Interesting thoracic case...

[bcat85]

I always enjoy the clinical cases presented on here. Here's one I had earlier this week. 30's F with a history of tricuspid atresia s/p Fontan in infancy with a bidirectional glenn shunt about 10 years ago. Getting along alright with occasional dyspnea requiring diuresis. Admitted with septic shock--cellulitis is the presumed source complicated by renal failure, DIC (platelet count in the 60's and INR of 2.4), and anasarca secondary to her baseline protein-losing enteropathy in addition to the 14-days of shoddy nutrition in the ICU. She also has Afib with an AICD/pacemaker.

In the meantime, she develops pneumonia with a persistent parapneumonic effusion, and the thoracic surgeons want to take her for a decortication.

Go.

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[Noyac]

Is she tubed? Any drops she is on?

 

[OB1🤙]

Gnarly. Please clarify one thing: the Fontan comes after the Glenn. A procedure in infancy is generally a variant of a BT shunt, followed by a Glenn, with the Fontan being the final stage.

So is this a Glenn or a Fontan patient? Makes a big difference in management. Assuming the latter, do we know if it's a fenestrated Fontan?

 

[sevoflurane]

Agree with HB. Also, what are her sats now? Will she be able to tolerate OLV? Createnine? Is she dialyisis bound? What's the echo show and what are her B.Ps? She's in DIC with out of whack coags? What's her fibrinogen? ABG? Sorry. More questions than asnwers. Tough case.

 

[urge]

Pent sux tube.

She should come lined up from icu. Don't think OLV is needed, or a good idea, for the case.

Why does she have an ICD?

 

[bcat85]

Is she tubed? Any drops she is on?

No tube. Weaned off of levophed about a 2 days ago. Baseline BP's in the 80's/40's range

Gnarly. Please clarify one thing: the Fontan comes after the Glenn. A procedure in infancy is generally a variant of a BT shunt, followed by a Glenn, with the Fontan being the final stage.

So is this a Glenn or a Fontan patient? Makes a big difference in management. Assuming the latter, do we know if it's a fenestrated Fontan?

Good catch. She has Fontan physiology, but it is not fenestrated.

Agree with HB. Also, what are her sats now? Will she be able to tolerate OLV? Createnine? Is she dialyisis bound? What's the echo show and what are her B.Ps? She's in DIC with out of whack coags? What's her fibrinogen? ABG? Sorry. More questions than asnwers. Tough case.

Sats are ok. On eval in the ICU preop, BP's are in the 80's/40's (reportedly baseline), SpO2 in the low 90's, and HR Afib with RVR 140's (they attempted most everything including MAZE and ablation to try to treat this, but the a fib has been refractory). Previously on CVVHD, but now weaned off with improving renal function. Echo shows decent ventricular function. Coags are improving too (INR 2.0, fibrinogen normal). Platelets are in the 70's. H/H are low-normal.

 

[sevoflurane]

oooohhh... RVR at 14o no es bueno. This usually precludes any trips to the OR until it is at least rate controlled.

Maybe I'm missing something here, but we do OLV on loculated/parapneumonic effusions all the time. If you get into trouble you can always bring up the down lung. I agree however, OLV is not necessary for this case. Those purulent catacombs are disgusting.

 

[Gas you down]

Gnarly. Please clarify one thing: the Fontan comes after the Glenn. A procedure in infancy is generally a variant of a BT shunt, followed by a Glenn, with the Fontan being the final stage.

So is this a Glenn or a Fontan patient? Makes a big difference in management. Assuming the latter, do we know if it's a fenestrated Fontan?

folks we have just waded into the deep end! i had to go look it up, i'm not gonna lie.

we rarely have to do OLV on our decort's. i'd just tell the surg beforehand if i didn't think she'd take it, and he'd deal with it (at my hospital anyway). RVR and coagulopathy needs to be fixed prior to coming to OR.

cellulitis of what by the way? OP started with something about septic shock. so what's the word on that.

 

[Triple AAA]

No tube. Weaned off of levophed about a 2 days ago. Baseline BP's in the 80's/40's range

Good catch. She has Fontan physiology, but it is not fenestrated.

Sats are ok. On eval in the ICU preop, BP's are in the 80's/40's (reportedly baseline), SpO2 in the low 90's, and HR Afib with RVR 140's (they attempted most everything including MAZE and ablation to try to treat this, but the a fib has been refractory). Previously on CVVHD, but now weaned off with improving renal function. Echo shows decent ventricular function. Coags are improving too (INR 2.0, fibrinogen normal). Platelets are in the 70's. H/H are low-normal.

Contrary to your initial post, it would appear that this patient does not have septic shock, DIC, or renal failure. That changes the pre-operative risk significantly.

That leaves us with malnutrition and Afib w/ RVR. While the latter would normally concern me, it appears that this condition is as optimized as possible, and this surgery is urgent given the need for source control. That leaves us with hypoalbuminemia, an independent predictor of increased morbidity and mortality. To me, this would be the most compelling reason to think twice about sending this lady to the OR.

Not being a pediatric anesthesiologist, I admit I do not have a strong knowledge of Fontan physiology and it's possible complications after general anesthesia. I would ask one of my colleagues with such training to evaluate this patient prior to surgery. However, it would appear that the patient is reasonably functional/optimized in that respect. Even if it weren't, we may not have a choice if this were declared a true emergency.

If it were me, I would have a discussion with the thoracic surgeon about the risk vs benefit of going to the OR under general anesthesia instead of having a chest tube placed at bedside. If this had to go to OR, I would do everything possible to avoid lung isolation. I may even consider iNO or inhaled vs IV epoprostenol. The silver lining is that no lung tissue is removed, and this ideally would not be a very long procedure.

I would want an Aline and CVC, and even a PAC if it were not contraindicated by her congenital defects. I would not be able to meaningfully interpret the TEE alone and would request the presence of a peds cardiologist or peds anesthesiologist if one were to be used.

 

[deleted171991]

I am not Peds either, but is it only me, or does this lady sound not optimized for the OR? (RVR 140??? INR 2, s/p recent sepsis, emaciated, hypoproteinemic, God knows what else.)

That parapneumonic effusion should be first treated with a chest tube, IMO.

 

[sevoflurane]

OLV is possible with fontan physiology. In fontan you def. want to keep PVR as low as possible as flow is passive (abscence or rudementery RV). Pulmonary vasodialators may indeed be required to offset the increase in PVR by OLV. You may be able to optimize surgical conditions and make it a quick in and out... if you run into trouble, you may just give the down lung back. I think I remember doing one or two of these years ago.

CT prolly won't get rid of the loculated puss. May help tho. Taking down the catacombs will help the most.

I'm not sure I'd be able to interpret the info on a PAC as there is really no driving force behind the pressure and it would be pretty hard to float (prolly end up in the RA or IVC). I guess trends and a slowly rising CVP would give you some clues as to a decrease in flow due to congestion. TEE is your friend here. I haven't see a patient like this in a while so I'm a little out of my league here.

Cool case.

 

[HalO'Thane]

If she has normal cardiac function I would probably avoid placing a central line, particularly if you are not expecting large fluid shifts. I would be concerned about introducing any source of thrombi or infection into the fontan circuit. As mentioned, I also think a PAC would be difficult to float and interpret and not worth the risks. 2 PIV's, Art Line for lab sampling and beat to beat variability of the BP, "narcotic" induction (as even well functioning fontan patients do not tolerate the drop in preload with standard induction doses of propofol), tube, magnet on the patient, rate control, blood products in the room, likely post-op vent.

 

[OB1🤙]

So in Fontan physiology, the cardiac output is inversely related to the PVR. If the Fontan is fenestrated, there is a "pop off" of sorts, as backed up venous blood can enter the systemic circulation, maintaining cardiac output and limiting the increase in CVP at the expense of systemic oxygenation.

If not fenestrated, you must not let the PVR increase excessively, so OLV is a dicey proposition. However, 1) the patient needs the procedure, no other way to get source control and 2) as Sevo points out, you can reexpand the lung as needed if you fun into trouble.

No role for us to place a PAC in a Fontan patient, a central line may be useful for vasoactive medications in this particular patient, that's a judgment call depending on how things look. My default is not to place one but if there's sepsis afoot it could help. As mentioned, TEE is your best friend if things go bad.

iNO or Flolan would be good to have immediately available.

I would use vasopressin as a first line pressor, as it will selectively increase the SVR without increasing PVR. A little levophed is OK, but not too much.

Bottom line, avoid hypoxemia/hypercarbia, minimize PVR, OLV can be attempted but may not be well tolerated, tell the surgeon to get in and get out fast fast fast.

One last thing- the pulmonary flow in a Fontan isn't really passive. Its driving force is the systemic ventricle, and if the ventricle has good systolic and diastolic function, and the pulmonary venous pressures are low, these patients will do OK. It's when there's pulmonary venous hypertension that backs up through the Fontan system that they start to teeter.

 

[Orin]

I always enjoy the clinical cases presented on here. Here's one I had earlier this week. 30's F with a history of tricuspid atresia s/p Fontan in infancy with a bidirectional glenn shunt about 10 years ago. Getting along alright with occasional dyspnea requiring diuresis. Admitted with septic shock--cellulitis is the presumed source complicated by renal failure, DIC (platelet count in the 60's and INR of 2.4), and anasarca secondary to her baseline protein-losing enteropathy in addition to the 14-days of shoddy nutrition in the ICU. She also has Afib with an AICD/pacemaker.

In the meantime, she develops pneumonia with a persistent parapneumonic effusion, and the thoracic surgeons want to take her for a decortication.

Go.

I'm concerned by doing a decortication on someone with a severe coagulopathy and sepsis. This kinda source control is not going to go well. I'd love to just put a chest tube in, shake and back with some TPA x 3, and let her settle out.

 

[Triple AAA]

In fontan you def. want to keep PVR as low as possible as flow is passive (abscence or rudementery RV)...

I'm not sure I'd be able to interpret the info on a PAC as there is really no driving force behind the pressure and it would be pretty hard to float (prolly end up in the RA or IVC). I guess trends and a slowly rising CVP would give you some clues as to a decrease in flow due to congestion. TEE is your friend here. I haven't see a patient like this in a while so I'm a little out of my league here.

Cool case.

You bring up a good point.

The concerns for lowering PVR was my rationale for wanting to place a PAC, so that I could directly titrate pulmonary vasodilator therapy to RV afterload. As I haven't been in this situation before, I wasn't aware of the difficulty of placing it. I too, would be out of my league.

 

[OB1🤙]

You bring up a good point.

The concerns for lowering PVR was my rationale for wanting to place a PAC, so that I could directly titrate pulmonary vasodilator therapy to RV afterload. As I haven't been in this situation before, I wasn't aware of the difficulty of placing it. I too, would be out of my league.

As you now realize, there is no RV here to offload.

 

[itwasalladream]

Nice case, and a lot of good ideas.

My approach would be first see if we can't improve coagulopathy and HR. Then pre-induction art line, good PIVs, careful induction, and TEE to start with iNO in room. TEE will help with volume status and diagnosis of pump failure vs sepsis vs hypovolemia when they start stirring up the chest. I'd avoid placing a CVC, mainly to avoid any chance of clot or stenosis in the fontan circuit. Avoid OLV, or do the usual stuff to deliver a bit more O2 to the down lung, minimizing hypoxic vasoconstriction. Keep a close eye on volume status and keep volume up. Err on the side of hyperventilation, lower intrathoracic pressures/lung volumes. iNO at the first sign of trouble. Agree with HB on vaso, but I think norepi isn't that much different, especially if you have iNO readily available. Extubate if she looks good and isn't on iNO at the end to get her back to spontaneous respiration as quickly as possible.

 

[Noyac]

This case doesn't make any sense to me:
DIC
not tubed but pneumonia bad enough for a decortication, yeah right!
Not on levo therefore sepsis debatable

Things just are not adding up for me.

 

[deleted171991]

The definition of sepsis does not include pressors. Good point with the ETT, though, Noyac.

Another thing to consider: if she survived a septic shock with that ventricular rate, at least she has good cardiac reserve.

 

[bigdan]

Agree with Noyac that the patient's presentation isn't exactly adding up.

Agree with those saying to further optimize before OR, if she goes to the OR at all.

From the initial post, I would've guessed the follow-up post would have been her M&M or autopsy. But alas, she's not only alive, but better. If she can somehow improve renal function and coagulopathy without the OR, and has managed to stay extubated - with her comorbidities AND a new onset pneumonia - I'd just keep her in whatever ICU spot she's in, and suggest some form of bedside drainage until things get even better. I'd give her whatever form of high protein chow your hospital offers, and am impressed she's made it 2 weeks without TPN/PPN. Peds/Congenital EP service to help control that HR, and do all the housekeeping to ensure electrolytes & volume status aren't driving that AFib. Thoracic should come back in a week, or try percutaneous drainage. Get her out of bed and make her human again.

I'm curious - do you know the effusion & pneumonia development time course? Did she fail prior effusion drainage? How long has she been in the hospital before coming to you?

It's impressive she's done so well on her own.

 

[Lurch]

Who has a strong preference/opinion for mechanical ventilation strategies in a Fontan patient? Obviously avoiding hypercarbia, acidosis, hypoxia is essential; maintaining spontaneous ventilation preferable (if possible) and minimizing intrathoracic pressures. Beyond these basic tenets, what strategies would you employ? Would you accomplish a respiratory alkalosis by first adjusting respiratory rate to minimize the effects of increased tidal volume on peak airway pressure? Or...would you deliver higher tidal volumes with lower RR with a short I time effectively lowering overall mean airway pressure? How would you adjust these strategies with one lung ventilation?

 

[HalO'Thane]

Vent strategies IMHO are very important. The keys, as you mentioned, are to minimize airway pressure and to enhance passive venous return. This is usually accomplished with a relatively larger TV (8-10 ml/kg), lower RR, long expiratory time (I:E ratio of 1:2.5-1:3) and physiologic PEEP (4-5 mm Hg). Spontaneous ventilation probably wouldn't be feasible in this case. I also try to avoid respiratory alkalosis because the minute ventilation needed might impair preload and raise airway pressures and because it may lead to cerebral vasoconstriction, which can again effect venous return to the fontan circuit.

 

[urge]

Not sure why some of you want to "optimize" her (treat the RVR, feed her, treat the coagulopathy...). The way I see it she has an infected effusion. That's not going to get better. Give her albumin, ffp if bleeding, and leave her HR alone. She has done fine so far.

 

[sevoflurane]

No tube. Weaned off of levophed about a 2 days ago. Baseline BP's in the 80's/40's range


.....HR Afib with RVR 140's (they attempted most everything including MAZE and ablation to try to treat this, but the a fib has been refractory).

I guess I'm not clear about her afib. Maze and ablation failure doesn't necessarily preclude rate control. Is she chronically in the 140s? If the HR of 140 is new, I'm not taking her to the OR unless this is indeed a documented urgency/emergency. Her sats are in the low 90's and she's not intubated. We have time here to manage the HR if it's new afib w/ RVR. Funny things can happen to the heart rhythm once you start mucking around in the chest. Going from 140 to 220 bpm is not inconceivable. If the HR of 140 is chronic then that's a little different. Def. put pads on if this case goes to the OR weather it's chronic or new.

 

[bcat85]

Lots of great points, and you guys identified several things that I was concerned about when I first saw her in the ICU. They had previously attempted to drain the effusion with a chest tube, but weren't able to break up the loculations and felt that a decort was the only way to effectively achieve source control. She remained in raging RVR, despite amio and dig. They were (appropriately) reticent to start any BB/CCB on her because of her marginal blood pressures.

So in Fontan physiology, the cardiac output is inversely related to the PVR. If the Fontan is fenestrated, there is a "pop off" of sorts, as backed up venous blood can enter the systemic circulation, maintaining cardiac output and limiting the increase in CVP at the expense of systemic oxygenation.

If not fenestrated, you must not let the PVR increase excessively, so OLV is a dicey proposition. However, 1) the patient needs the procedure, no other way to get source control and 2) as Sevo points out, you can reexpand the lung as needed if you fun into trouble.

No role for us to place a PAC in a Fontan patient, a central line may be useful for vasoactive medications in this particular patient, that's a judgment call depending on how things look. My default is not to place one but if there's sepsis afoot it could help. As mentioned, TEE is your best friend if things go bad.

iNO or Flolan would be good to have immediately available.

I would use vasopressin as a first line pressor, as it will selectively increase the SVR without increasing PVR. A little levophed is OK, but not too much.

Bottom line, avoid hypoxemia/hypercarbia, minimize PVR, OLV can be attempted but may not be well tolerated, tell the surgeon to get in and get out fast fast fast.

One last thing- the pulmonary flow in a Fontan isn't really passive. Its driving force is the systemic ventricle, and if the ventricle has good systolic and diastolic function, and the pulmonary venous pressures are low, these patients will do OK. It's when there's pulmonary venous hypertension that backs up through the Fontan system that they start to teeter.

Vent strategies IMHO are very important. The keys, as you mentioned, are to minimize airway pressure and to enhance passive venous return. This is usually accomplished with a relatively larger TV (8-10 ml/kg), lower RR, long expiratory time (I:E ratio of 1:2.5-1:3) and physiologic PEEP (4-5 mm Hg). Spontaneous ventilation probably wouldn't be feasible in this case. I also try to avoid respiratory alkalosis because the minute ventilation needed might impair preload and raise airway pressures and because it may lead to cerebral vasoconstriction, which can again effect venous return to the fontan circuit.

This is essentially exactly what we did. We did end up going on OLV and the patient seemed to tolerate it well. 2 big PIV's, a DLT, and a pre induction arterial line + a magnet and zoll pads. We didn't place a central line for the reasons outlined above, and we had iNO available in the room. We basically used the same ventilatory strategy that HalO'Thane talks about above to try to minimize the influence of PPV on her PVR. Her RVR worsened intraoperatively (HR up to the 160's/170's), but she seemed to maintain her BP and was very volume responsive. We left her intubated and took her to the CVICU postoperatively, where she was extubated that night. She ended up doing well. Overall though, it really made me think about managing and optimizing PVR intraoperatively--something that isn't normally at the forefront of our thought process.

 

[urge]

Lots of great points, and you guys identified several things that I was concerned about when I first saw her in the ICU. They had previously attempted to drain the effusion with a chest tube, but weren't able to break up the loculations and felt that a decort was the only way to effectively achieve source control. She remained in raging RVR, despite amio and dig. They were (appropriately) reticent to start any BB/CCB on her because of her marginal blood pressures.





This is essentially exactly what we did. We did end up going on OLV and the patient seemed to tolerate it well. 2 big PIV's, a DLT, and a pre induction arterial line + a magnet and zoll pads. We didn't place a central line for the reasons outlined above, and we had iNO available in the room. We basically used the same ventilatory strategy that HalO'Thane talks about above to try to minimize the influence of PPV on her PVR. Her RVR worsened intraoperatively (HR up to the 160's/170's), but she seemed to maintain her BP and was very volume responsive. We left her intubated and took her to the CVICU postoperatively, where she was extubated that night. She ended up doing well. Overall though, it really made me think about managing and optimizing PVR intraoperatively--something that isn't normally at the forefront of our thought process.

And this is why all my anesthetic plans are Pent Sux Tube.

You start the story with a "super sick" patient and then finish it saying that you didn't do anything out of ordinary.


I bet that if you had a 20 yr old Olympic marathon runner coming for the same procedure you would have done the same thing.