ccTGA - Questions / Future

[jasontroj]

I am a 26 yr old male with ccTGA, repaired VSD and conduit in 1989.

My recent cardiologist visit yielded; through MRI, that my right ventricle is fairly dilated, my RVEF is 23%, and I am having more and more PVC's. Seeking a second opinion I visited my Pediatric Cardiologist and he had mentioned that he saw no changes in my echo other than the frequency of PVC's. Upon revisiting my Adult Congenital Cardiologist (yesterday) he is recommending me to a Heart Failure specialist for medications and a visit 12mos. from now.  He also noted that since this was my first MRI that things may have been like this for a long time and may just be the way things work for me.

It's hard not to look at what is all over the internet. But not everything is reassuring when I do.

I have a lot planned in life, time with family, engagement, etc. and I am fearful that this is all just the begining of the end. Has anyone any experience with ccTGA and what I could/should expect in the future? Will this just morph into CHF and the necessity for a transplant?

[Cleveland Clinic]

The right ventricle is not intended by nature to deal with high pressures such as those in the systemic circulation.  Even though, your transposition is congenitally corrected, the ventricles are still switched such that the right one pumps blood against the systemic circulation and the left one pumps blood against the pulmonary circulation.  Unfortunately, prolonged exposure to systemic pressures on the RV causes it to fail.  This is what normally happens in the ccTGA patients and many of them require transplantation.  The fact that your functional capacity is relatively unchanged is reassuring, however, the frequent ectopy really worries me.  It might mean that the ventricle is under a lot of stress.  I am not an expert in the field of adult congenital heart disease, however, I would urge you to seek a second opinion regarding your further care.  I know that Mayo clinic and the  Brigham Hospital both have excellent adult congenital heart disease programs.  We also have a few very experienced cardiologist here at the Cleveland clinic who deal with this as well.  

[jasontroj]

I just want to add that before these tests my biggest concern was what movies I wanted to rent on Netflix and what I wanted to do on the weekend.  I have no symptoms otherwise.  Exercise capacity is lower, but I never was marathon runner to begin with.