Your son's symptoms are consistent with congestive heart failure. This is due to the enlargement and weakening of the left sided anatomic right ventricle (L-TGA). The leaking tricuspid valve contributes to the enlargement of the left atrium and that can result in cardiac arrhythmias. Unfortunately, doing a double switch operation is a complicated, staged surgical process, and can take a long time to accomplish the goal of strengthening the right sided left ventricle to try to take on the workload of the systemic circulation. The process of pulmonary artery banding (first part of the staging towards a double switch) is often accompanied by postop heart dysfunction, which your son already has. So it seems he would not be a good candidate for that type of procedure right now. Perhaps if he improves alot in the next few months with medical management, that is something that could be discussed further, after assessing his cardiopulmonary status by cardiac catheterization. If his heart function does not improve over time, then your doctors may need to discuss listing him for a cardiac transplant. Unfortunately, patients with L-TGA behave very differently, with some patients becoming symptomatic in infancy or childhood, and others remain asymptomatic until adulthood. But right ventricular dysfunction occurs in most patients at some point in their lives. It is a difficult type of congenital heart disease to manage since there is no simple surgical approach. It would be important to discuss these options with your cardiology team.
It would depend upon what they thought would be the best long term option: replace the tricuspid valve and leave the right ventricle as the main pumping chamber to his body, knowing that he will likely develop heart dysfunction again and then need to be listed for a transplant, or attempt staging to a double switch and then if that does not work, then list for a heart transplant. Again, the results of a heart catheterization in the future will help decide his options. I do not think I can add anything further at this point.
So if his heart's function recuperates to a point where surgical intervention is possible. What are the possible surgical options.
Thank you
Unfortunately there is no clear cut "best time" for an intervention in patients with L-TGA, and in this particular situation. Doing a tricuspid valve replacement now will not solve the problem, as the valve is leaking because the ventricular function is poor. Again, the critical issue will be if your son's heart function recuperates to a point where he is well enough to be considered a candidate for some type of surgical intervention (or not).
Hi
Sorry forgot to ask something else. I keep hearing that timing of intervention is crucial to the outcome of children like johnny. When is the best time for intervention. What is the best treatment at this point,
Thank you
confusedmom
Hi
Thank you for your response, I had read that with moderate tricuspid regurgitation the valve should be replaced early to prevent any further right side deterioration. Is this accurate? What is the prognosis for children like my son?
Thanks