A Look into Reiss’s Heart and Q&A with the Doctors

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A Look into Reiss’s Heart

After Reiss’s surgery was set, we wrote down all of our questions as we wrapped our minds around what was in Reiss’s future – you can read many of those questions, as well as the answers to them below.  We set up a meeting with our local pediatric cardiologist to go over the results of the MRI.  He presented us with a drawing of what her heart looked like, based on the MRI and 3D echo.  The picture below shows a normal heart on the left, and the drawing of Reiss’s heart on the right. I sent this picture to Dr. E at St. Louis Children’s Hospital to confirm if it was accurate.  His response: “I think this is pretty accurate. The only caveat being the location of the VSD, which would be hard to draw in two dimensions, is not so close to the great vessels and is more remote.”  The picture also shows where the Glenn would intersect the SVC to the pulmonary artery.

The diagram of Reiss’s heart is a little different than we first posted before Reiss was born.  The aorta and pulmonary arteries are still coming out of the right ventricle – but the new thing is that the right ventricle actually sits on the left side, as mentioned in our last post.

heart diagram

Q&A

Talking with Dr. E, St. Louis Children’s Hospital.

From June 29th via email.  Dr E’s responses are in red

  1. Is a heart transplant inevitable for single ventricle patients? No, not at least up to now but vast majority of “old” SV patients are only in their 30s to 40s. Depends on the lesion: overall about 15to20% but higher proportion are single right ventricles than left; very little data on kids like Reiss who have both. You might extrapolate they would do even better, but don’t know for certain. 
    1. When do the majority of patients like Reiss have to have a heart transplant? Depends on the lesion, but for those that make it through the Fontan, there is a continuous phase through adulthood.
    2. What is the success rate of heart transplants for that age group? Again, depends on starting point/lesion. If they are sick Fontans who have had multiple surgeries they don’t do as well
    3. How hard is it to get on the transplant list? Depends; it is a multi-disciplinary selection process and there are a number of criteria that have to be considered.
    4. How long do you normally have to wait for a heart?Depends on age, size, blood type, etc. Not a fixed number
    5. What is the recovery time for heart transplants? Depends on condition going into it.
    6. Do heart transplant patients have limited mobility/activity/etc? No, not necessarily. Some are excellent athletes.
    7. If the transplant is a success…what is the failure rate down the road? Again, depends at what age and what complications, if any, rejections, etc.
  2. When deciding between Fontan and 2 ventricle option when Reiss is 3-4 years old, will she be better off with the Fontan/heart transplant option or try to fix it with the 2 ventricle option? Which has the least consequences based on her situation/anatomy? We have to see where things stand when she is 3 to 4 years of age.  A good SV is better than bad 2V
  3. Can a single ventricle heart patient be active in sports? Yes
  4. Should we limit Reiss’s activity between Glen and Fontan?No
  5. Do Glen patients have to be on medication? If so, which ones?Short term, yes (Aspirin, maybe blood pressure meds and diuretics). BTW, this would be the case also with 2V pathway.
  6. Do Glen patients have to be on oxygen?Not necessarily. Doubt it for Reiss
  7. Do Glen patients have to be on any other type of monitor daily or weekly, etc?No
  8. Do single ventricle heart patients (post Fontan) have to be on medication long term? If so, which ones?Maybe; we still don’t know and practice patterns differ. It might be beneficial to be on some meds but not all physicians keep patients on meds long term and again underlying diagnosis and heart condition/other factors are important to take into consideration.
  9. Do heart transplant patients have to be on medication long term?Yes, for ever.
  10. If Reiss has a Glen – what are possible side effects/risks? Problems with the anastomosis/need for intervention on those; injury to right phrenic nerve; transient head swelling; risks common to all surgeries (infection, bleeding, need for blood transfusion and risks from heart lung machine).
  11. Does Reiss need to be on any special diet?No
  12. Does having any surgery – whether that be Glen, Fontan, double ventricle, heart transplant – affect development?Depends on whether there are complications such as stroke or need for hemodynamic support with ECMO. Also, younger children/babies are more susceptible. Reiss is fortunate in that she is much older than typical patient
  13. Does any of the surgeries affect other organs?Yes, they all can, mainly related to the amount of time on the heart lung machine. Longer surgery, greater the risk of injury to any of the organs specially the kidneys
  14. What will Reiss’s “normal” oxygen level run after the Glen? 75 to85%, I.E., same as now. Will this affect her brain?Not as best as we know.
  15. What will Reiss’s “normal” oxygen levels run after completion of the Fontan? Low to mid 90s Will that affect her brain? Don’t know but doesn’t appear based on clinical experience (I don’t know if anyone has specifically looked at this question and how they would answer it)
  16. Other than the possibility of a heart transplant later on, are there any other possibly procedures after completion of the single ventricle pathway that we need to know about? Any surgery, can lead to need for other interventions or surgeries. For example, some kids with SV pathway can end up with other complications such as plastic bronchitis or protein losing enteropathy (though many have other issues such as heterotaxy, etc.). On the other hand, going down to 2V pathway has the risk of heart block, need for pacemaker(s), guaranteed need for repeated surgeries/interventions for the conduit, risk of outflow tract obstruction, etc.

From July 8th via phone

Dr E response in bold.  These are my notes as Dr. E was talking.

  1. With the Glenn – will one lung get more blood than the other? Is this a problem?  The answer is yes. Typically the right lung gets 70-75% gets more of the flow.  In some cases, it could be a problem, but the Fontan addresses that issue.
  2. How do Reiss’s pulmonary arteries to the lungs look? Are they the same size? If they are differing sizes, does it pose a problem for her as she develops if one is smaller?  If they are substantially different, it could be an issue.  Usually not a problem, however. Reiss’s pulmonary arteries look good
  3. Is there anything wrong with her lung growth? In January, Dr. Murphy (who widened the stent) mentioned one lung was smaller than the other.  Grossly looking at it, it doesn’t look like there is an issue.
  4. How many Glenn procedures have you done? What is your success rate? Have you performed a Glenn on a heart like Reiss’?  100’s.  each year 20-25. In the last 15 years, 200-300.  Success rate – never had a mortality with a Glenn. Never taken down a Glenn   Once needed a shunt in addition to Glenn   Yes, he has performed a Glenn on a heart like Reiss’.
  5. I know you mentioned that we were focusing too much on a heart transplant – but we like to have as much information as possible on all possible outcomes.  Do heart transplant patients have a higher risk for cancer due to anti-rejection meds? Possibly skin cancer far down the road.
  6. If she did have a heart transplant, would she need a lung also? Most likely no
  7. Will you definitely be the one to do the surgery? Yes unless we (Roy and I) chose someone else.
  8. Will Reiss be on some sort of bypass machine during the procedure? If so, what is it called and what are the risks? May not need a heart lung machine. 50/50 chance may not need heart lung machine. Risks of those with the heart lung machine are pretty small. 5-10 hours on bypass is when you start to see problems. Bypass is a pretty safe tool.
  9. Do remove the stent? Or tie it off? How does that work? Tie off and divide.  Tie above and below and cut in between. 
  10. Reiss has an infarct on the right side of her brain. Can we get a checkup while we are in the hospital with the neurologist (she saw one when she was born)? He did not see this at the time of the last MRI.  If he would have, he would have asked for an MRI of the brain.  We could do an MRI prior to surgery, but the likelihood of that having an affect on this surgery is quite small.  If we have the MRI after the surgery, and we see a difference in the 1st brain MRI from the 2nd, then we will not know why there was a change – was it time between MRI’s? or was it the surgery. Regardless, the fact that you won’t know what caused it will not make a difference on future outcomes.
  11. Does the surgery have any effects on her infarct – or vice versa? Probably not. During a Glenn, pressures inside brain go up for a short amount of time.
  12. Should my husband or I plan to donate blood for the surgery? Wouldn’t worry about it. Blood bank does not allow for direct donation any more. No better/no safer/huge cost.
  13. How long with the surgery take? 4-5 hrs.  from the time we leave her to the time she is back in the ICU
  14. Will any foreign material be used? Most likely, no.  If there are problems with where the stent is tied off, if it isn’t closing up easily, they will put a patch in the area.
  15. Will Reiss be on any machines directly after the surgery? Which ones and for how long? 1) Ventilator for a period of time depending on how long it takes for the drugs to wear off – could be 24 hours. However, 99% of time the ventilator is out after surgery.  2) Heart lung machine/bypass machine – do not expect. Emergency only.
  16. Will she be sedated after surgery? Yes. To make comfortable. They do have headaches after the surgery. They give them pain meds and sedate to keep happy, comfortable
  17. Will we be able to hold her? Or only hold her a certain way post surgery? Yes, after all drains/tubes are removed. Scoop her up instead of from under the arms. – 24-48 hours out.
  18. Can she wear her own clothes post-op? Eventually, yes. Once settled. Could get fluids on them – cleaning fluids, blood, etc. after 2-3 days we will not have to worry about clothes being stained or contaminated.
  19. Can we have visitors? Yes, ICU. Parents 24/7. 2 people at a time in the room. On “the floor”, same.
  20. Is there a risk for arrhythmias after the Glenn? If so, how are they treated? Rare. Sinus node dysfunction could happen. Sinus node – tissue at top of right atrium where SVC goes into it. Can be damaged or injured during the procedure. The sinus node is the natural pacemaker of the heart.  They evaluate for a minute or two to make sure it is intact at the beginning of the surgery.  Sometimes need a pacemaker <1% of time. Any other arrhythmias – would be because of some abnormal tissue they don’t see.  Stress of surgery make bring them out. Treated with meds sometimes with catheters.
  21. I hear that Reiss may suffer from migraine headaches for a couple weeks after the surgery.  Is this true? Please explain. No, we make the assumption they have a headache because head is swollen.  They do not have the head ache for a couple of weeks. More like first 4-5 days. End of the week, kids go home with ibuprofen or tylenol.
  22. Will she be on pain meds? Afterwards yes. Once drains and tubes are removed, they decrease the amount of pain meds. They go home with Tylenol.
  23. What is our expected length of stay at STL Children’s? Will we be in the CICU the whole time? About 1 week.  Have had kids leave anywhere from 4 to 10 days after surgery. Average is 7 days.  Typically, 1-2 in icu, another 4-5 days on the floor.  Older the kids, the better with the glenn. Some sail through. 1:2 day ratio ICU:Floor
  24. How long can we stay with Reiss prior to surgery? How soon can we see her after? Prior to surgery – stay with her until they take her to the operating room. See her after when she gets back to the unit.
  25. What changes should we expect to see in Reiss after recovery from the Glenn?  Increase in SAT’s, increased growth, increased energy?  How will the Glenn surgery improve her health or quality of life? Stats wouldn’t change. Growth will be the same maybe a little better. Maybe a little more energy.  Glenn protects her lungs. Quality? She needs another step other than the glenn in order to assess that.
  26. Based on what you know of children similar to Reiss, can you tell me if Reiss will be able to swim, run, fly in a plane, etc.  In what ways will she be limited? Both after glen and after fontan (assuming that is the route we go in 3-4 years)? Yes to all of the above, but it is variable. Some kids do remarkable well and can be active.  Others have limitations.  If they are active as a child and stay active as an adult, they do better in the long run.  May not be an olymjpic athlete, triathlete, etc.  May not be able to do varsity sports and competitive at a high level of athletic endurance.
  27. What is the best current prediction for life expectancy for Reiss? With both ways (either single or double ventricle pathways) – reasonable to think 30-40s. Beyond that is a crap shoot.
  28. Will she be able to have kids? Maybe.  Assuming she is fertile, no reason to think she is not. Yes, she would be considered higher risk.
  29. What is the chance her kids could have this or any other heart defect? Little more than average pop, but not substantially more.  Baseline is 1 out of 100. Cannot quantity.
  30. What is the change that if Roy and I decide to have another child that it would have this or any heart defect? Approx.. 1 out of 50. Recommendation to get fetal ultrasound. Could see genetics counselor. Odds are in our favor that we would not have another child with a heart defect.
  31. Can you recommend any reading materials geared toward non-doctors so we may learn more about Reiss’s heart and future? Don’t think with her particular heart defect, that there is anything out there.  Reiss’s heart condition is more unusual. 80-90% are correct in terms of atrioventricular discordance.
  32. Having a heart defect – will that stunt her growth at all? Yes and no. in short, without question, her circulation is not efficient. She has had reasonable growth, though. Regardless of the path, there is always inefficient circulation. There is also significant variability from case to case – Some are stunted, some do well. Some have a genetic component which plays a part in growth. Some have a hormonal deficiency and do not grow well. Only way to know – with adequate calories, see where they go. Most kids with congenital heart defects tend to have slower growth or less than normal.

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2 thoughts on “A Look into Reiss’s Heart and Q&A with the Doctors

  1. Do you follow Pray for Caleb on facebook. His parents are kickapoo grads of 2002 and caleb is a heart baby. Caleb Just received a heart and was transplanted. His dr is dr E too at childrens!! I have told desire about you!! I can only imaginé the stress you heart mommas go through. Praying for Reiss.

    1. Yes, I have seen posts about Caleb. It is SO wonderful he has a heart! However, I do not read the posts on his page too much – it is too hard sometimes.

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