogram followed by a cardiac catheterization, in which they thread a tube into a large vein (probably my groin) and up to the heart. They want to test the pressures in the vessels and see if there’s anything keeping them from doing the less invasive procedure. They can also inject dye through the tubing to see detail of the vessels. The surgeon will call my cardiologist and an interventional cardiologist (doc who does the cath) in order to fill them in and have me scheduled. The catheterization will be an outpatient procedure, and if they use a vessel in my groin instead of my neck, I’ll have to lay flat for about six hours.
After those tests are run, then it’s back to the surgeon’s office to see what’s next. Until then, I just keep doing what I’m doing. I’ve attached a video of the surgeon’s explanation of my weird vasculature for your enjoyment.