My son is 15 years old and was born with L-TGA. He had a pacemaker implanted when he was 6 years old. He was diagnosed with a platelet disorder, where his blood has a hard time clotting. He is given desmopressin before any surgery or proceedures.
My first question is, at his yearly check-up his cardiologist informed us that he does not have much slack on his pm leads and with him continuing to grow, we are looking at having to replace them soon. Since we will not see him again for another year, what would happen if he grew to where the lead dislodged? Would there be a chance that he would be in danger? How would we know this happened?
My second question is that he has a leaky tricuspid valve, that they continue to watch. His cardiologist is suggesting that we could need to replace it soon as they really do not have anyway of measuring how the ventrical is doing. Since he has a bleeding disorder, his Dr. suggests that this is a risky proceedure and he doesn't know if a mechanical valve would be an option. At what age does the heart with L-TGA start to fail? Everything with this condition seems to suggest that you need to act before it starts to fail.
Any information you could give would be greatly appreciated.
I cannot say exactly what will happen if his pacemaker lead dislodges, since I do not know how dependent upon the pacemaker your son is, or why the pacemaker was placed. Your cardiologist should be able to tell you this, though. There are several different reasons why pacemakers are placed, so knowing why your son has one would be important. Often, when a patient with a pacemaker undergoes routine pacemaker interrogation in the clinic, the cardiologist is able to stop the pacemaker to see what the underlying rhythm is. At that time, he can tell your son and you that it is off, so he can see what it feels like. However, if he is completely dependent upon the pacemaker and has a very sick electrical conduction system, this may not be recommended. If that is the case, the pediatric electrophysiologist would be able to tell you the right timing of replacement of the lead. When a pacemaker is implanted, an extra length of pacemaker lead is put in to accomodate growth. However, growth can certainly cause it to either dislodge or fracture, rendering it nonfunctional.
With regard to his tricuspid valve regurgitation, this is something that is often seen in l-TGA. Placement of a prosthetic tricuspid valve has not been demonstrated to be particularly helpful in these patients over the long term, unfortunately. Other interventional options have included placing a band on the pulmonary artery to increase right ventricular pressure and change the geometry of the left ventricle, thus decreasing the tricuspid regurgitation. Finally, the performance of a "double-switch" has been done, in which the atria are surgically directed to the opposite ventricles AND the great arteries are switched, as well. In the experience of the group that had previously done the most of these, these were best done before age 16 years. Sadly, the long term outcomes for l-TGA are not great if the systemic ventricle is failing, and may eventually require a cardiac transplant. There is no one age that the ventricle fails--it varies widely among patients, actually. However, it is important to be aggressive about preventing the failure.
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