Late night weekend case.. evacuating intracranial bleed, fontan physiology!

[coffeebythelake]

It is 2 am. After working for 17 hours straight out of your 24 hour shift, you get a phone call from neurosurgery.

We have a patient coming in from OSH that was just discharged, and she has a large cerebellar subdural. She is "kind of sick", you are warned. You collect as much information as you can from the neurosurgery resident and look up the pt on EMR (some minor identifying details altered)

28 y.o. 128 kg (BMI 52)
- Unbalanced AV septal defect and TGA, pulmonic stenosis s/p fontan palliation, followed by extra cardiac fontan conversion. Baseline BP 130/80s, HR v paced 80, 85% on RA due to shunting
- cardiac cirrhosis with recurrent ascites and esophageal varices. Varices never banded. Ascites tapped usually 2 to 3 liters Qweekly.
- atrial tachycardia, and now more recently CHB with PPM
- IDDM on insulin pump. Bg is 300
- graves disease on methimazole, now hypothyroid on levothyroxine
- morbid obesity
- severe OSA on cpap
- DVT on anticoagulation, received vit k and kcentra at OSH

Pt was recently admitted for acute CHF exacerbation and was aggressively diuresed. You are unclear on current volume status, but presumably it is near euvolemia.

Pt baseline mental status axo3 with no deficits. Over the past 4 hours has progressed from headache to neuro deficits. When you see the patient in the ER, she is minimally responsive, probably not protecting airway. Her eyes open to voice and pain, has no verbal responses, and has flexion withdrawals from pain. Obvious concern for elevated ICPs.

You have no airway history on this patient, and you are unable to get a good exam on the patient. Externally she has good HM distance, thick neck but no obvious deformities, seems to move neck ok. Family member says no loose teeth.

Pt ate dinner 4 hours ago.

Assume anything i have not listed is probably normalish.

You are tasked to anesthetize this patient and keep them alive. BEGIN.

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[WholeLottaGame7]

Do you want a non-peds cardiac person to go first?

It is 2 am. After working for 17 hours straight out of your 24 hour shift, you get a phone call from neurosurgery.

We have a patient coming in from OSH that was just discharged, and she has a large cerebellar subdural. She is "kind of sick", you are warned. You collect as much information as you can from the neurosurgery resident and look up the pt on EMR (some minor identifying details altered)

28 y.o. 128 kg (BMI 52)
- Unbalanced AV septal defect and TGA, pulmonic stenosis s/p fontan palliation, followed by extra cardiac fontan conversion. Baseline BP 130/80s, HR v paced 80, 85% on RA due to shunting
- cardiac cirrhosis with recurrent ascites and esophageal varices. Varices never banded. Ascites tapped usually 2 to 3 liters Qweekly.
- atrial tachycardia, and now more recently CHB with PPM
- IDDM on insulin pump. Bg is 300
- graves disease on methimazole, now hypothyroid on levothyroxine
- morbid obesity
- severe OSA on cpap
- DVT on anticoagulation, received vit k and kcentra at OSH

Pt was recently admitted for acute CHF exacerbation and was aggressively diuresed. You are unclear on current volume status, but presumably it is near euvolemia.

Pt baseline mental status axo3 with no deficits. Over the past 4 hours has progressed from headache to neuro deficits. When you see the patient in the ER, she is minimally responsive, probably not protecting airway. Her eyes open to voice and pain, has no verbal responses, and has flexion withdrawals from pain. Obvious concern for elevated ICPs.

You have no airway history on this patient, and you are unable to get a good exam on the patient. Externally she has good HM distance, thick neck but no obvious deformities, seems to move neck ok. Family member says no loose teeth.

Pt ate dinner 4 hours ago.

Assume anything i have not listed is probably normalish.

You are tasked to anesthetize this patient and keep them alive. BEGIN.

 

[dannyboy1]

Not a peds or cardiac guy so I would call them and then go to sleep.

 

[abolt18]

It is 2 am. After working for 17 hours straight out of your 24 hour shift, you get a phone call from neurosurgery.

We have a patient coming in from OSH that was just discharged, and she has a large cerebellar subdural. She is "kind of sick", you are warned. You collect as much information as you can from the neurosurgery resident and look up the pt on EMR (some minor identifying details altered)

28 y.o. 128 kg (BMI 52)
- Unbalanced AV septal defect and TGA, pulmonic stenosis s/p fontan palliation, followed by extra cardiac fontan conversion. Baseline BP 130/80s, HR v paced 80, 85% on RA due to shunting
- cardiac cirrhosis with recurrent ascites and esophageal varices. Varices never banded. Ascites tapped usually 2 to 3 liters Qweekly.
- atrial tachycardia, and now more recently CHB with PPM
- IDDM on insulin pump. Bg is 300
- graves disease on methimazole, now hypothyroid on levothyroxine
- morbid obesity
- severe OSA on cpap
- DVT on anticoagulation, received vit k and kcentra at OSH

Pt was recently admitted for acute CHF exacerbation and was aggressively diuresed. You are unclear on current volume status, but presumably it is near euvolemia.

Pt baseline mental status axo3 with no deficits. Over the past 4 hours has progressed from headache to neuro deficits. When you see the patient in the ER, she is minimally responsive, probably not protecting airway. Her eyes open to voice and pain, has no verbal responses, and has flexion withdrawals from pain. Obvious concern for elevated ICPs.

You have no airway history on this patient, and you are unable to get a good exam on the patient. Externally she has good HM distance, thick neck but no obvious deformities, seems to move neck ok. Family member says no loose teeth.

Pt ate dinner 4 hours ago.

Assume anything i have not listed is probably normalish.

You are tasked to anesthetize this patient and keep them alive. BEGIN.

As I'm reading the description of this patient, every line has me saying "holy crap how does this person get worse?" And then I read the next line and it gets worse.

 

[shepardsun]

Interesting case. She needs a secure airway, and given mental and NPO status that airway needs to be an RSI. Unfortunately being a single ventricle, PPV will be deleterious to her pulmonary blood flow and cardiac output. Time and extra hands providing I would place a pre-induction A-line and induce with 0.1 mg/kg of etomidate and RSI dose rocurionium. CVC. Keppra, mannitol. Couple units crossed. Coags and TEG to get a handle on coagulation status before other products. Maintain sats at baseline and avoid hypercarbia. Milrinone, NO, Epi close at hand for the inevitable qp:qs death spiral. To me, diabetes and thyroid issues can be triaged until patient is more stable.

 

[vector2]

I’m not worried at all. Any congenital kid who’s able to reach NFL O-tackle BMI clearly has adequate cardiac output and oxygen delivery. Prop, sux, tube.

 

[PainDrain]

Palliative care consult stat.

 

[T-burglar]

Good thing this dude has a pacemaker since you’ll be messing around near the brain stem

 

[coffeebythelake]

This was my case a few weeks back.

Wanted to post this for education purposes because it was very complicated, with conflicting physiologic goals, like something from oral board's

No, I am not cardiac or peds trained.
I have no subspecialty training.
Maybe for one of you guys it is pretty standard type case, but for me it was quite an experience.

Will tell you guys our plan and intraop management later. Thankfully the Pt did well.

 

[deleted171991]

I sincerely hope you're a handsomely paid-partner if you have to deal with crap like this solo, in the middle of the night.

What this patient needs first and foremost is a burr hole stat. Then go from there.

I would be careful about fixing her sugar. It may increase the cerebral edema. It is not the reason for the mental status.

The thyroid is not an issue anymore.

Bleeding may still be a problem, even after K-centra. Place 2 large bore IVs and have blood in the room before they start poking holes in her head.

That airway probably needs to be protected, before anything else is done. A-line, RSI with glidescope, maintain BP during induction (propofol/etomidate, pick your poison, high dose roc). It's a woman, so the airway will be much better than the same BMI male, sleep apnea or not.

Otherwise just a usual crani, or easier.

 

[deleted171991]

As I'm reading the description of this patient, every line has me saying "holy crap how does this person get worse?" And then I read the next line and it gets worse.

Welcome to the real world, Neo!

 

[deleted171991]

Palliative care consult stat.

28 years old.

 

[PainDrain]

28 years old.

First off, she has terminal congenital heart disease for which the only “cure” is transplant; there is no further palliation at this point. She also has ESLD as a result of her cardiac disease. This combination makes her risk of M&M following a heart transplant very high and that is excluding all her other issues. I also wonder how well her kidneys are holding up to the combination of ESLD, CHF and chronic diuretic use. If she has any kidney damage then she has an astronomical risk of death going to transplant. She hasn’t helped her case by developing DM, obesity and OSA. Now she has a brain bleed. It’s time for a GOC discussion.

 

[WholeLottaGame7]

First things first. This case will be challenging from an anesthetic standpoint (neuro bleed is up there with spine surgery as far as cases I don't want to do), but it's the post-op management that is going to really make or break this patient. Since you're doing it and not a peds or cardiac person, I'm guessing you're not at a place that handles this kind of patient that frequently. I would have shipped this patient to the nearest tertiary hospital when it became evident she was heading towards surgery. Assuming it's an emergency and couldn't wait, do what you have to do to get them through surgery and get them transferred.

By organ system, since this is going to get complicated.

Airway/resp: As others have mentioned, PPV is deleterious for Fontan physiology, but the combination of NPO status, body habitus, and surgical procedure is going to force your hand towards intubation. I'd use physiologic PEEP (4 or 5), but not too much higher. You're not going to get away with low airway pressures given the weight, but as low as reasonably achievable to maintain normocapnia. Would run slightly higher rate with lower tidal volumes. FiO2 to keep the sats in the 80s but would err on the higher side to keep PA pressures low.

Cardiac: Agree with a-line but don't feel super strongly about doing it awake vs asleep. Would be nice to know the last echo: you mentioned shunting, but is it through a fenestration in the Fontan or MAPCAs? Which direction was the AVCD unbalanced (is it more LV or RV)? AV-valve regurgitation? Function? Volume status? Would be hesitant to place a TEE given the esophageal varices, and while a TTE might be possible under the drapes, the windows/image quality is not going to be great. Plus it's not going to look like anything that familiar to you.

Not unreasonable to place a CVL; it would give you PA pressures (assuming no obstruction at the cavopulmonary junction or other funky anatomy) and a route to give infusions such as milrinone or epi as mentioned. Likelihood of needing support probably dependent on underlying ventricular morphology and function. Downside obviously if you get a clot in the Fontan circulation that's not bueno.

Volume status is going to be a stab in the dark. Obviously you want to keep them euvolemic, but if you're going to miss, probably a little safer to miss on the hypervolemic side. Much easier to diurese fluid off later than it is to have the Fontan circuit go down from hypovolemia/low cardiac output/thrombosis. Would not tolerate a lot of hypotension in this patient, if suspect euvolemic and hypotensive, will need to add something. A little phenylephrine to counteract the anesthetic is probably OK, but wouldn't want to jack up the afterload too high on a single ventricle.

I like milrinone/epi, to help with inotropy, lusitropy (usually have pretty thick ventricles with high diastolic pressures), keep the PA pressures low, and keep the SVR reasonable.

Obviously lots of arrhythmia concerns, some of which are resolved with the PM. Would not be unreasonable to just put pads on this patient in case things go south.

Vascular/heme: This is where it's going to get messy. This patient is going to bleed like a m*****f*****. The failing Fontan is going to have pretty high venous pressures at baseline and a ton of collaterals. Throw in the hepatic dysfunction from the cardiac cirrhosis and the anticoagulation from treatment for the DVT and you have a disaster on your hands. As I mentioned above, neuro bleeds and major spines are no fun in failing Fontans. Agree with TEG to help guide things, but overall this patient is going to need a lot of product. If you can't get 2 big PIVs, may need a CVL for that reason.

Endocrine: Not even going to worry that much about the hypothyroidism/graves disease/diabetes. Keep the glucoses reasonable.

GI: Apart from being reluctant to put a TEE probe down, nothing special. May need to put an OG down to suck out the stomach given the NPO status, but be careful.

Renal: Obv keep an eye on the electrolytes, especially the calcium/magnesium. Don't want to exacerbate any arrhythmias.

Don't even worry about extubating this patient. She's altered at baseline, they're going to be in her skull, and she's morbidly obese. If you extubate her and she obstructs or becomes hypercarbic/hypoxic, she's going to jack her PA pressures up and everything shuts down. Plus they're going to want to optimize her volume status again and she's going to be pretty high risk for TRALI given the blood products she needs.

I think that's mostly it...for now?

It is 2 am. After working for 17 hours straight out of your 24 hour shift, you get a phone call from neurosurgery.

We have a patient coming in from OSH that was just discharged, and she has a large cerebellar subdural. She is "kind of sick", you are warned. You collect as much information as you can from the neurosurgery resident and look up the pt on EMR (some minor identifying details altered)

28 y.o. 128 kg (BMI 52)
- Unbalanced AV septal defect and TGA, pulmonic stenosis s/p fontan palliation, followed by extra cardiac fontan conversion. Baseline BP 130/80s, HR v paced 80, 85% on RA due to shunting
- cardiac cirrhosis with recurrent ascites and esophageal varices. Varices never banded. Ascites tapped usually 2 to 3 liters Qweekly.
- atrial tachycardia, and now more recently CHB with PPM
- IDDM on insulin pump. Bg is 300
- graves disease on methimazole, now hypothyroid on levothyroxine
- morbid obesity
- severe OSA on cpap
- DVT on anticoagulation, received vit k and kcentra at OSH

Pt was recently admitted for acute CHF exacerbation and was aggressively diuresed. You are unclear on current volume status, but presumably it is near euvolemia.

Pt baseline mental status axo3 with no deficits. Over the past 4 hours has progressed from headache to neuro deficits. When you see the patient in the ER, she is minimally responsive, probably not protecting airway. Her eyes open to voice and pain, has no verbal responses, and has flexion withdrawals from pain. Obvious concern for elevated ICPs.

You have no airway history on this patient, and you are unable to get a good exam on the patient. Externally she has good HM distance, thick neck but no obvious deformities, seems to move neck ok. Family member says no loose teeth.

Pt ate dinner 4 hours ago.

Assume anything i have not listed is probably normalish.

You are tasked to anesthetize this patient and keep them alive. BEGIN.

 

[deleted59964]

Firstly - yikes.

Issues

A - difficult exam, no history, unfasted
B - morbid obesity and osa
C - Fontan, ppm, recent admit for CHF
D - minimally responsive, to unable to protect own airway secondary to large cerebellar subdural and raised icp
E - IDDM with current hyperglycaemia (300mg/dL = 16.6 mol/L), presumably well treated hypothyroidism
F - probably euvolaemic (till the blood letting starts)
G - has varices - No TEE?
H - anticoagulated for DVT ... with what? ?warfarin ?
I - really don’t want to do this case

... ok

So ... I would start with a discussion with the neurosurgeon and ensure they appreciated the complexity, and they were not of the “there is a sub dural, I have to fix it” mentality. This patient may die tonight.

Once on the same page I would have a family meeting with the surgeon and intensivist present ... the focus being to establish the patient’s wishes if they had been articulated and the family’s values and expectations. If proceeding I would make it clear that she may not survive, and the team is focussed on trying to get her through this acute event to buy some more time.

Any chance the patient / family prefers dignity over heroics?
.

 

[MoMoGesiologist]

Any chance the patient / family prefers dignity over heroics?

Patient is 28yo. If we are doing heroics on 70-90 year olds, then what are the chances someone in their 20s wouldn’t get heroics?

 

[deleted59964]

Not a peds or cardiac guy so I would call them and then go to sleep.

If I’m the peds guy on, and you call me to do a 28 yo ...

 

[deleted59964]

Patient is 28yo. If we are doing heroics on 70-90 year olds, then what are the chances someone in their 20s wouldn’t get heroics?

28 yo with a fontan ...you get that right?

 

[deleted171991]

28 yo with a fontan ...you get that right?

Somebody's daughter or sister, who was still functional 4 hours before, and may be again, for a few more years.

Come on, @eikenhein, tell us how you fought the windmill dragon.

 

[deleted59964]

What do you like? 20?

50?

80???

For peds cover < 15 sounds fair ... generally < 2yo really

 

[deleted59964]

... for a few more years.

Come on, @eikenhein, tell us how you fought the windmill dragon.

yeah - who knows ...maybe a few years (if they survive the current issue), patient and family where I come from may or may not want to go out in a cloud of morphine instead - this stuff should be discussed in documented in advance of course.

 

[deleted59964]

If going ahead ...

I'd want a pre induction art line, and I anticipate this patient may have difficult IV access, so I'd just double stick the femoral artery and vein (with something that also gives a big lumen). A central line would be difficult to get once positioned and prepped anyway , and the patient is highly likely to need vasoactives.

For induction - we don't have etomidate - so it'd be fentanyl heavy, propofol light, with pressors and inotropes ready. a "RSI" but really CVS stability trumps preventing aspiration for me here.

A) I'd preoxygenate with HHFNP, have a video laryngoscope, position carefully, and have another pair of hands in case it goes badly ... I don't really expect it to, but she won't tolerate CICO for long.

B) morbid obesity and osa -- positioning for surgery is going to be tricky -- plan this with the surgeon first ... with her obesity and ascites ... does this need tapping urgently? -- not keen on it interfering with ventilation and causing increased PVR

C) The real concern here ... Fontan, ppm, recent admit for CHF -- trying to keep the patient euvolaemic will be a like walking a greased tightrope, also arrhythmia concerns, would have pads on pre induction.

D) - minimally responsive, unable to protect own airway secondary to large cerebellar subdural and raised icp ... yep, needs a tube asap -- not going to be able to maintain spontaneous ventilation here

E) - IDDM with current hyperglycaemia (300mg/dL = 16.6 mol/L), presumably well treated hypothyroidism ... I'd treat the glucose but gently

F) - probably euvolaemic (till the blood letting starts) ... as above

G) - has varices - No TEE? and TTE won't be worth much even in the hands of a cardiologist specialising in CHD because of the patient habitus and PPV

H) - anticoagulated for DVT ... with what? ?warfarin ? -- needs reversing, and blood products guided by ROTEM would be useful, as would haematology input -- these patients die from thromboembolisms.



Well ... how did you do it???

 

[WholeLottaGame7]

The adult/cardiac people always talk s*** about how easy peds cardiac call is, and how few emergencies there are, and how they want to get in on that action, until this case rolls through the door and it's crickets...

Job security, I guess.

Also, we need the gripping conclusion to this cliffhanger...

If I’m the peds guy on, and you call me to do a 28 yo ...

 

[coffeebythelake]

A lot of what people have posted here was
The fatigue going into the case and the limited prep time definitely made it harder for me.

Little more background
I work in a large, academic, tertiary care hospital with anesthesiology residents.
I am a general anesthesiologist, I do not have subspecialty training
The patient was ambulanced over from a smaller affiliated hospital in the suburbs. She was discharged less than 24 hours prior after hospitalized for ADHF with aggressive diuresis. She was deemed not a candidate for transplant at the time due to BMI.
We had about 45 min to 1 hour notice of this emergency case and activated our neurotrauma OR upon hearing of the case.
The patient was on lovenox being bridged to warfarin, got vit k and kcentra at the OSH

Course of events
After pt arrived around 300 am, I performed a quick physical exam and conducted a brief interview with the patient's relative who accompanied her. Not much new information. Labs were spun, T+C obtained, the other results were largely unremarkable. Superstat coag studies were ok. She had 2 large bore peripheral IVs. Pt was not following commands, couldn't get a good airway examination, and had no airway history notes for review. She was likely not protecting her airway, but was not grossly aspirating, was breathing spontaneously and hemodynamically appeared ok.

The emergency medicine attending wanted to intubate her right away. It was apparent they didn't really understand the physiology aside from trying to keep the patient stable for it. I told them to it would be better for us to do the intubation in the OR. We tested to see if the patient can lay flat for 30 seconds to perform a head CT and then brought her up ASAP to the OR. Had a realistic discussion with the neurosurgeon and family about the high risks.

Arrived in OR around 320 am, kept pt HOB elevated, placed pre-induction arterial line. Gave lidocaine IV (100 mg). Cardiac stable induction, induced with etomidate (20 mg), high dose rocuronium (120 mg), touch of propofol (20-30 mg) while watching hemodynamics closely and having a variety of pressors and inotropes within reach. Intubated with glidescope, easily, one attempt. Induction was very stable. Vented on pressure control mode, being careful with airway pressures and using minimal PEEP. Hyperventilated patient to EtCO2 30, and sent off ABG. Gave 10 mg decadron IV. Didn't give antiepileptic per neurosurgery (I think not an epileptogenic area of the brain). Kept anesthetized with combo sevo/prop/fent, light neo gtt, kept muscle relaxed throughout. BIS was 20's. Neurosurgery placed a frontal EVD to help decompress. Mayfield pins applied. We then applied a magnet to the PPM and flipped the patient prone for surgical exposure.

Concerned about prone positioning and venous return, but the flip went smoothly and remained hemodynamically ok with nominal airway pressures. 1g/kg of mannitol administered, kept a close eye that it may significantly drop volume status and worsen preload. We also administered several doses of insulin at this point and had her insulin pump running on basal rate throughout, with gradual improvement in BG from 300 -> 200's. We weren't in a hurry to rapidly correct BG.

Neurosurgery was able to evacuate the subdural bleed, achieve hemostasis, and close. Minimal blood loss. A small part of the cerebellum was herniated into the surgical incision and was resected as well. Surgical closing, normalized EtCO2, flipped back supine, kept intubated to CT scan and then handed over to our anesthesia CCM team in neurosurgical ICU by 730 am. No pressors or inotropes. Propofol running at 50 mcg/kg/min. (I was kind of pissed off at the respiratory therapist who put the patient on PEEP of 10 for no reason, completely mismanaged the ventilator airway pressures were high 30's and TV 350's, and who obviously had no idea about the implications of the pt's physiology. I fixed the vent settings before I left, only to find out later she put the patient back on PEEP 10!!) Pt was extubated later that day, fully neuro intact. Postop labs looked ok. Pt took a hit to the kidneys which recovered within 48 hours.

Thought process and concerns
1. Fontan physiology and cardiac status
- recognizing the need to maintain adequate preload, avoid conditions that increased PA pressures
- presence of PPM was probably a good thing since neurosurgeon was working close to the brainstem which could cause significant HR, hemodynamic fluctuation.
- unclear volume status, unclear if patient's starting BP reflected any part of Cushing's response, unclear if muscle relaxation and PPV could cause dramatic drop in hemodynamics... especially with such obese + ascites abdomen compressing on IVC. Induction was clearly the most dangerous part of the case.
- minimize PEEP, minimize airway pressures, attempt to minimize R -> L shunting
- meticulous de-airing of lines
- c/f bleeding from varices, no intention for TEE

2. ICP and conflicting physiologic goals from point 1.
- need to prevent hypertension which could worsen bleeding, need to prevent hypotension which could decrease CPP
- ideally want to run pt dry but this could affect preload.
- considered the potential for (and actualized) renal insufficiency with diuresis.
- HOB up whenever we could
- induction with lidocaine (although hasn't been shown to significantly decrease ICP), hyperventilate, decadron, mannitol, monitor and correct Na

3. Airway
- no airway history, poor exam
- awake intubation not an option due to lack of patient cooperation, potentially worsening ICP due to coughing or bucking
- ramped up into optimal position, glidescope to maximize first pass success. if fail might have needed surgical airway.
- given severe OSA, morbid obesity, and pre-existing neuro status we planned to keep patient intubated postop

4. Endocrine
- somewhat lower priority, treat BG for gradual reduction.
- obviously want to prevent hypoglycemia.


I think if this patient also had ESRD, she would have had a much more difficult anesthetic and much much higher morbidity/mortality.

 

[deleted59964]

Kudos - that’s a sphincter puckering case.

 

[pgg]

Very nice

 

[deleted162650]

Honestly, after reading the stem I wasn't too worried. I mean - you've got someone with a Fontan, ESLD, super morbid obesity, OSA, poorly controlled diabetes, DVT's, and an acute posterior fossa bleed on anticoagulation and they're STILL not dead = there's pretty much nothing I can do that's gonna kill this patient. I'm just not that conceited.

 

[urge]

Pent sux tube

 

[deleted171991]

Honestly, after reading the stem I wasn't too worried. I mean - you've got someone with a Fontan, ESLD, super morbid obesity, OSA, poorly controlled diabetes, DVT's, and an acute posterior fossa bleed on anticoagulation and they're STILL not dead = there's pretty much nothing I can do that's gonna kill this patient. I'm just not that conceited.

Must have been a veteran.

 

[MoMoGesiologist]

Must have been a veteran.

Nothing can kill those dudes... god bless ‘em

 

[WholeLottaGame7]

Nice work, for a generalist... But seriously, nice work.

The post-op course is why they should be cared for in a CICU or PCICU w/ neurosurgery consulting. The physiology is totally different, the ICU got lucky.

I am always pro-meticulous de-airing, but just for clarification, everything given IV to a Fontan should go straight to the lungs first, so not as big an issue. You mention shunting, but didn't clarify where. It's usually not R to L intracardiac shunting unless it's been fenestrated and the physiology is really s****y. Most of the time when we talk about "shunting" in Fontan physiology, it's arterial (aorta, subclavian, IMA) --> pulmonary collaterals.

A lot of what people have posted here was
The fatigue going into the case and the limited prep time definitely made it harder for me.

Little more background
I work in a large, academic, tertiary care hospital with anesthesiology residents.
I am a general anesthesiologist, I do not have subspecialty training
The patient was ambulanced over from a smaller affiliated hospital in the suburbs. She was discharged less than 24 hours prior after hospitalized for ADHF with aggressive diuresis. She was deemed not a candidate for transplant at the time due to BMI.
We had about 45 min to 1 hour notice of this emergency case and activated our neurotrauma OR upon hearing of the case.
The patient was on lovenox being bridged to warfarin, got vit k and kcentra at the OSH

Course of events
After pt arrived around 300 am, I performed a quick physical exam and conducted a brief interview with the patient's relative who accompanied her. Not much new information. Labs were spun, T+C obtained, the other results were largely unremarkable. Superstat coag studies were ok. She had 2 large bore peripheral IVs. Pt was not following commands, couldn't get a good airway examination, and had no airway history notes for review. She was likely not protecting her airway, but was not grossly aspirating, was breathing spontaneously and hemodynamically appeared ok.

The emergency medicine attending wanted to intubate her right away. It was apparent they didn't really understand the physiology aside from trying to keep the patient stable for it. I told them to it would be better for us to do the intubation in the OR. We tested to see if the patient can lay flat for 30 seconds to perform a head CT and then brought her up ASAP to the OR. Had a realistic discussion with the neurosurgeon and family about the high risks.

Arrived in OR around 320 am, kept pt HOB elevated, placed pre-induction arterial line. Gave lidocaine IV (100 mg). Cardiac stable induction, induced with etomidate (20 mg), high dose rocuronium (120 mg), touch of propofol (20-30 mg) while watching hemodynamics closely and having a variety of pressors and inotropes within reach. Intubated with glidescope, easily, one attempt. Induction was very stable. Vented on pressure control mode, being careful with airway pressures and using minimal PEEP. Hyperventilated patient to EtCO2 30, and sent off ABG. Gave 10 mg decadron IV. Didn't give antiepileptic per neurosurgery (I think not an epileptogenic area of the brain). Kept anesthetized with combo sevo/prop/fent, light neo gtt, kept muscle relaxed throughout. BIS was 20's. Neurosurgery placed a frontal EVD to help decompress. Mayfield pins applied. We then applied a magnet to the PPM and flipped the patient prone for surgical exposure.

Concerned about prone positioning and venous return, but the flip went smoothly and remained hemodynamically ok with nominal airway pressures. 1g/kg of mannitol administered, kept a close eye that it may significantly drop volume status and worsen preload. We also administered several doses of insulin at this point and had her insulin pump running on basal rate throughout, with gradual improvement in BG from 300 -> 200's. We weren't in a hurry to rapidly correct BG.

Neurosurgery was able to evacuate the subdural bleed, achieve hemostasis, and close. Minimal blood loss. A small part of the cerebellum was herniated into the surgical incision and was resected as well. Surgical closing, normalized EtCO2, flipped back supine, kept intubated to CT scan and then handed over to our anesthesia CCM team in neurosurgical ICU by 730 am. No pressors or inotropes. Propofol running at 50 mcg/kg/min. (I was kind of pissed off at the respiratory therapist who put the patient on PEEP of 10 for no reason, completely mismanaged the ventilator airway pressures were high 30's and TV 350's, and who obviously had no idea about the implications of the pt's physiology. I fixed the vent settings before I left, only to find out later she put the patient back on PEEP 10!!) Pt was extubated later that day, fully neuro intact. Postop labs looked ok. Pt took a hit to the kidneys which recovered within 48 hours.

Thought process and concerns
1. Fontan physiology and cardiac status
- recognizing the need to maintain adequate preload, avoid conditions that increased PA pressures
- presence of PPM was probably a good thing since neurosurgeon was working close to the brainstem which could cause significant HR, hemodynamic fluctuation.
- unclear volume status, unclear if patient's starting BP reflected any part of Cushing's response, unclear if muscle relaxation and PPV could cause dramatic drop in hemodynamics... especially with such obese + ascites abdomen compressing on IVC. Induction was clearly the most dangerous part of the case.
- minimize PEEP, minimize airway pressures, attempt to minimize R -> L shunting
- meticulous de-airing of lines
- c/f bleeding from varices, no intention for TEE

2. ICP and conflicting physiologic goals from point 1.
- need to prevent hypertension which could worsen bleeding, need to prevent hypotension which could decrease CPP
- ideally want to run pt dry but this could affect preload.
- considered the potential for (and actualized) renal insufficiency with diuresis.
- HOB up whenever we could
- induction with lidocaine (although hasn't been shown to significantly decrease ICP), hyperventilate, decadron, mannitol, monitor and correct Na

3. Airway
- no airway history, poor exam
- awake intubation not an option due to lack of patient cooperation, potentially worsening ICP due to coughing or bucking
- ramped up into optimal position, glidescope to maximize first pass success. if fail might have needed surgical airway.
- given severe OSA, morbid obesity, and pre-existing neuro status we planned to keep patient intubated postop

4. Endocrine
- somewhat lower priority, treat BG for gradual reduction.
- obviously want to prevent hypoglycemia.


I think if this patient also had ESRD, she would have had a much more difficult anesthetic and much much higher morbidity/mortality.

 

[deleted697535]

Great case well done

Couple qs.
Why not afoi with some precedex/ketamine? Keep spont vent as long as possible...
Why not ask surgeons to just do evd under local?
Did you have a boatload of suggamadex ready?

I agree with what others have said. Very tricky case but the fact that this person isn't cachetic actually says they might have some reserve. Which it turns out they have as they tolerated PPV and loads of peep very well

 

[coffeebythelake]

Great case well done

Couple qs.
Why not afoi with some precedex/ketamine? Keep spont vent as long as possible...
Why not ask surgeons to just do evd under local?
Did you have a boatload of suggamadex ready?

I agree with what others have said. Very tricky case but the fact that this person isn't cachetic actually says they might have some reserve. Which it turns out they have as they tolerated PPV and loads of peep very well

Not going to attempt AFOI in situation that is time sensitive, in an uncooperative patient, where bucking or coughing could cause herniation and death. A ketamine induction could he a possibility but would it have made a big difference?

EVD under local probably doable, and indeed we do this sometimes in the ICU. In this particular case, neurosurgeon wanted to do it after pt was anesthetized. The surgeon was actually a spine guy, doesnt do much crani work in his usual practice. Unclear if their concerns were relating to upward shift of brain from the infratentorial bleed.

No sugammadex needed. When we decided to induce we have committed ourself down that pathway. No amount of sugammadex would allow us to reverse course. Also we planned to keep pt tubed postop.

 

[deleted59964]

Not going to attempt AFOI in situation that is time sensitive, in an uncooperative patient, where bucking or coughing could cause herniation and death. A ketamine induction could he a possibility but would it have made a big difference?

EVD under local probably doable, and indeed we do this sometimes in the ICU. In this particular case, neurosurgeon wanted to do it after pt was anesthetized. The surgeon was actually a spine guy, doesnt do much crani work in his usual practice. Unclear if their concerns were relating to upward shift of brain from the infratentorial bleed.

No sugammadex needed. When we decided to induce we have committed ourself down that pathway. No amount of sugammadex would allow us to reverse course. Also we planned to keep pt tubed postop.

patient is definitely on a way way trip to a snorkel... plan how you’re going to get it in 5 different ways - coz one way or another it’s going in because the patient needs a tube anyway

 

[coffeebythelake]

I am always pro-meticulous de-airing, but just for clarification, everything given IV to a Fontan should go straight to the lungs first, so not as big an issue. You mention shunting, but didn't clarify where. It's usually not R to L intracardiac shunting unless it's been fenestrated and the physiology is really s****y. Most of the time when we talk about "shunting" in Fontan physiology, it's arterial (aorta, subclavian, IMA) --> pulmonary collaterals.

Sorry, I'm not sure what you mean. Can you clarify this?
Pt has known R -> L shunt with baseline sats in mid 80's on RA.
I'm assuming there is some sort of fenestration, although notes were unclear and i wasn't going to waste too much time digging for something when time was sensitive and a solution would be relatively straightforward

 

[IlDestriero]

It is 2 am. After working for 17 hours straight out of your 24 hour shift, you get a phone call from neurosurgery.

We have a patient coming in from OSH that was just discharged, and she has a large cerebellar subdural. She is "kind of sick", you are warned. You collect as much information as you can from the neurosurgery resident and look up the pt on EMR (some minor identifying details altered)

28 y.o. 128 kg (BMI 52)
- Unbalanced AV septal defect and TGA, pulmonic stenosis s/p fontan palliation, followed by extra cardiac fontan conversion. Baseline BP 130/80s, HR v paced 80, 85% on RA due to shunting
- cardiac cirrhosis with recurrent ascites and esophageal varices. Varices never banded. Ascites tapped usually 2 to 3 liters Qweekly.
- atrial tachycardia, and now more recently CHB with PPM
- IDDM on insulin pump. Bg is 300
- graves disease on methimazole, now hypothyroid on levothyroxine
- morbid obesity
- severe OSA on cpap
- DVT on anticoagulation, received vit k and kcentra at OSH

Pt was recently admitted for acute CHF exacerbation and was aggressively diuresed. You are unclear on current volume status, but presumably it is near euvolemia.

Pt baseline mental status axo3 with no deficits. Over the past 4 hours has progressed from headache to neuro deficits. When you see the patient in the ER, she is minimally responsive, probably not protecting airway. Her eyes open to voice and pain, has no verbal responses, and has flexion withdrawals from pain. Obvious concern for elevated ICPs.

You have no airway history on this patient, and you are unable to get a good exam on the patient. Externally she has good HM distance, thick neck but no obvious deformities, seems to move neck ok. Family member says no loose teeth.

Pt ate dinner 4 hours ago.

Assume anything i have not listed is probably normalish.

You are tasked to anesthetize this patient and keep them alive. BEGIN.

Call cardiac anesthesia, send the fellow to do the preop, and go back to sleep.

 

[Hork Bajir]

Nice case, thanks for sharing!

I think I’ve mentioned this in other threads before, but you could always transduce peripheral venous pressure (PVP) and use it as a proxy for CVP- or in this case, PAP. I’ve personally done this in a Fontan patient, and it correlates with true CVP within a few points... pretty cool stuff. Not to mention that with the failing ventricle and high venous pressures, these patients often have pipes for veins, making IV access the least of your concerns.

Also not sure how useful TEE would be during this case (prone patient, confusing anatomy)... But if you ever feel strongly that TEE would help you, remember that varices are NOT an absolute contraindication. At my center we routinely do TEE for OLT in the setting of varices.

Transesophageal echocardiography during orthotopic liver transplantation in patients with esophagoastric varices - PubMed

TEE is a relatively safe method for monitoring cardiac performance with a low incidence of major hemorrhagic complications in patients with documented esophagogastric varices undergoing OLT.

www.ncbi.nlm.nih.gov

Well done OP, strong work!

 

[deleted697535]

I dont really understand. Transduce peripheral venous pressure as a surrogate of CVP for what purpose? Does anyone measure CVP anymore?

 

[vector2]

I dont really understand. Transduce peripheral venous pressure as a surrogate of CVP for what purpose? Does anyone measure CVP anymore?

Just because CVP has fallen out of vogue in the anesthesia and ICU literature in as far as assessing volume status, don't make the mistake of throwing the baby out with the bathwater. The utility of monitors needs to be considered with respect to an individual patient's condition, and single ventricle physiology s/p fontan or glenn, severe TR, severe pulm HTN, RV failure are all legitimate conditions where CVP (and PAP) can actually give you information about volume status and ventricular function since veins are typically maximally distended and pressure/volume coupling is likely occurring.

In other words, a CVP measurement that fluctuates between 5-10 in an otherwise healthy patient getting a colectomy doesn't really mean fckall to me. A CVP that acutely goes from 25 to 75 in the OP's patient means someone should start drawing up epi, milrinone, iNO and get the crash cart in the room.

 

[Merely]

Not a peds or cardiac guy so I would call them and then go to sleep.

I’m only a med student so this is naive but if the pt is 28 why would a peds guy be involved

 

[Ronin786]

Nice case, thanks for sharing!

I think I’ve mentioned this in other threads before, but you could always transduce peripheral venous pressure (PVP) and use it as a proxy for CVP- or in this case, PAP. I’ve personally done this in a Fontan patient, and it correlates with true CVP within a few points... pretty cool stuff. Not to mention that with the failing ventricle and high venous pressures, these patients often have pipes for veins, making IV access the least of your concerns.

Also not sure how useful TEE would be during this case (prone patient, confusing anatomy)... But if you ever feel strongly that TEE would help you, remember that varices are NOT an absolute contraindication. At my center we routinely do TEE for OLT in the setting of varices.

Transesophageal echocardiography during orthotopic liver transplantation in patients with esophagoastric varices - PubMed

TEE is a relatively safe method for monitoring cardiac performance with a low incidence of major hemorrhagic complications in patients with documented esophagogastric varices undergoing OLT.

www.ncbi.nlm.nih.gov

Well done OP, strong work!

You've mentioned this before and I still don't understand its utility. Maybe as a marker of extreme badness like Vector2 mentioned (though I'd argue one would see that in many other ways in this particular patient). What does a peripheral CVP of 3 mean? Or 5? Or 10? or 12?

Are you really using that guide fluid therapy? Or inotrope titration? That's an extremely ridiculous proposition if so, unless you have some sort of evidence to back it up.

 

[vector2]

You've mentioned this before and I still don't understand its utility. Maybe as a marker of extreme badness like Vector2 mentioned (though I'd argue one would see that in many other ways in this particular patient). What does a peripheral CVP of 3 mean? Or 5? Or 10? or 12?

Are you really using that guide fluid therapy? Or inotrope titration? That's an extremely ridiculous proposition if so, unless you have some sort of evidence to back it up.

The coupling of CVP and PVR with fontans is pretty well known in the peds cards literature. The so-called fontan syndrome (hepatic congestion, cardiorenal, protein losing enteropathy) is also pretty well correlated with CVP. I'd argue that the causes of "extreme badness" with this patient in this particular case could be myriad. If this patient develops intraop hypotension, your differential still includes extreme vasodilation, neurogenic shock, adrenal crisis, hypovolemia, hemorrhage, PE, air embolus, MI, IVC compression from a prone obese abdomen, electrolyte abnls, and on and on and on. However, if the pt develops hypotension as the CVP doubles, that can really be only be about 2 things (acute worsening of pulmonary hypertension or ventricular failure). And yes, in this patient I would certainly use CVP to guide fluid therapy, because as I stated earlier, pt's with this kind of vein distention actually have pressures that couple with volume due to capacitance being maxed.

And we still haven't gotten to the part where CVP can be used to aid in the differential of hypoxemia in this patient as it appears from the stem that they have way too much deoxygenated flow through the fenestration....

 

[Ronin786]

The coupling of CVP and PVR with fontans is pretty well known in the peds cards literature. The so-called fontan syndrome (hepatic congestion, cardiorenal, protein losing enteropathy) is also pretty well correlated with CVP. I'd argue that the causes of "extreme badness" with this patient in this particular case could be myriad. If this patient develops intraop hypotension, your differential still includes extreme vasodilation, neurogenic shock, adrenal crisis, hypovolemia, hemorrhage, PE, air embolus, MI, IVC compression from a prone obese abdomen, electrolyte abnls, and on and on and on. However, if the pt develops hypotension as the CVP doubles, that can really be only be about 2 things (acute worsening of pulmonary hypertension or ventricular failure). And yes, in this patient I would certainly use CVP to guide fluid therapy, because as I stated earlier, pt's with this kind of vein distention actually have pressures that couple with volume due to capacitance being maxed.

And we still haven't gotten to the part where CVP can be used to aid in the differential of hypoxemia in this patient as it appears from the stem that they have way too much deoxygenated flow through the fenestration....

PE, air embolism, MI and IVC compression are all going to result in elevated CVPs. That said, I already acknowledged the utility of CVP in guiding management and alerting about badness, but you're actually going to trust a pressure tracing off a periperal IV? Some of the pitfalls of CVP are how it can widely fluctuate based off PPV, juxtaposition of intrathoracic pressures and the location of your line. How is the peripheral IV CVP any less prone to error? You could be up against a valve, malpositioned, kinked, or have compression anywhere retrograde from the RA. It's a setup for error!

 

[vector2]

PE, air embolism, MI and IVC compression are all going to result in elevated CVPs. That said, I already acknowledged the utility of CVP in guiding management and alerting about badness, but you're actually going to trust a pressure tracing off a periperal IV? Some of the pitfalls of CVP are how it can widely fluctuate based off PPV, juxtaposition of intrathoracic pressures and the location of your line. How is the peripheral IV CVP any less prone to error? You could be up against a valve, malpositioned, kinked, or have compression anywhere retrograde from the RA. It's a setup for error!

Fair enough, it's more than 2 things. So lump in MI with ventricular failure. And let's hope that hypotension from IVC compression post-flip or an air embolus once the fossa is opened would be easy enough to detect temporally from the events that were currently going on. The random PE is a tougher diagnosis but you'll still be empirically treating high CVP and hypotension with inotropes and vasopressors. I think even if the differential goes from 2 things to 5 things there's still plenty of utility having the CVP available in this patient.

As far as the peripheral VP, I would probably put some stock in a transduced 18 or 16 in a fat A/C vein, and maybe not so much in a 22 in a thumb vein. My guess is that pts like this one with massive venous distension probably have less error because there's less of a gradient between central and peripheral veins.

 

[Hork Bajir]

Peripheral Venous Pressure as a Hemodynamic Variable in Neurosurgical Patients

Neurosurgical patients undergoing either craniotomy or complex spine surgery are subject to wide variations in blood volume and vascular tone. The ratio of these variables yields a pressure that is traditionally mea-sured at the superior vena cava and referred to as “central venous pressure” (CVP).

insights.ovid.com

Peripheral Venous Pressure as a Measure of Venous... : Anesthesia & Analgesia

drenergic catecholamine stimulation. We simultaneously monitored PVP, CVP, and mean arterial blood pressure during resection of pheochromocytoma in a 63-yr-old woman and found excellent correlation between the three pressure variables, suggesting that fluctuations of PVP reflect overall changes...

journals.lww.com

There are plenty of papers validating the correlation between PVP and CVP. Whether you choose to care about CVP is a different issue... but for those who say that they would delay induction of this case to put in a central line to measure CVP (not an unreasonable proposition in a Fontan patient), the suggestion of PVP was just meant as food for thought

 

[2010houston]

I’m only a med student so this is naive but if the pt is 28 why would a peds guy be involved

Because in general, peds anesthesiologists have more experience with Fontan physiology than others as they take care of single ventricles in the various stages of their repairs. Though this will likely be less of an issue as these patients live longer and longer.

 

[Timeoutofmind]

A lot of what people have posted here was
The fatigue going into the case and the limited prep time definitely made it harder for me.

Little more background
I work in a large, academic, tertiary care hospital with anesthesiology residents.
I am a general anesthesiologist, I do not have subspecialty training
The patient was ambulanced over from a smaller affiliated hospital in the suburbs. She was discharged less than 24 hours prior after hospitalized for ADHF with aggressive diuresis. She was deemed not a candidate for transplant at the time due to BMI.
We had about 45 min to 1 hour notice of this emergency case and activated our neurotrauma OR upon hearing of the case.
The patient was on lovenox being bridged to warfarin, got vit k and kcentra at the OSH

Course of events
After pt arrived around 300 am, I performed a quick physical exam and conducted a brief interview with the patient's relative who accompanied her. Not much new information. Labs were spun, T+C obtained, the other results were largely unremarkable. Superstat coag studies were ok. She had 2 large bore peripheral IVs. Pt was not following commands, couldn't get a good airway examination, and had no airway history notes for review. She was likely not protecting her airway, but was not grossly aspirating, was breathing spontaneously and hemodynamically appeared ok.

The emergency medicine attending wanted to intubate her right away. It was apparent they didn't really understand the physiology aside from trying to keep the patient stable for it. I told them to it would be better for us to do the intubation in the OR. We tested to see if the patient can lay flat for 30 seconds to perform a head CT and then brought her up ASAP to the OR. Had a realistic discussion with the neurosurgeon and family about the high risks.

Arrived in OR around 320 am, kept pt HOB elevated, placed pre-induction arterial line. Gave lidocaine IV (100 mg). Cardiac stable induction, induced with etomidate (20 mg), high dose rocuronium (120 mg), touch of propofol (20-30 mg) while watching hemodynamics closely and having a variety of pressors and inotropes within reach. Intubated with glidescope, easily, one attempt. Induction was very stable. Vented on pressure control mode, being careful with airway pressures and using minimal PEEP. Hyperventilated patient to EtCO2 30, and sent off ABG. Gave 10 mg decadron IV. Didn't give antiepileptic per neurosurgery (I think not an epileptogenic area of the brain). Kept anesthetized with combo sevo/prop/fent, light neo gtt, kept muscle relaxed throughout. BIS was 20's. Neurosurgery placed a frontal EVD to help decompress. Mayfield pins applied. We then applied a magnet to the PPM and flipped the patient prone for surgical exposure.

Concerned about prone positioning and venous return, but the flip went smoothly and remained hemodynamically ok with nominal airway pressures. 1g/kg of mannitol administered, kept a close eye that it may significantly drop volume status and worsen preload. We also administered several doses of insulin at this point and had her insulin pump running on basal rate throughout, with gradual improvement in BG from 300 -> 200's. We weren't in a hurry to rapidly correct BG.

Neurosurgery was able to evacuate the subdural bleed, achieve hemostasis, and close. Minimal blood loss. A small part of the cerebellum was herniated into the surgical incision and was resected as well. Surgical closing, normalized EtCO2, flipped back supine, kept intubated to CT scan and then handed over to our anesthesia CCM team in neurosurgical ICU by 730 am. No pressors or inotropes. Propofol running at 50 mcg/kg/min. (I was kind of pissed off at the respiratory therapist who put the patient on PEEP of 10 for no reason, completely mismanaged the ventilator airway pressures were high 30's and TV 350's, and who obviously had no idea about the implications of the pt's physiology. I fixed the vent settings before I left, only to find out later she put the patient back on PEEP 10!!) Pt was extubated later that day, fully neuro intact. Postop labs looked ok. Pt took a hit to the kidneys which recovered within 48 hours.

Thought process and concerns
1. Fontan physiology and cardiac status
- recognizing the need to maintain adequate preload, avoid conditions that increased PA pressures
- presence of PPM was probably a good thing since neurosurgeon was working close to the brainstem which could cause significant HR, hemodynamic fluctuation.
- unclear volume status, unclear if patient's starting BP reflected any part of Cushing's response, unclear if muscle relaxation and PPV could cause dramatic drop in hemodynamics... especially with such obese + ascites abdomen compressing on IVC. Induction was clearly the most dangerous part of the case.
- minimize PEEP, minimize airway pressures, attempt to minimize R -> L shunting
- meticulous de-airing of lines
- c/f bleeding from varices, no intention for TEE

2. ICP and conflicting physiologic goals from point 1.
- need to prevent hypertension which could worsen bleeding, need to prevent hypotension which could decrease CPP
- ideally want to run pt dry but this could affect preload.
- considered the potential for (and actualized) renal insufficiency with diuresis.
- HOB up whenever we could
- induction with lidocaine (although hasn't been shown to significantly decrease ICP), hyperventilate, decadron, mannitol, monitor and correct Na

3. Airway
- no airway history, poor exam
- awake intubation not an option due to lack of patient cooperation, potentially worsening ICP due to coughing or bucking
- ramped up into optimal position, glidescope to maximize first pass success. if fail might have needed surgical airway.
- given severe OSA, morbid obesity, and pre-existing neuro status we planned to keep patient intubated postop

4. Endocrine
- somewhat lower priority, treat BG for gradual reduction.
- obviously want to prevent hypoglycemia.


I think if this patient also had ESRD, she would have had a much more difficult anesthetic and much much higher morbidity/mortality.

Very impressive
Great work
That patient was lucky you were in the hospital that night

 

[BigRedBeta]

Patient is 28yo. If we are doing heroics on 70-90 year olds, then what are the chances someone in their 20s wouldn’t get heroics?

And in my experience in the Peds CVICU, most of the teenagers with complex CHD develop an exaggerated sense of invincibility beyond even what the typical teenager has. Those adults that come in for fontan revisions either have gone one of two ways as they've gotten older - either they become weeping, worried, fragile adults who do nothing of any risk at all, or their sense of invincibility only grows larger...

My guess, given all the care that she currently receives, this woman is probably the latter.

 

[WholeLottaGame7]

Sorry, I'm not sure what you mean. Can you clarify this?
Pt has known R -> L shunt with baseline sats in mid 80's on RA.
I'm assuming there is some sort of fenestration, although notes were unclear and i wasn't going to waste too much time digging for something when time was sensitive and a solution would be relatively straightforward

You said the patient has "known" R --> L shunt, but it wasn't clear if there was TTE evidence of a shunt or if you were assuming that based on the sats. Generally speaking, there are no intracardiac shunts remaining in a Fontan, unless a fenestration was intentionally left as a pop-off to preserve cardiac output at the expense of O2 sats. And oftentimes those clot/close off/are closed off once the Fontan has stabilized post-operatively. It's certainly possible that there's a fenestration still open, and there's R-->L flow across it, causing hypoxia, but there are also many other reasons a failing Fontan might be hypoxic, especially one of this size/with these comorbidities.

 

[WholeLottaGame7]

And in my experience in the Peds CVICU, most of the teenagers with complex CHD develop an exaggerated sense of invincibility beyond even what the typical teenager has. Those adults that come in for fontan revisions either have gone one of two ways as they've gotten older - either they become weeping, worried, fragile adults who do nothing of any risk at all, or their sense of invincibility only grows larger...

My guess, given all the care that she currently receives, this woman is probably the latter.

I'd say the same thing about CF patients...