Pediatric Heart Expert Forum
fontan surgery
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fontan surgery

Thank you very much for the answer. she had DORV, PS, VSD, ASD. Fontan intracardic surgery done with mitral valve repair. Is the pace maker can solve the synus dysfunction?, what about the ventricular function in the future? does it give her any problem. Is there any precuations to be taken?.

Thank you very much again.

howwa
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773637_tn?1327450515
Dear Howwa,

I would reference our readers back to the previous posting which I responded to dated May 18, 2009, for details of our initial discussion.  For the rest of our readers, DORV is short for double-outlet right ventricle. A right ventricle that is recruited into being the systemic ventricle has a lot of extra work to do.  From an architectural as well as a cellular standpoint, the right ventricle was never meant to be a systemic ventricle.  There is some data to suggest that patients who have undergone the Fontan palliation who have a true left ventricle as their systemic ventricle do better than those with a right ventricle.  Overall, though, it is difficult to say how long the ventricular function can hold out.  The Fontan was first described in 1971, and was only for a very specific heart defect called tricuspid atresia.  As time as gone on, the Fontan procedure has been modified numerous times to be able to function better, to reduce some of the complications, and to be used in other types of single ventricle hearts.  Thus, you can do the math—we don’t have a very long term set of outcomes using the most up-to-date kinds of surgical techniques in these patients.  We think that we’ve done better for them, but we know that there are plenty of long-term complications besides the sinus node dysfunction.  These include liver dysfunction, increased risk of forming clots in the bloodstream (increased risk of stroke and other clotting complications), protein-losing enteropathy (a condition where the intestines actually leak protein and can cause malnutrition), and other heart rhythm abnormalities.  If her ventricular function declines, she can be treated with medications that would be used for patients with congestive heart failure, in which the pumping function of the heart doesn’t meet the needs of the body.  I will say, though, that we have certainly seen patients with the Fontan come to heart transplant.  That said, there are also plenty of Fontan patients who have grown up without significant complications, get married, and even have children.  I don’t think that we know of any specific precautions, other than to make sure that she is getting good aerobic exercise on a daily basis, in order to improve the reserve of her heart, and to make sure that her teeth are kept clean and that she sees the dentist on a routine basis, in order to prevent an infection of the heart called endocarditis.  Finally, the last precaution is making sure that you (and, eventually, she) know that her heart condition requires lifelong cardiology follow-up and surveillance with a pediatric cardiologist, then an adult congenital heart disease specialist when she becomes an adult.  

In regard to sinus node dysfunction, pacemakers can overcome the problem of the dysfunction, though it doesn’t actually heal the sinus node.  It stimulates the heart to beat at a higher rate, as programmed by your child’s doctor.  As mentioned in my previous response, the recommended published consensus criteria for pacemaker placement is one of our best sources at this point for guidance on when to put in a pacemaker.  The other source would simply be her symptoms, especially if they start to worsen.
2 Comments
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306245_tn?1244388567
Hi how is your daughter doing. my son was 3 months old when he had a large VSD, and a FPO and a Patent Ductus Ligation done. he is having problems sometimes when he is walking around his HR will drop in to the low 40's he has has a EP study, but they increased his HR and couldn't gt him into an arrythmeia, they have done several holters on him and on time they said his HR was 48 and he was sleeping another time they got it as high as 198. they saw him running in the office get really tired and his Hr dropped to 50's they said something was srong with the machine. so I don't know. Finally after all these symptoms, and basically saying I want something done, i feel I am going to loose him his pediatric cardiologist says he does need a pace maker his does okay have issues with the sinus node. he said being symptomatic with passing out once and coming close to it with a low HR would qualify him for one, this cardiologist isn't equipped to do it in some one his age, 5 years old. Boston children's said not enough info has come through and to put one in a person of his age is tough on the child so what they have done is put a "implantable loop recorder" reveal monitor, in him this is like a holter/event monitor. they set the marks so if his HR goes below 40 or above 200 it will record, and it will record arrythmeias and pauses that he has. if I see anything unusual then I record it and it will go back 6 minutes and record for  minutes after the button is pushed. he has these symptoms every 5-6 months.
I hope that you can get some answers soon
good luck
michelle
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