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I put ** behind the blog post title because while we do have some answers post testing, there is a qualifier.  There are answers but they are not definitive.


Gaining Access

As mentioned in previous posts today, access into Reiss’s arteries with the catheter was an issue.  Her cath procedures at 7 days and 3 months left scar tissue in the arteries on both legs, causing the arteries to be blocked.  She luckily still has great blood flow to the legs, due largely to her body’s ability to grow collateral vessels to redirect blood.  Unfortunately, these collaterals make it very hard to weave a catheter through the twisting maze.  See this maze here.

Answers from the Cath

We talked to Dr. Murphy about what he saw while in the diagnostic cath lab.  After he talked to Dr. Coste about what he found during the flexible bronch scope, he was looking for collaterals that may be the answer to the bleeding and also the red, irritated area just below the cauterized tissue from the scope in Springfield last Wednesday.

Collaterals:  Dr. M did find some collaterals.   There was one arterial collateral that traveled near the trachea – near the cauterized portion of the airway.  Dr. M blocked this off with a coil and hopes that this was the source of the bleeding.  He also found a venous collateral that was taking blue blood away from the Glen and back to the heart – bypassing the Glen.  Dr. M blocked this off with a coil.  There was one more collateral to the lungs that was large enough to draw attention, but upon injecting the dye into the vessel, they found that it was too narrow to place a coil.  He hopes that this one will just close off over time.

Blood Flow to Her Lungs:  When they injected the dye through the lungs, they saw all the lung fields and branches they needed to see.  Additionally, they filled and cleared uniformly, which is good.  View a video of what Reiss’s lungs look like here and a short explanation from Dr. M.

Pulmonary Artery:  After looking a the heart in the echo from last week, the cardiologists were worried there was an issue with the right pulmonary artery.  They did find that the right pulmonary artery was indeed narrow, but the placement of the narrowed artery was near the glen and too near a branch into the lungs.  This awkward placement did not bode well for a stent or ballooning in today’s procedure.  Dr. M decided to leave it as is and continue with the procedure.  If the cardiac surgeons wanted to intervene with this artery later, they could do so at her next open heart surgery.  The pressures measured the same into both lungs, which is good.

Issues with blocked arteries in terms of future cath procedures and access points: Reiss shouldn’t have any complications with arteries being blocked in her legs.  She still has a good pulse and blood flow.  Most likely, however, while there are cases where the blocked arteries in the legs would be able to be used later on, she probably will never be able to use them during a cath procedure.

They did have to go in through her right carotid artery to gain access.  She had to lie flat until 9pm so that the arteries in the legs where they tried to gain access and the carotid do not bleed out.  (This is easier said than done, even with sedation, with a 2 year old.)  They have a NIRS patch on her head monitoring the pulse ox coming from her head back to her heart.

NIRS monitor
NIRS monitor

[Side note:  this will always be 20% lower than the regular pulse ox since it is measuring how much oxygen your body has used up as it flows from the heart, out to the body, and back to the heart.]  Reiss was on heparin – a blood thinner – during he procedure to prevent clotting and will continue to be on a blood thinner at home.  They will do an ultrasound of the access point on the neck in the morning to make sure everything is okay with the wound before they discharge us.

Will future cath procedures have to use this carotid access point? Maybe, maybe not.  Other options include going in through a vein, or, when she is older, through the brachial or radial vessels.

Future Surgery:  We asked if the cardiologists could tell which surgery option – single ventricle option or two ventricle option – would be the best from what they saw on the CT Scan and the diagnostic cath.  At this point, it is too early to tell, but they are leaning towards the Fontan.  We will probably go home and get a follow-

Heart rate, Pulse Ox and Respiratory monitor
Heart rate, Pulse Ox and Respiratory monitor

up appointment with our Springfield cardiologist and ask them to send information up to St Louis to review in the next 3-6 months.  Dr. M mentioned that if they choose Fontan, then she is probably not ready for the surgery.   She most likely would need to be 3 or 4 years old for that. You can view a video of the Fontan here.  It is technically for a different heart defect, but you’ll get the idea.

If Reiss had the Fontan, would that damage her lungs? The lungs will be fine with the Fontan, according to Dr. M.  Some kids are going to stay blue like they were prior to surgery and some will see an increase in energy and pulse ox levels.  Getting rid of some of the collaterals should eliminate trouble like this down the road.

Answers from the Scope

Dr. Coste didn’t find any mucous in the lungs or infection.  Reiss was, however, found to be positive for Influenza Type A – which we probably had when we got here. We did go into the pediatrician a week ago today complaining of flu-like symptoms and were disappointed when we left with a mild ear infection and chest cold diagnosis. I think it is safe to say that diagnosis was wrong.  However, looking on the bright side of things – if we DID get a positive flu diagnosis and DID get meds for the flu in the first 48 hours of getting sick, Reiss might have not puked blood and we might have continued to get zero answers on her chronic coughing/puking.

Ridged Scope Follow-up:  In 4-6 weeks, we will come back up to St. Louis Children’s Hospital for a follow-up scope.  they will look at the irritated area to see if it is any better or worse.  Hopefully, the collateral Dr. M found and blocked during the cath will help this area.  The ENT will take high definition pictures of the trachea during this time.  If we have bleeding again, they will have to go in with the flexible scope during this visit also.

Vocal Chords:  They did see that the vocal chords were inflamed a bit during this test.  He thought that this could be from the breathing tubes used during the bronch scopes in Springfield last week.  To look into this further, Dr. C recommended getting a swallowing study in Springfield at a later date to see if she is aspirating any fluids or food.  If they do find that she is aspirating, we may need get in touch with a speech pathologist so Reiss can do physical therapy to reduce aspiration.  Food studies to see if certain consistencies of foods irritate her more than others may also have to be done.  If her aspiration is due to one or both of her vocal chords not working properly, we may have to get with an ENT doctor for surgery.

Follow-up appointment:  If they find that the collaterals that were possibly growing into the trachea were the culprit of the bleeding and coughing, then pulmonary really is not needed after this visit.  We are going to go ahead and schedule a follow-up for 4-6 months out to get it on the books since the wait list is 3-4 months long.  Better to have the appointment now and cancel if not needed versus need the appointment and have to wait 3 months.

Engorged vessels:  These vessels, found throughout the left and right lungs, could be a source of her cough because of the increased pressure to them.  It is a possibility that these vessels could rupture if Reiss were to go into a coughing fit.  We asked if we needed to restrict her activity level to help prevent this rupturing.  Dr. C said no.  At her age, her ability to reach an unhealthy level (high level of exercise which sends an increased level of blood through the heart/to the lungs) is highly unlikely.  He would be more worried about her coughing fits.

Blood Clot: The blood clot Dr. C found in the right lung was most likely a clot left over from her initial bleeding.  They cleaned it out of the lung and did not find a blood source where they found it.  He does not expect to get any positive results for infection from the biopsy.

Spots on her Trachea: The cauterized area and the inflamed area on her trachea should heal within 2-3 weeks.  If we start seeing issues at 4 weeks or later – like when we come back for a follow-up bronch scope – that is abnormal.  We may see Reiss spit up small amounts of blood from time to time as these areas heal.  However, if we see 2 tablespoons or more, we need to go to the ER.

So, what was the source of the blood?

Well, they aren’t really sure.

  • It could have been the group of vessels the Springfield ENT found and cauterized
  • It could have been an engorged vessel that ruptured
  • It could be something they didn’t find all together.

Most likely, they believe that the reason for the group of vessels in the trachea was that a collateral from the heart grew into the lining of the trachea, causing Reiss to cough until it ruptured. Since the group of vessels has been cauterized and the collateral they *think* was traveling through the trachea has been blocked, they think that we will possibly see a reduction in coughing and a complete stop to the bleeding.

What could make her cough up blood again?

  • If her body grows more collaterals that go into the lungs
  • If her cauterized area breaks back open
  • If an engorged vessel ruptures.

What is making her Cough?

They think these items may be making her cough:

  • Aspiration of fluid or food (future swallowing study to confirm/deny this) irritating the airway
  • Engorged vessels irritating her airways
  • The collaterals that grew into her trachea
  • Something else

So, while we did find multiple reasons why she might have a cough, as well as reason to cough up blood, we are still not sure if any of the items found are definitive answers to her problems.  We will have to watch her heal from this week’s ordeal and go from there.  We will most likely be discharged some time tomorrow with a list of follow-up appointments and directions.

For now, Reiss is sleeping and on clear fluids.  We will introduce foods as her stomach permits throughout the night and tomorrow morning.

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