Options for right ventricular failure

Hi

My son is 15 years old.  He was born with l transposition.  At birth he had a small vsd and very mild pulomonary stenosis. During the past 15 years, he has had no significant problems and the echo's have been good.  About 6 months ago, he developed fatigue, palpitations, chest pain, and fainted a couple times.  His recent echo showed the following

moderately to severe right ventricle dysfunction
moderate to sever right ventricular hypertropy
moderate dilated left sided  right ventricle
mild mitral valve regurgitation
moderate tricuspid regurgitation
moderately dilated main pulmonary artery
moderately dilated left and right pulmonary artery
at least moderately dilated left atrium
patent foramen oval septal defect
left to right shunting

His stress test had a VO2max of 31 65% predicted
His 24 hour monitor revealed frequent supraventricular beats with rare atrial couplets.  Recently he has developed a significant number of bigemy with a resting heart rate of 100.

He was started on digoxin 250 once a day, enalapril 10 twice a day and a baby asprin,  My question is would he still be a candidate for the double switch procedure.  If not what are the treatment options for him and the prognosis,  I am confused because at a recent admission for his arrhythmia I was given so may  different and conflicting
treatment options that I am totally confused.  Should any other testing be done.  What is the prognosis

Thank you

[Marie M Gleason, M.D.]

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.

[confusedmon1961]

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

[confusedmon1961]

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

[Marie M Gleason, M.D.]

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).

[confusedmon1961]

So if his heart's function recuperates to a point where surgical intervention is possible.  What are the possible surgical options.

Thank you

[Marie M Gleason, M.D.]

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.