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Adult congenital disease in the ICU
2 Plays1 year ago
Due to advancements in medical and surgical care, the survival of patients with congenital conditions into adulthood has dramatically increased. However, as these individuals transition to adulthood, their unique physiology, chronic complications, and evolving care needs create significant challenges for their management when they are admitted to adult intensive care units (ICUs). This episode will discuss adult congenital disease in the ICU. Dr. Zanotti is joined by Dr. Cameron Dezfulian, a pediatric and adult critical care physician. He is the director of the Adult Congenital Heart Disease Program Development for the Section of Critical Care at Texas Children's Hospital and a faculty member at Cardiothoracic Critical Care at Baylor St. Luke's Medical Center. He is also a Senior Faculty member at Baylor College of Medicine in Houston, Texas.
Additional resources:
ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease. Circulation 2008: https://www.ahajournals.org/doi/pdf/10.1161/CIRCULATIONAHA.108.190690
Special Considerations in Critical of the Congenital Heart Disease Patient. E Neethling, et al. Can J Cardiol. 2023: https://pubmed.ncbi.nlm.nih.gov/36682483/
Management of the Critically Ill Adult with Congenital Heart Disease. WB Kratzert, et al. J Cardiothorac Vasc Anesth 2018: https://pubmed.ncbi.nlm.nih.gov/29500124/
Adults with childhood-onset chronic conditions admitted to US pediatric and adult intensive care units. J Edwards, et al. J Crit Care 2015: https://pubmed.ncbi.nlm.nih.gov/25466316/
Down Syndrome. MJ Bull. N Eng J Med 2020. https://www.nejm.org/doi/full/10.1056/NEJMra1706537
Books and Music mentioned in this episode:
The Bible: https://bit.ly/3EK4LL6
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Transcript
Podcast Introduction
00:00:06
Speaker
Welcome to Critical Matters, a sound podcast covering a broad range of topics related to the practice of intensive care medicine.
00:00:14
Speaker
Sound provides comprehensive critical care programs to hospitals across the country.
00:00:19
Speaker
To learn more about our programs and career opportunities, visit www.soundphysicians.com.
00:00:26
Speaker
And now your host, Dr. Sergio Zanotti.
Impact of Advancements in Medical Care
00:00:32
Speaker
Due to advancements in medical and surgical care, the survival of patients with congenital conditions into adulthood has dramatically increased.
00:00:39
Speaker
However, as these individuals transition to adulthood, their unique physiology, chronic complications, and evolving care needs create significant challenges for their management when they are admitted to the adult intensive care unit.
Focus on Adult Congenital Disease in ICUs
00:00:51
Speaker
Today's podcast episode will discuss adult congenital disease in the ICU.
00:00:57
Speaker
Our guest is Dr. Cameron Desfoulian, a pediatric and adult critical care physician.
00:01:01
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He is the director of adult congenital heart disease program development for the section of critical care at Texas Children's Hospital.
00:01:08
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He is faculty for cardiothoracic critical care at Baylor St.
00:01:11
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Luke's Medical Center and a senior faculty member at Baylor College of Medicine in Houston, Texas.
00:01:17
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It's a true honor and pleasure to have him as our guest today.
00:01:20
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Cameron, welcome to Critical Matters.
00:01:23
Speaker
Thank you, Sergio.
00:01:24
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Really pleasure to be here.
00:01:25
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Well, this is a topic that I know fascinates both of us for different reasons, but definitely, I mean, you with your extensive training in pediatric and adult medicine and then critical care obviously have, I think, a very unique perspective on this growing population.
00:01:42
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But I would like to start maybe with why should other intensivists who don't have your niche and training care about this topic?
00:01:51
Speaker
Yeah, well, great question.
00:01:52
Speaker
I mean, I think the practical answer is because it's coming your way.
00:01:56
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And if it's not already there, the reality is, is across the spectrum of what used to be called pediatric illness.
00:02:03
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So that's kids with genetic defects, syndromic children, you know,
00:02:07
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our preemies, and really I'm talking about our micro preemies, adults who are now, you know, used to be children with congenital heart disease.
00:02:16
Speaker
Our survivals are incredible, right?
00:02:18
Speaker
But what that means is that there's all these patients who had chronic illnesses that would die as children that adult intensivists never dealt with that now all of a sudden are surviving into adulthood.
00:02:30
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So there's a lot of intensivists who, if they already haven't experienced it, are going to be seeing lots of
00:02:37
Speaker
chronically ill young adults, because these are often 20s and 30s, who have illnesses that started at the moment of birth and have been managed for the first two decades of their life by a pediatric group, often not just one physician, but a group of physicians.
00:02:53
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And now all of a sudden they're transitioning to adult care.
00:02:56
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And I think there hasn't been a whole lot of
00:02:59
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planning, education, or kind of multidisciplinary meeting of minds between the two sides of peds and adults.
00:03:06
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And so basically, this is a group of patients that's coming to you soon if you're an adult intensivist, but you may or may not be fully aware of what's coming to you.
00:03:15
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Yeah.
00:03:16
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And I read it.
00:03:16
Speaker
I don't know if this is a true stat, but in terms of, like you said, they're coming our way.
00:03:22
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I read that specifically with heart disease, there are more adults with congenital heart disease than kids with heart disease right now, congenital heart disease.
00:03:32
Speaker
So as they live longer and longer, that population keeps growing, right?
00:03:37
Speaker
Absolutely.
00:03:38
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And I think that, like you mentioned, this also applies, as we'll talk, to many other conditions.
Transitioning Challenges to Adult ICUs
00:03:43
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So why are these patients different from any other adult patient in the ICU?
00:03:48
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Yeah, well, I mean, I think the, you know, the chronic illnesses or chronic comorbid conditions that these patients carry are very different than what I think we're used to as adult intensivists.
00:04:00
Speaker
So, you know, our classic chronic conditions that we deal with all the time are atherosclerotic disease, obesity, diabetes, hypertension, you know, things like that, maybe a prior stroke, dementia,
00:04:11
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that's very different than what the chronic comorbidities of these patients are.
00:04:16
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And with that, that's a lot of the challenge in critical illness because you've got to then deal with what is essentially the baseline.
00:04:24
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It's not a normal baseline.
00:04:26
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It's a different baseline than what you're used to.
00:04:29
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So you have to understand what's the best case scenario, where are you starting from, which I think most of us would agree that in critical illness, our hope is to get back to baseline.
00:04:40
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We're not going to improve the patient in most situations, but we want to get them back to the baseline.
00:04:45
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But if you don't understand what that baseline is and how it impacts your patient, then you really don't know what you're shooting for.
00:04:52
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And the other question I wanted to ask you before we move on to more specific conditions is, historically, when this group was a smaller group, they just stayed in the care of pediatricians.
00:05:03
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What's changed?
00:05:05
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Well, I think the biggest thing that's changed is the sheer numbers and the age of these patients, right?
00:05:10
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So you're absolutely right.
00:05:12
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They used to stay within the PETE setting, but that was because, you know, let's take like, you know, congenital heart disease or, you know, in the old days, cystic fibrosis before the care got so good.
00:05:22
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Chances are they were going to die by the time they got to 30.
00:05:25
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So the pediatricians would just kind of flex a little and handle the 20, 30 year olds.
00:05:30
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Now, all of a sudden, you're dealing with adults with congenital heart disease who are in their 50s and 60s who now have the overlay of adult disease, things like atherosclerosis and dementia happening and a variety of other adult problems such that the pediatricians don't feel equipped to handle it.
00:05:47
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And yet...
00:05:48
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the adults don't really feel equipped handling it either because they don't understand the pediatric illness.
00:05:52
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So these patients really start falling through the cracks.
00:05:55
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It becomes really challenging for them to be managed in either setting.
00:05:59
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Many pediatric hospitals, as this phenomenon has been going on, have started setting hard and fast rules as to when patients have to transition.
00:06:07
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And so most places right now are setting numbers between 21 and 25 years of age where they basically tell their clinicians,
00:06:16
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you have to stop admitting patients after this age.
00:06:18
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And so it forces the issue of transition because of the problems that were being created by physicians who were holding on a little too long to the patients.
00:06:27
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And yet what that means is that these patients now are delivered to adult centers where they're not necessarily equipped to handle what the comorbidities are.
00:06:37
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Let's dive into some of the specific congenital diseases that might result in adults with these diseases presenting to an ICU care.
Congenital Heart Disease in Adult ICUs
00:06:48
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And there's three big categories that I wanted to go over.
00:06:52
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First and foremost is congenital heart disease.
00:06:55
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Second is genetic diseases and syndromes.
00:06:57
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And third, I mean, you talked about the prematurity-related conditions in adults or like the super preemies.
00:07:03
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which seems to be something that we are younger and more and more premature babies are surviving to adulthood.
00:07:10
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So that has its own set of problems.
00:07:12
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And maybe we can start with congenital heart disease, which I understand is the most common congenital defect in newborns, affects thousands of babies every year in the United States.
00:07:24
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What are some of the common conditions that might end up in the ICU of an adult and also some of the general ICU considerations that you would consider there?
00:07:34
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Sure.
00:07:35
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Thanks.
00:07:35
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So, you know, when we talk about congenital heart disease, first of all, we're talking about roughly nine in a thousand births.
00:07:42
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Okay.
00:07:42
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So just short of 1% have congenital heart disease and the vast majority of those.
00:07:49
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So about seven out of those nine are, you know,
00:07:54
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forms of congenital heart disease that honestly are not all that impactful in the long term.
00:07:58
Speaker
So when we talk about things like atrial septal defects, ventricular septal defects, patent ductus arteriosus, most of those are handled fairly early in life.
00:08:08
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The treatment is essentially curative and the comorbidity 10, 20 years later is next to nothing.
00:08:15
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So that if you have simple congenital heart disease,
00:08:19
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you know, your life expectancy and your chances of having a cardiovascular death are no more increased than the general population.
00:08:27
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So I almost put those aside because they don't need specialty care, nor do I think most intensivists need to think much about it beyond just the, you know, like, huh, that's interesting.
00:08:37
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This guy had an ASD.
00:08:38
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But then when we start talking about some
00:08:40
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of the more what would be considered by the Bethesda criteria moderate or high complexity anatomic lesions.
00:08:47
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Those are the ones that really do have quite a bit of cardiovascular excess mortality and morbidity when they start getting into adulthood.
00:08:57
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And those are the ones that are going to need some very strong
00:09:00
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kind of, you know, out of the ordinary planned out care pathways that are not typical and are not something that's going to just come readily to your average critical care physician.
00:09:13
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So now we're talking about things like Tetralogy of Fallot, transposition of the great arteries.
00:09:18
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a variety of lesions that essentially end up in a fontan, which means you have a single ventricle, whether that's a right ventricle or left ventricle pumping, and then a variety of lesions that wind up with Eisenbanger's disease, which is essentially pulmonary hypertension taken to the extreme where you have systemic pulmonary pressures.
00:09:39
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And then finally, Eisenmanger's physiology and Eisenmanger's syndrome, which is a dysplastic tricuspid valve and atrialization of the right ventricle that then leads to lots of problems with RV failure as well as arrhythmias.
00:09:53
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So that list of lesions ends up
00:09:57
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having high rates of mortality in adulthood and a lot of very unique comorbidities and complications that your average intensivist is just not going to be used to taking care of.
00:10:11
Speaker
And obviously, as we were discussing earlier, one of the biggest problems with this population is that our adult intensivists like myself just don't know what they don't know, right?
00:10:21
Speaker
And you're treating somebody in their 40s and you are missing things that...
00:10:26
Speaker
are very common in these pathologies.
00:10:29
Speaker
The other thing that I know and maybe you can comment that's very unique about particularly complex congenital heart disease is that the way they are fixed keeps evolving and changing.
00:10:42
Speaker
So then you have all these pockets of patients surviving with different physiology, right?
00:10:47
Speaker
Yeah, absolutely.
00:10:48
Speaker
Absolutely.
00:10:49
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So, I mean, a great example of that that I think you're very familiar with is transposition of the great arteries.
00:10:54
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And so in the case of transposition, detransposition of the great arteries, so that particular form, the older surgeries, which are no longer performed or what are known as the sending or mustard shunts, which essentially are atrial switch operations.
00:11:10
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So
00:11:11
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Transposition is one where the great vessels, the aorta and the pulmonary artery are not aligned with the ventricles, you know, the right ventricle and the left ventricle.
00:11:22
Speaker
And the circulations are essentially running in parallel, but not connected and going the wrong way, meaning oxygen poor blood is staying on the right side and oxygen rich blood staying on the left side, but they're not communicating, right?
00:11:34
Speaker
And so you have to fix that.
00:11:36
Speaker
And the old way of fixing that was to switch the atrial flow of blood.
00:11:41
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and ultimately to deliver the blood that was going into the right atrium into the left ventricle and into the left atrium into the right ventricle, which then fixed the kind of parallel circuits, but created the issue that you had a systemic right ventricle, as well as this baffling between the atria that then was susceptible to lots of arrhythmias and clots.
00:12:03
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For those reasons and all the complications that resulted of those surgeries, now the standard of care has become what's called the arterial switch operation.
00:12:11
Speaker
So now you're switching the pulmonary artery and the aorta and re-implanting the coronaries.
00:12:17
Speaker
Those have their own core morbidities.
00:12:18
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They're very different than that of the atrial switch operations.
00:12:24
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if you did not take care of the atrial switch operations, and I'm 50 years old, and the reality is the atrial switch had already stopped by the time I went through fellowship.
00:12:32
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And so I know these folks only as adults with congenital heart disease.
00:12:38
Speaker
You know, these operations now, you're still going to encounter the patients because they have the disease, but the pediatricians aren't even dealing with
00:12:45
Speaker
it because it's not in front of them.
00:12:46
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So the only folks that are seeing them are going to be adult intensivists, but it's an operation that historically is no longer happening.
00:12:54
Speaker
And so I think the knowledge of what the complications and all is now becoming isolated to a very select group of people.
00:13:00
Speaker
And the biggest group in this particular disease process is adult congenital heart disease cardiologists, which there's about 570 in the country.
00:13:09
Speaker
So they exist, but it's not plentiful.
00:13:13
Speaker
And what are some of the ICU considerations for these patients with congenital heart disease that make it to the ICU as adults?
00:13:21
Speaker
Well, so there's a variety of issues that need to be dealt with.
00:13:24
Speaker
First of all, arrhythmias is a huge problem, getting up to almost 50% of patients with complex disease by the time they're getting into adulthood.
00:13:33
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Heart failure is a huge issue.
00:13:36
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And the arrhythmias and the heart failure don't necessarily look like what we're used to in structurally normal
00:13:41
Speaker
The arrhythmias are, you know, with structurally normal hearts, the most common thing we see on the suprasintricular level is atrial fibrillation and flutter.
00:13:49
Speaker
And that happens in congenital heart disease also.
00:13:53
Speaker
But there's a variety of other arrhythmias, things like intra-atrial re-entrant tachycardias.
00:13:59
Speaker
You have wolf-clurps and white and aberrant pathways.
00:14:01
Speaker
You've got a variety of atrial tachycardias that are from different foci.
00:14:06
Speaker
You have re-entrant circuits that are essentially happening because of scarring and
00:14:10
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and areas where there's suture lines and things that happen from multiple repairs.
00:14:16
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And those are much harder to deal with, both in terms of medication and also in terms of electrophysiologic ablations than the standard arrhythmias we have.
00:14:25
Speaker
The heart failure, which results in a lot of patients, is because they have a systemic right ventricle.
00:14:29
Speaker
So that right ventricle is trabeculated.
00:14:31
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It has two muscle layers instead of three.
00:14:33
Speaker
It's not
00:14:35
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dealing with the same morphology as what you dealt with in a left ventricle.
00:14:40
Speaker
And so how to handle that and what that ventricle is capable of is very different than what a systemic left ventricle is capable of.
00:14:47
Speaker
You know, when you start talking about considerations of advanced supports and things like putting an impella in or putting a fad in place, that's also very different when you have anatomic constraints such as a diminutive left ventricle or right ventricle
00:15:04
Speaker
common atria, things that really make it challenging to use the technology that we're using right now with our standard structurally normal hearts in this congenital population.
00:15:15
Speaker
So for all those reasons, you have to really give a lot of thought to the approach to these patients.
00:15:21
Speaker
And it's not straightforward and algorithmic.
00:15:24
Speaker
And a lot of what we have is our standard
00:15:27
Speaker
kind of algorithms and protocols for structurally normal hearts just don't apply or won't be very successful in terms of resolving the arrhythmias and the heart failure in these patients.
00:15:38
Speaker
And the last quick thing I'll add is just the issue of endocarditis.
00:15:41
Speaker
You know, these patients, by virtue of their multiple repairs and then the subsequent work that we do now with transcutaneous placement of valves and a variety of devices like AICDs and
00:15:51
Speaker
sequential pacing leads and things like that have a lot of prosthetic material in their body.
00:15:56
Speaker
So the risk of endocarditis is present and it's been growing because we're doing a lot of things such as valve and valve repairs that are creating turbulence and are setting them up for both for thrombosis and also for endocarditis.
00:16:09
Speaker
And so a lot of these patients fly under the radar and we're
00:16:13
Speaker
you know, intensivists will get them and not recognize that this is somebody who has indolent endocarditis.
00:16:18
Speaker
And instead you're treating their heart failure or their arrhythmia and not understanding why they keep having the trouble they're having.
00:16:25
Speaker
And another thing that I think is very important that you can comment on, Cameron, is the reliance on what we usually do for hemodynamic monitoring for our patients might not work.
00:16:36
Speaker
And also maybe our approach with mechanical ventilation needs to be rethought.
00:16:39
Speaker
These are things that we just bread and butter in the ICU, but maybe in these populations, if we're not really thinking about it, we might have a little bit of a misfit.
00:16:46
Speaker
Could you talk about that?
00:16:47
Speaker
Yeah, I mean, you're 100% correct.
00:16:49
Speaker
So let's start with the monitoring.
00:16:51
Speaker
You know, if you take somebody like a Fontan, their IVC and SVC ultimately are converging in their pulmonary arteries.
00:16:57
Speaker
So there's no way to put a standard PA catheter in.
00:17:01
Speaker
If you try to float a PA catheter, you're basically going to most likely go from one vena cava to the other one and not get anything in terms of a reading.
00:17:10
Speaker
At that point, if you're looking for a mixed venous oxygen saturation, the best you're going to do is land an IJ right down around the level of the Fontaine, which is basically where the pulmonary arteries connect to the shunt that, I mean, usually it's a shunt that is connecting the SVC and IVC together.
00:17:29
Speaker
And at that level, you'll get blood that is roughly mixed venous, but it's not 100%.
00:17:34
Speaker
And likewise, the pressures that you're getting there, which we would normally call a mixed venous, I'm sorry, a central venous pressure, that's actually a mean pulmonary pressure.
00:17:47
Speaker
because it's running right into the pulmonary arteries.
00:17:49
Speaker
So I think, you know, understanding that you have these, this very different anatomy, you have to realize that the monitoring is different for that reason.
00:18:00
Speaker
And you have to use very different strategies.
00:18:02
Speaker
From the perspective of
00:18:05
Speaker
Things like mechanical ventilation that you mentioned in the case of single ventricles, that's probably the most extreme case where you're dealing with now no right ventricle pumping blood through the lungs.
00:18:15
Speaker
So the blood flow through the lungs is completely passive.
00:18:18
Speaker
The central venous pressure essentially now has to drive or I should say the mean systemic pressure has to drive pressure that goes from
00:18:27
Speaker
pre-palmonary artery to post-palmonary capillary.
00:18:30
Speaker
And that pressure then has to end in a common atrium that's gonna be pumped by the systemic ventricle.
00:18:37
Speaker
That passive blood flow that's happening through the lungs means that anything that takes up intrathoracic pressure or alters pulmonary vascular resistance is gonna have huge consequences on blood flow.
00:18:47
Speaker
So just intubating and going onto positive pressure ventilation may actually drop your cardiac output dramatically because of the fact that you have risen
00:18:57
Speaker
the intrathoracic pressure and impeded the passive blood flow that's going through the lungs.
00:19:01
Speaker
So you have consequences on the physiology that are not at all straightforward and are not your straightforward heart-lung interactions that you expect when you ventilate somebody or put them on positive pressure with structurally normal biventricular heart.
00:19:17
Speaker
Perfect.
00:19:18
Speaker
And we can talk more about multidisciplinary approaches to these patients, but specifically for this population of congenital heart disease, you did mention there's 500-plus cardiologists who have expertise in adult congenital heart disease.
00:19:34
Speaker
Can you just talk about the importance of collaborating with them when these patients are in the ICU?
00:19:38
Speaker
Yeah.
00:19:39
Speaker
Yeah, I can't underestimate the importance of collaboration when it comes to dealing with with adult congenital heart disease and just generally speaking, congenital processes.
00:19:51
Speaker
You know, the the truth is, is that every one of these patients
00:19:56
Speaker
even if they fall into a specific group.
00:19:58
Speaker
So let's I've talked already about Fontans.
00:20:00
Speaker
Let's talk about Fontan physiology.
00:20:02
Speaker
A Fontan can be a patient who has a shunt running through their atrium or involving their atrium or external to their atrium.
00:20:10
Speaker
They can have a ventricle that's primarily right ventricle or it can be left ventricle or it can actually be a mix of right and left ventricle.
00:20:17
Speaker
They can have pulmonary veins that are all running back into their common atria or perhaps going to opposite atria or perhaps going into a confluence that drains into one of the atria.
00:20:28
Speaker
And so right there, I've already laid out 15 different permutations of what would all be called a fontan.
00:20:37
Speaker
And so you have to realize that they're all different.
00:20:40
Speaker
They're all unique.
00:20:41
Speaker
It makes it very hard to have a protocol, a pathway, and to just have a single approach to the patient.
00:20:48
Speaker
And so this is really one of those cases, I think more than almost anything else, where a multidisciplinary discussion and a thoughtful kind of planning before you start therapeutic interventions is called for.
00:21:01
Speaker
Because if you just launch into it thinking, oh, I've done a couple of Fanteans before, I can take care of this.
00:21:06
Speaker
you're in for some trouble because this Fontan may be very different than the last one you took care of.
00:21:13
Speaker
And I'll tell you that, you know, as somebody who's probably now cared for as many adults with congenital heart disease in an ICU setting as anyone in the world, I don't consider myself an expert.
00:21:23
Speaker
I think it's crucial to sit down with the cardiologists and with the surgeons and talk about every patient because of the heterogeneity and all the impacts that it's going to have on physiology and on their ultimate outcome.
00:21:37
Speaker
Excellent.
00:21:38
Speaker
Let's move to a different category, and maybe we can talk a little bit about genetic diseases and syndromes.
Genetic Diseases and Syndromes in Adult ICUs
00:21:43
Speaker
And obviously, this is, I'm sure, a very big group of patients.
00:21:48
Speaker
But if you can give us maybe some examples of diseases that might present in the adult ICU, and then maybe we can go into some of the basic ICU considerations for this group.
00:21:59
Speaker
Yeah, great.
00:22:01
Speaker
So I think, you know, when we talk about genetic diseases and syndromes, we really are talking about a broad set of things.
00:22:06
Speaker
But let me try to break it down into a few groups.
00:22:08
Speaker
I think, first of all, you've got the known chromosomal abnormalities.
00:22:12
Speaker
So things like trisomy 21 being by far the most common.
00:22:15
Speaker
That's Down syndrome, as everyone knows it.
00:22:17
Speaker
But there's other trisomies, 18 and, you know,
00:22:23
Speaker
Not all of these trisomies make it into adulthood, but again, increasingly, we're getting successful at surviving them, right?
00:22:29
Speaker
So a trisomy, as one would assume with having an extra chromosome, it doesn't just give you one genetic defect or one thing wrong.
00:22:38
Speaker
Ultimately, there's multiple things that are different than kind of normal.
00:22:42
Speaker
So taking trisomy 21, because it's by far the most common, if you're caring for a Down syndrome patient, you're going to have to worry about atlantoaxial instability.
00:22:50
Speaker
You're going to worry about their thyroid.
00:22:52
Speaker
There's a very good chance they have congenital heart disease.
00:22:55
Speaker
They're going to have early dementia, such that a 50-year-old is like an 80-year-old from a brain perspective.
00:23:01
Speaker
They're going to have some level of immunocompromise where they're much more susceptible to have pneumonia and certain other infectious illnesses.
00:23:08
Speaker
So all of those things,
00:23:10
Speaker
are the expectation of down syndrome and down syndrome is really common actually um and so i think just being prepared for how these patients are different and what the unique issues are is going to be critical moving aside from kind of chromosomal abnormalities there's a bunch of syndromes that are ultimately due to genetic defects but we're only starting to understand what those
00:23:32
Speaker
genetic defects are.
00:23:33
Speaker
And many of them are not a single gene defect, but there might be, you know, small chromosomal deletions or translocations or things like that.
00:23:42
Speaker
And those syndromes, again, very much like the trisomies, have multiple facets, often involving the brain, often involving the heart, maybe involving skeletal features and things like that.
00:23:55
Speaker
One of the things that I think gets underestimated, for example, is if you have severe scoliosis, which is not uncommon with the syndrome in kids, you have a form of restrictive lung disease where essentially one lung is squashed by your scoliosis and there's only one lung that's effectively ventilating and doing all the oxygenation.
00:24:12
Speaker
So if you get pneumonia in that one lung, you got a problem, a much bigger problem.
00:24:15
Speaker
problem than you would in most normal people.
00:24:18
Speaker
There's also limits to what's reasonable in terms of breathing that you can expect and what their FEV1 is going to be and their FRC and things like that.
00:24:27
Speaker
You know, when we then go to other a little bit more straightforward genetic diseases, things like Duchenne's muscular dystrophy or Becker's muscular dystrophy, these are patients who are going to have lots of problems in the case of the Duchenne's population,
00:24:40
Speaker
with progressive muscle weakness leading to respiratory failure and then progressive cardiomyopathy leading to heart failure.
00:24:47
Speaker
And although most of them die from the respiratory, there's a good chunk of them that die from their heart.
00:24:52
Speaker
And so you really have to have an understanding of what those processes are and also a lot of heart-to-hearts with the patient about where their values are and what they want for goals of care.
00:25:02
Speaker
Then there's others such as Marfan syndromes or Ehlers-Danlos, which are collagen vascular diseases, where we're dealing with things like
00:25:09
Speaker
repeated coarctations, I'm sorry, not coarctations, aneurysms of the aorta and problems with their particular aortic valve, which then leads to multiple reoperations.
00:25:21
Speaker
So all of these have various comorbidities.
00:25:26
Speaker
I think it gets more complex when you're dealing with things on a chromosomal level than a single gene defect.
00:25:32
Speaker
But you have to understand what the natural history of disease is for these different genetic syndromes.
00:25:37
Speaker
and then be able to deal with the various comorbidities that come with it.
00:25:42
Speaker
So obviously, a lot of diseases, like you said, with specific issues, but it sounds like in terms of ICU considerations, again, going back to airway challenges with these patients that we should be aware of, right, not just walk into these intubations kind of with a laissez-faire kind of attitude and respiratory issues in terms of how we support them and complications they could have.
00:26:05
Speaker
And then, like you mentioned, cardiovascular complications, which are much more common in this population than maybe some people are
00:26:12
Speaker
think of.
00:26:14
Speaker
Yeah, and I think you said it really well there, Sergio, because it's don't have the laissez-faire attitude.
00:26:21
Speaker
And this is hard, right?
00:26:22
Speaker
Because I think as intensivists, once you've got a lot of experience under your belt, you have a way you handle things, right?
00:26:29
Speaker
A way you handle shock and respiratory failure.
00:26:31
Speaker
And, you know, generally speaking, the approaches that you've learned over years and over, you know, dozens and hundreds of patients are
00:26:40
Speaker
100% right.
00:26:41
Speaker
You know, I mean, those work and they're efficient and effective and, you know, get things under control.
00:26:46
Speaker
But if you try to apply those same principles to somebody who has a complex genetic disease or adults congenital heart disease, you'll quickly find out that their unique comorbidities, the fact that their C-spine is not stable or they have macroglossia or microanathia or, you know, a variety of other illnesses means you get into airway issues.
00:27:05
Speaker
And before you know it, you have a coat on your hands.
00:27:07
Speaker
And that's, you know, that's the situation nobody wants to be in.
00:27:10
Speaker
So, you know, the biggest thing I would say is these patients require forethought a lot more than most of our ICU patients.
00:27:17
Speaker
And I'm not saying that we don't we don't think I think as intensivists, we think an awful lot in caring for our patients, but I don't think we pre plan on most.
00:27:25
Speaker
of them because the nature of ICU care is that a patient gets sent to us kind of in extremis and we very quickly deal with it, right?
00:27:32
Speaker
And this is one of those situations where short of the things that are life-threatening, I think what we need to do is take a minute and kind of think and plan before acting, which I don't know is always, you know, the usual routine in an ICU setting.
00:27:47
Speaker
Excellent.
00:27:48
Speaker
And the last category that we mentioned that I want to hear a little bit more about, Cameron, is prematurity-related conditions in adults.
Long-term Effects of Prematurity in Adults
00:27:56
Speaker
Now, I would imagine that every five years, I don't know, every five years, 10 years, whatever, the...
00:28:03
Speaker
The number of weeks that are survivable preterm keeps increasing, right?
00:28:07
Speaker
Seems that way, right?
00:28:08
Speaker
And we are seeing things that maybe, I mean, 20 years ago were not something that we would imagine possible.
00:28:16
Speaker
But like you said, these premature newborns are surviving longer and longer, and there might be some consequences.
00:28:23
Speaker
So how do you think that's going to be a little bit?
00:28:25
Speaker
Tell us a little bit about, I mean, this is an area that I really have no, no, no experience with.
00:28:29
Speaker
And I'm just curious in terms of where we are today and how you think about it as a pediatric and adult critical care specialist.
00:28:36
Speaker
Yeah, well, I mean, I think what you said is 100% accurate.
00:28:39
Speaker
I mean, when I was going through residency, you know, we were pushing the limits talking about 25 weekers and, you know, roughly under a kilo and a half was considered to be really low birth weight, right?
00:28:51
Speaker
Now, there, I think, you know, the current discussions and I'm not a neonatologist, so I might be wrong on this, but I think the current discussions are about 23 week viability and 500 grams as very low birth weight.
00:29:04
Speaker
So we're really, we've pushed the extremes of what is survivable.
00:29:08
Speaker
And unfortunately, when a child is born, you know, weighing a pound and, you know, being 23 weeks of gestation, so about half of what's normal, there's a whole lot of complications that often occur within those first couple months of life that
00:29:25
Speaker
go on.
00:29:26
Speaker
I think breaking it into some of the common characteristics, chronic lung disease owing to prematurity and lack of surfactant, which then manifests itself as pretty significant pulmonary hypertension as they become older.
00:29:40
Speaker
The issues that will happen with immature brain circulation, in particular with intraventricular hemorrhage and germinal matrix hemorrhages, which then can leave them neurologically quite devastated or having things like cerebral palsy or other kind of chronic diseases.
00:29:58
Speaker
motor and sometimes cognitive.
00:30:00
Speaker
They don't always go together, dysfunctions moving into life.
00:30:04
Speaker
And, you know, I think those types of illnesses, because of their severity, will then lead to things like being chronically trached, vented, G-tube fed, problems with swallowing.
00:30:18
Speaker
And not all of these problems are always because they're incapable of swallow, but children will actually develop significant symptoms
00:30:27
Speaker
dysphagias that are as much behavioral and because of the complications they had as a child as they are actually physical and, you know, based on muscle and nerve.
00:30:38
Speaker
And so then dealing with those adults who have the lingering consequences of that from their childhood is challenging because, again, it's a very difficult
00:30:51
Speaker
And I think for us to deal with a 20 year old in the ICU who has severe pulmonary hypertension from lung disease and potentially is neurologically normal in the terms of cognition, but has spastic quadriplegia as a result of some of their intraventricular hemorrhages when they're a child.
00:31:11
Speaker
But that's the type of comorbidities that are not uncommon coming out of extreme prematurity.
00:31:18
Speaker
Excellent.
00:31:18
Speaker
And we discussed, obviously, I mean, different categories of disease and some specific considerations.
00:31:24
Speaker
Ultimately, I think the real goal of our conversation today, Cameron, is to bring awareness to the critical care adult community, right, of this growing number of patients and particular differences that their management might require.
00:31:40
Speaker
And we can't cover everything.
00:31:42
Speaker
Like you said, I mean, even yourself who sees this on a regular basis feels that obviously the expertise is hard to grasp.
00:31:49
Speaker
But what about what I would like to do now is maybe talk more general of ICU management and maybe of congenital disease in the adult ICU and talk about pitfalls to avoid and maybe some pearls of management that you can share with us.
00:32:02
Speaker
And going over more of like a systems approach of different areas and start maybe with physiology and recognizing the unique physiology and what is normal for these patients, right?
00:32:15
Speaker
Right.
00:32:15
Speaker
Yeah,
Managing Unique Physiology of Congenital Patients
00:32:16
Speaker
absolutely.
00:32:16
Speaker
So, so, um, gosh, very broad, right?
00:32:20
Speaker
So, uh, let, but let's, let's start with kind of in adult congenital heart disease with, um, some of our single ventricles, um, and what is unique about them.
00:32:30
Speaker
So I touched upon the fact that in single ventricle physiology, you, you,
00:32:35
Speaker
have some very unique heart-lung interactions because you have passive blood flow through the lungs.
00:32:39
Speaker
In addition to that, the actual mechanics of the blood flow are quite different in that the IVC and the SVC, which ultimately connect to the pulmonary arteries,
00:32:50
Speaker
the IVC often doing so through some kind of grafting or shunt, they don't always feed the pulmonary arteries to the same degree.
00:32:59
Speaker
So it's not like the SVC blood flow and the IVC blood flow end up 50-50 to the left and right PA.
00:33:06
Speaker
Often there are asymmetries, and those asymmetries may be as much related to the directionality and the positioning of the attachments between the SVC and IVC with the pulmonary arteries as they are by
00:33:19
Speaker
potential stenosis within the pulmonary arteries, both proximally and distally.
00:33:24
Speaker
And, uh, and so you have, um, individuals who actually have differential pulmonary blood flow for their entire life where, uh, most of their, um, um,
00:33:35
Speaker
Pulmonary blood flow goes more to the right or to the left.
00:33:38
Speaker
And so then obviously having a pulmonary embolism, depending on whether you get it on the majority side or the minority side, has a very different impact on them.
00:33:46
Speaker
But also what that means is for the diagnosis of pulmonary embolism, you got to be really careful because if you do what we usually do, which is to give an IV contrast bolus into an arm IV and let it circulate through and shoot your CT scan to try to diagnose a PE, what's going to happen in most cases with these Fontans is
00:34:04
Speaker
is that that SVC, which is going to accept that contrast load, is going to connect to one PA more than the other.
00:34:10
Speaker
So you're going to end up shunting most of your contrast load through your, say, right PA and not through the left PA.
00:34:17
Speaker
And that's going to get read as a massive left PE.
00:34:19
Speaker
And I've had probably a dozen referrals for massive PE, and actually none of them have been pulmonary embolism.
00:34:26
Speaker
They've all been an artifact of the contrast bolus in reading.
00:34:30
Speaker
And it's not surprising because it's not something that the radiologist expects, right?
00:34:35
Speaker
But in that situation, you either need to do a delayed phase study where you give time for an entire circulation of the contrast load to go through the heart and then get pumped out and come back onto both IVC and S.
00:34:50
Speaker
or you need to put IVs both in the upper and lower extremity and be able to time your contrast bolus on both of them, which is not something that a non-congenital center is going to have as a protocol.
00:35:00
Speaker
So it really doesn't happen in most of the world.
00:35:05
Speaker
So that's just one example.
00:35:08
Speaker
The, you know, I think that once you get past that, there's the issue of in congenital heart disease, what is the normal sat?
00:35:17
Speaker
So many of the diseases in congenital heart disease either have residual shunts or the formation of abnormal collaterals.
00:35:23
Speaker
So take the Fontan group.
00:35:25
Speaker
There's a lot of aural pulmonary collaterals, and there's also veno-venous collaterals, where it's essentially a systemic vein, often from the lower extremity that's experiencing high
00:35:35
Speaker
venous pressures that then anomalously forms a collateral to a pulmonary vein.
00:35:40
Speaker
So that's supposed to be an oxygen-rich vein because it's post-pulmonary capillary.
00:35:44
Speaker
So what that equates to is oxygen-poor blood getting shunted into the oxygen-rich blood.
00:35:49
Speaker
And so these patients...
00:35:51
Speaker
normal saturation declines often with age.
00:35:56
Speaker
And so what you'll have is a Fontaine who's 30 years old, who on their best day is going to have a SATA 82.
00:36:01
Speaker
And the patient can often tell you what their baseline is.
00:36:05
Speaker
But if you don't ask and you just look at their SAT, you're going to think they're hypoxic when actually they're at their baseline, which is their day-to-day for the last five years.
00:36:14
Speaker
And so it's really important to be aware of what that baseline is and what's driving it, because things like intubating them and giving them 100% oxygen not only won't help them, but by virtue of compromising the problem of blood flow is likely to hurt them and put them in a worse space.
00:36:29
Speaker
So it's really important to understand the norms and the physiology.
00:36:34
Speaker
And this is something, I mean, we could go on for a long time about this, but hopefully that just that one description gives folks who are listening an idea of some of the
00:36:44
Speaker
and some of the features they need to think about.
00:36:48
Speaker
You know, I want to get back to something you said right at the beginning of this section, which is, you know, it's important that we're talking about it.
00:36:57
Speaker
And I think the only way that we as a field are going to be able to handle the influx of these patients and really give them great care is that we continue to talk about it.
00:37:06
Speaker
And those conversations really involve multidisciplinary conversations between not only patients
00:37:14
Speaker
different adult specialists, but it's actually reaching out between adults and peds.
00:37:19
Speaker
And that can be really hard because as you know, there's a whole lot more adult ICUs and adult medical centers than there are pediatric ones.
00:37:28
Speaker
So I don't know the statistics.
00:37:29
Speaker
You probably know this.
00:37:31
Speaker
The number of adult ICUs, how many of them are in a med center that also has a pediatric ICU?
00:37:37
Speaker
I would guess it's somewhere on the order of 10%, 15%, which means that most adult practitioners don't actually have a pediatric intensivist in their house that they can turn to and say, hey, give me some advice about this process.
00:37:50
Speaker
And that right there is actually a big problem.
00:37:52
Speaker
I agree.
00:37:53
Speaker
And I think that what a lot of pediatric beds get, that get counted are really neonatal beds, which is a different story again, right?
00:38:01
Speaker
Yes, very much so.
00:38:03
Speaker
Yeah.
00:38:03
Speaker
And the neonatologists don't have experience with, outside of prematurity, don't have experience with a lot of this.
00:38:08
Speaker
I agree.
00:38:09
Speaker
Yeah.
00:38:09
Speaker
And so what about the one thing I wanted to ask you here before we move on to airway and ventilation considerations is we like to give a lot of volume in the ICU and that pendulum keeps swinging back and forth with sepsis and we're learning.
00:38:24
Speaker
And despite being one of the most common things we do in the ICU, we still haven't figured it out.
00:38:28
Speaker
But in particular, I think we have to be very careful with some of these patients, right?
00:38:32
Speaker
You do.
00:38:34
Speaker
You know, I think the amount of right heart failure that I deal with in congenitals, adult congenitals, is just so much more common than what I'm dealing with in structurally normal hearts.
00:38:45
Speaker
And it's driven by things that include chronic shunts that for the most part are going left to right.
00:38:52
Speaker
So what you're doing there is you're essentially overloading the right because a portion of the left heart blood is being pumped back into the right.
00:38:59
Speaker
Okay, so if you have an ASD or a VSD, that's essentially what happens, or anomalous pulmonary venous return that dumps into the right side.
00:39:08
Speaker
You're basically taking a portion of what should be blood pumped on the left side and bringing it back to the right.
00:39:14
Speaker
And so the result of that is the right side is volume overloaded.
00:39:17
Speaker
Now, the right heart, as we know, is a more compliant ventricle and it's able to handle higher degrees of volume load and, you know, can continue to pump that without getting into trouble.
00:39:28
Speaker
But there's a limit to that.
00:39:31
Speaker
And if you're chronically overloaded and then all of a sudden you bolus a bunch of fluid, the chances of setting that patient at the right heart failure are substantial.
00:39:39
Speaker
And this is particularly common in some of the Fontans where they don't even have an RV.
00:39:45
Speaker
You're dealing with passive flow and they always have high CVPs and very high EDPs.
00:39:52
Speaker
within their systemic ventricle.
00:39:54
Speaker
This is also common in things such as Eisenmenger's syndrome for a variety of causes and with Epstein's anomaly, because in Epstein's, you've already got an abnormal RV, which often is failing, and a very leaky tricuspid valve, which results in overload of that RV.
00:40:12
Speaker
So in these cases, you quickly get to RV failure if you give too much fluid.
00:40:16
Speaker
That'll be manifested by what looks like liver failure because they already have congested livers and now you fill them even more and quickly the AST-ALT will get into the many hundreds and almost look like an infarct.
00:40:28
Speaker
but it's not.
00:40:29
Speaker
It's just that you've congested the liver.
00:40:31
Speaker
And then the very unique thing about congenitals that you don't see very often in other processes is they can have lymphatic abnormalities.
00:40:39
Speaker
And so these lymphatic abnormalities are really...
00:40:45
Speaker
abnormal lymphatic return into the venous circulation.
00:40:50
Speaker
And normally the thoracic duct dumps into the asagus.
00:40:53
Speaker
And so if you have venous hypertension, you impede lymphatic return as it is.
00:40:58
Speaker
But now if you have abnormal lymphatic connections that may be empty into the pleural space or into the gut, which is what you see over the bronchioles, which is what you see with congenital heart disease,
00:41:08
Speaker
Now, all of a sudden, the lymph starts pouring into their pleural space and they get chylos effusions or dumps into their lungs and they develop plastic bronchitis or dumps into their GI tract and they've got protein losing neuropathy.
00:41:19
Speaker
So these are problems that you rarely deal with when you're dealing with non-congenital patients.
00:41:26
Speaker
And these are exacerbated by being an RV failure or having high venous pressures.
00:41:31
Speaker
Like you said, I mean, a lot of things to consider that can be very nasty surprises if we don't pre-plan.
00:41:39
Speaker
Now, as we move to the next topic, airway and ventilation considerations, I'll share with you very briefly a nightmare experience I had some years ago.
Challenging Airway Management Case Study
00:41:49
Speaker
I was doing a night call at a large community hospital here in Houston, and there was a 20-something-year-old, late 20s, who got transferred from an outside hospital with what was labeled a STEMI.
00:42:02
Speaker
So it was taken directly to the cath lab, and then they call a code, and they call me emergently to the cath lab because I needed to intubate this patient.
00:42:12
Speaker
When I get there, they tell me he has some congenital issue.
00:42:16
Speaker
That's all he said, the cardiologist.
00:42:17
Speaker
Yeah, right.
00:42:20
Speaker
It was a horrible experience, bad outcome, and the patient had Pierre Robin sequence.
00:42:26
Speaker
And I was trying to intubate this poor patient in a cath lab without support.
00:42:33
Speaker
Anesthesia came.
00:42:35
Speaker
They couldn't do it.
00:42:36
Speaker
It was a disaster.
00:42:37
Speaker
Yeah, I'm sorry about the difficult airway management and how I mean, we I mean, I think our anesthesia colleagues are always very cautious because they, I think, see a lot more airways than we do.
00:42:49
Speaker
And we always get called in emergencies.
00:42:51
Speaker
And I think.
00:42:52
Speaker
And the nature of practice is that by the time you get too cocky, you get humbled, right?
00:42:58
Speaker
So you get, you get, you get, you bring down a couple of notches, but I think it's always important to understand in particular with this population, you can be in for a lot of surprises.
00:43:09
Speaker
Yeah, no, I think you're right.
00:43:11
Speaker
I mean, just hearing your experience, like, you know,
00:43:14
Speaker
gives me chills because I completely understand, you know, I mean, I've dealt with this before and it's, you know, I've dealt with it knowing what all the issues are.
00:43:21
Speaker
So I can only imagine being surprised by it.
00:43:23
Speaker
Right.
00:43:24
Speaker
So Pierre Robin syndrome, which your sequence that you're talking about is a combination of macroclossia, microdaphymia.
00:43:31
Speaker
And so what you're dealing with is a very small
00:43:34
Speaker
jaw and a very oversized tongue.
00:43:37
Speaker
And so as a result, they effectively have complete obstruction of their mouth so that you can't really ventilate them through the mouth.
00:43:43
Speaker
And so now you've got the nose, but often in these situations, you also get coenal atresia, which means that their nasal passages are narrowed or perhaps even completely stenotic.
00:43:54
Speaker
So now you don't have an airway.
00:43:56
Speaker
when you're dealing with somebody who is not conscious and able to keep their mouth open and breathe.
00:44:01
Speaker
You know, you may not recognize it at all, but for a lot of patients who have Pierroban syndrome, when you look at them, at their face, you can tell that
00:44:12
Speaker
something looks a little funny, right?
00:44:13
Speaker
And it's a little hard to necessarily put words on it, but what it is is that their jaw, their bottom, you know, their chin is just too small for the rest of their face.
00:44:23
Speaker
And that's the sign of microanathia, which then, you know, you have to look in their mouth to see they have macroglossia, but that's essentially what brings together the sequence.
00:44:33
Speaker
So these patients are going to need an oral airway introduced very quickly after you induce anesthesia, and still they may be very difficult to bag.
00:44:45
Speaker
And if you can't bag them effectively and because of the kind of short distance to...
00:44:50
Speaker
their larynx, getting a view of their airway may be impossible by standard techniques.
00:44:59
Speaker
And so you may need something like bronchoscopic guidance to be able to intubate.
00:45:04
Speaker
And sometimes you have to move to trach emergently.
00:45:06
Speaker
So it's a lot of challenges.
00:45:10
Speaker
And Pierre Robbin is probably the most extreme, but there are variants of that in all of the other, excuse me,
00:45:18
Speaker
in all of the other genetic diseases that I got to.
00:45:21
Speaker
So for example, Down syndrome kids have huge tongues.
00:45:24
Speaker
I think everybody who's seen a Down syndrome kiddo is used to kind of the tongue sticking out of their mouth.
00:45:29
Speaker
It's not that they love sticking their tongues out of the mouth.
00:45:31
Speaker
It's that their tongues are oversized relative to their oropharynx.
00:45:37
Speaker
And so that's a form of obstruction, especially when you're dealing with somebody who's sick or not fully conscious and not able to regulate their muscle tone the way they normally would.
00:45:46
Speaker
And they're
00:45:47
Speaker
and their ability to swallow.
00:45:48
Speaker
And so kind of preparing for that obstruction, having available adjuncts like oral airways, nasal airways, bronchoscopic guidance, those are all going to be things that are really crucial, as well as quickly calling for support from anesthesia and sometimes from ENT.
00:46:06
Speaker
Because like I said, in some cases, it may be an airway that's just not obtainable and you're going to trach.
00:46:12
Speaker
The other thing I wanted to ask you about in this kind of category is we use a lot of non-invasive ventilation more and more, I think, especially post-COVID and with all the literature coming out.
00:46:25
Speaker
Any particular concerns with particular subgroups of patients with the use of non-invasive ventilation?
00:46:33
Speaker
Yeah, I think they're the only one that, you know, I think is as a class, a consideration is that, again, when you're dealing with Fontans, non-invasive ventilation is in effect going from negative to positive pressure ventilation, although the impact on intrathoracic pressure is not nearly that of intubating and putting them on a mechanical ventilation.
00:46:55
Speaker
But nonetheless, that positive pressure really can result in issues with impeding polypathy.
00:47:02
Speaker
blood flow.
00:47:03
Speaker
And likewise, if that even if they have biventricular physiology, but they have Eisenmangers, so they have essentially systemic RV pressures, putting them on positive pressure may be that little bit of extra that tilts them into not having any flow from their right side to their left and having a cardiac arrest.
00:47:23
Speaker
And so I think you have to be really thoughtful when you go on positive pressure for those reasons.
00:47:32
Speaker
I'd like to talk a little bit about hemodynamic monitoring and support.
00:47:35
Speaker
We did mention some aspects of this in the adult congenital discussion, but just a couple more things to talk about, Cameron.
00:47:45
Speaker
Blood pressure measurements, can you talk a little bit about that?
00:47:49
Speaker
Yeah, I mean, first of all, when you're dealing with the adult congenital population, you have to recognize that some of the surgeries that they have potentially have affected things like their subclavian vessels, because we used to use those for things like blood lactose chunks.
00:48:06
Speaker
And so if you put a radio art line in on a side where the subclavian is compromised, you're obviously not going to get an accurate reflection of aortic blood pressure.
00:48:15
Speaker
You also can have issues with coarctation, particularly at the level of the ductus arteriosus, because that's where most coarcts form, but it can happen anywhere else through their blood.
00:48:25
Speaker
circulation.
00:48:26
Speaker
When you're dealing with some of the mutations like Marfan's, Ehlers-Danlos, actitude mutations, things like that, the propensity for a dissection can make it so that, again, putting in an arterial line on a
00:48:43
Speaker
radial versus ephemeral can give you a very different number because you've got essentially a stenosis or a dissection and you're measuring blood pressure through a false lumen.
00:48:53
Speaker
And so all of those are things that impact inflammation.
00:48:58
Speaker
how reflective your numbers are.
00:49:00
Speaker
And so sometimes we're forced to place multiple arterial lines because we care about, I mean, in the end, the arterial line down below often will reflect perfusion to things like the gut and the kidneys, whereas the arterial line up top
00:49:14
Speaker
is gonna and when I say up top I mean like right radial is gonna reflect brain and coronary perfusion and both are important right and so there there are definitely patients where we have two a lines in because we need to be monitoring perfusion pressures on both sides the other thing are the SATs related to that right so when you have a
00:49:33
Speaker
for example, a ductus arteriosus that's patent, that will happen roughly at the level of the left subplavian.
00:49:41
Speaker
And so if you're measuring the sat on the right finger and you compare that to something in either of the legs, either toe, you may see very different saturations because you have shunting of blood.
00:49:55
Speaker
And, you know, this would have to be a patient who has progressed to Eisenmangers.
00:49:59
Speaker
But if they're having right to left shunting and essentially oxygen poor blood is shunting across the ductus into systemic circulation, then in that situation, the right finger sat is going to be considerably higher than the left toe sat.
00:50:13
Speaker
And so it's just being aware of the fact that they are different.
00:50:16
Speaker
And again, they reflect the oxygen delivery to different parts of the body.
00:50:20
Speaker
So it just depends how concerned you are about that region of the body.
00:50:24
Speaker
Your monitoring should be appropriate to reflect that.
00:50:28
Speaker
Excellent.
Role of Echocardiography in ICUs
00:50:29
Speaker
And we talked about hemodynamic monitoring.
00:50:31
Speaker
You gave examples of the difficulties of placing PA catheters in some pathologies.
00:50:37
Speaker
Can you talk about the importance of echocardiography?
00:50:41
Speaker
Yeah, I mean, I think, you know, echo, point of care ultrasound in general is huge, right, and so helpful.
00:50:48
Speaker
But when you're dealing with critical care echocardiography and congenitals, it gets confusing really quickly.
00:50:54
Speaker
You know, the
00:50:57
Speaker
The most extreme example are going to be your patients who have things like dextrocardia and transition where literally everything's flipped.
00:51:04
Speaker
And, you know, if you're trying to get an apical four chamber on the left, you're going to quickly realize there's only lung there, you know, and so...
00:51:12
Speaker
What the windows are and being able to interpret, you know, what you're looking at in terms of the ventricular pictures really takes some expertise.
00:51:22
Speaker
And I think this is where partnering with an adult congenital cardiologist becomes really critical because they're going to be able to guide you through the echo and tell you what everything looks like.
00:51:33
Speaker
And so it does hamper the ability to get point of care echo, where I think a lot of us working in cardiac setting are doing that routinely on structurally normal hearts because we kind of have a few views where we have imaged so many times that we're comfortable making.
00:51:48
Speaker
making diagnoses like effusions or what the function is.
00:51:53
Speaker
But, you know, diagnosing the function on somebody whose ventricular function ultimately is kind of a combination of the RV and the LV and their transpose is not straightforward at all.
00:52:06
Speaker
Excellent.
00:52:07
Speaker
What are some other anatomic differences that might impact care in general ICU care for this population?
00:52:14
Speaker
Yeah, I mean, I alluded to a few of them.
00:52:16
Speaker
I mean, a lot of these genetic or syndromic children have severe sclerosis.
00:52:22
Speaker
And with that, restrictive lung disease, there's a lot of contractures in the extremities from things like cerebral palsy.
00:52:30
Speaker
And so that makes it very challenging to get, you know, it.
00:52:35
Speaker
atrial, sorry, arterial lines in certain places and PICC lines, things that we use very commonly.
00:52:41
Speaker
And then the lymphatic anomalies that we talked about, particularly seen in the single ventricle population, but really within, throughout congenital heart disease, there's a higher incidence of lymphatic anomalies.
00:52:53
Speaker
And that can result in things like plastic bronchitis and protein losing enteropathy, which are pretty rare to see in structurally normal hearts.
00:53:01
Speaker
And we talked about extensively, I think, about the important multidisciplinary collaboration and consulting specialists early.
00:53:09
Speaker
I mean, obviously, if you don't have access to adult congenital heart disease experts, getting your genetic colleagues involved, getting your pediatric colleagues involved.
00:53:19
Speaker
And when it's complicated, probably trying to refer patients to the right center where they have that expertise, I think, is very important for us in terms of trying to get the best outcomes from these patients.
Goals of Care Discussions with Families
00:53:31
Speaker
But I think that this is a perfect segue into the last portion of our discussion, which is navigating goals of care to conversations.
00:53:39
Speaker
And this is something that we talked a little bit about before we were recording offline.
00:53:44
Speaker
And I know that a lot of times when adults see some of these patients, there's an immediate nihilist reaction, like, what are we doing here?
00:53:54
Speaker
But that might be very different from the perspective of a pediatric patient and their family who has grown for decades with basically chronic critical illness.
00:54:06
Speaker
Can you talk a little bit about how you approach goals of conversations and maybe give us some pearls on how to do this a little bit better with this patient population?
00:54:16
Speaker
Yeah, and I'm so glad we're talking about this.
00:54:19
Speaker
I mean, just like you said, for parents who have raised children who have chronic illness, so let's take the example of a child who, for example, has a trisomy or some kind of other syndrome.
00:54:31
Speaker
And let's say they're chronically trached, vented, G-tube fed, spastic quadriplegia,
00:54:38
Speaker
may be limited in terms of verbalization.
00:54:41
Speaker
They may be completely non-vocal, right?
00:54:44
Speaker
Over the course of 20 years, which is typically, you know, how old the patient is by the time they show up in adult care, that parent has learned what is happy, sad, uncomfortable, you know, and they've found an interaction with their child that there's no way you're going to pick up on that in two minutes or even an hour.
00:55:06
Speaker
And so,
00:55:08
Speaker
you really need that parent to tell you what is quality of life, what is kind of a good or bad day, happy or sad, in pain or not in pain.
00:55:21
Speaker
And if you break that therapeutic alliance between yourself and the parent, you're really going to be treating that patient out of complete ignorance and not getting good outcomes as a result of it.
00:55:32
Speaker
And
00:55:33
Speaker
One of the biggest things that I see break that therapeutic alliance is that an adult intensivist walks into this room, sees this contractured, trached, vented, you know,
00:55:45
Speaker
patient laying there and just says, what the heck are we doing?
00:55:47
Speaker
Let's just, just DNR, DNI and stop.
00:55:50
Speaker
Right.
00:55:51
Speaker
And, you know, like I get it.
00:55:54
Speaker
Um, I I've taken care of enough chronically ill patients to understand where that comes from.
00:55:59
Speaker
And perhaps if this was somebody who was 65 and demented and all that, um, you know, that would be a very realistic conversation to have about, you know, should we talk about hospice?
00:56:10
Speaker
But when you're talking about somebody who is 20, um,
00:56:13
Speaker
That's just not something that that family's ready to even contemplate.
00:56:18
Speaker
And the reality is, is as they've been going in and out of health care on the pediatric side, they probably have an admission into the ICU once every five, you know, six, seven years.
00:56:29
Speaker
And then they have many years at home of what they consider to be excellent quality.
00:56:33
Speaker
Right.
00:56:34
Speaker
And if you don't recognize that or know that about this patient, you're, you
00:56:39
Speaker
kind of quick two minute overlook and diagnosis that this is a terminal condition and we need to progress to hospice is going to poison any chance you have of getting through to this family and to this patient.
00:56:50
Speaker
And so I think it's just really important when you're dealing with somebody who has chronic illness like this to understand what their history has been, understand what their baseline is, define what is good quality of life and what are the expectations.
00:57:06
Speaker
And then once you understand all that, then you can start making decisions.
00:57:09
Speaker
I'm not saying that withdrawal of life-standing therapy doesn't make sense in some cases.
00:57:16
Speaker
It does.
00:57:17
Speaker
But what I'm saying is you can't just look at the patient and have an idea of what is normal and what is their baseline without having a very deep conversation with the person that's been with them forever.
00:57:29
Speaker
And again, because a lot of these patients are coming from a pediatric center, often the electronic medical records don't carry over.
00:57:35
Speaker
I mean, even at my own center, I'm at Baylor right now.
00:57:38
Speaker
Before this, I was at the University of Pittsburgh.
00:57:40
Speaker
It's crazy, but we had at Pittsburgh Cerner, and here we have Epic.
00:57:45
Speaker
But the peds and the adult hospitals use completely different builds.
00:57:49
Speaker
And so they don't talk to each other.
00:57:50
Speaker
So my login on the adult side didn't get me the records from the peds side and vice versa.
00:57:55
Speaker
Meaning that if you're the adult intensivist, you don't really have the opportunity to go through the medical records from two decades of pediatric history and understand what was the background.
00:58:05
Speaker
And so you need the family member to fill you in on that so that you can understand what the baseline is.
00:58:11
Speaker
And then go from there.
00:58:12
Speaker
And, you know, sometimes this also means talking to the pediatrician who took care of them for the last 20 years and maybe still is considered their primary care physician.
00:58:23
Speaker
And so having those discussions, I think, is is really important.
00:58:27
Speaker
One other thing I just want to throw in before we move on, Sergio, is you talked about the
00:58:34
Speaker
the concept of kind of transferring care if you don't have the multidisciplinary collaboration that you need.
00:58:41
Speaker
And I think that's something that we don't think about a lot.
00:58:45
Speaker
You know, generally speaking, when folks transfer care, it's often because of some capability, some technology that their center doesn't have, right?
00:58:54
Speaker
So, you know, I don't have ECMO.
00:58:56
Speaker
I'm going to send this patient to an ECMO place.
00:58:58
Speaker
I don't
00:58:59
Speaker
do transplants, heart transplants, lung transplants, whatever.
00:59:02
Speaker
I'm going to send them to a transplant center, right?
00:59:05
Speaker
Here in the case of some of these congenital cases, it really becomes, I don't have the expertise of individuals who deal with this chronic illness.
00:59:13
Speaker
Let me get them to a place that has that.
00:59:15
Speaker
And I think that's every bit as valid a reason to transfer a patient as not having a piece of equipment or a particular program like transplant.
00:59:23
Speaker
I agree.
00:59:24
Speaker
And I think it's about ownership, right, of really being responsible for providing our patients with the best possible outcomes and recognizing the limitations that we have.
00:59:34
Speaker
But however, I think this is an important conversation, Cameron, because I'm sure that there are some intensivists who don't have that luxury of transferring to a place that has the expertise, right?
00:59:44
Speaker
There are plenty of places where you just have to take care of these patients and doing it the best way you can is going to be very important.
00:59:50
Speaker
Now,
00:59:51
Speaker
Regarding the goals of care conversations, I think that it's also a very important lesson for all patients, right?
00:59:59
Speaker
Trying to provide patient goal concordant care is about curiosity, asking what's important for our patients, not telling them what we think should be important for them.
01:00:10
Speaker
Yeah, you're so right.
01:00:11
Speaker
I think a lot of times we all fall into that.
01:00:14
Speaker
We all assume that I would never want that, but we don't know what they would want.
01:00:17
Speaker
And we don't know what we would want if we were in their shoes.
01:00:20
Speaker
So asking those questions and understanding more about what a good day looks like for this patient, what has the last 15 years looked like is extremely important.
01:00:31
Speaker
The other part of this topic that I think is very important
01:00:35
Speaker
is being able to differentiate between reversible and irreversible decompensation.
01:00:41
Speaker
I think we might see something that maybe people with expertise have treated a hundred times and got better, and we think, oh my God, this is the end of it.
01:00:49
Speaker
Can you talk a little bit about that?
01:00:51
Speaker
Yeah, no, I think you're right.
01:00:55
Speaker
As I've been talking about all of this, I think if somebody who doesn't deal with
01:01:02
Speaker
might be kind of horrified like oh my gosh I gotta deal with this and this and this and so when you're when you're handling all of these essentially chronic overlays it can seem like a hopeless case right like a just someone who's so sick that they can't possibly get along and yet
01:01:18
Speaker
they can, right?
01:01:19
Speaker
In fact, they have been for, you know, many years.
01:01:22
Speaker
And so I do think that understanding, you know, recognizing what is the natural history of disease and, you know, the fact that there can be quality and longevity to patients who have actually quite a bit of disease burden.
01:01:39
Speaker
And it's surprising if
01:01:41
Speaker
if you just haven't dealt with that particular process.
01:01:43
Speaker
But fortunately, there are people often who have that expertise and kind of can help you.
01:01:49
Speaker
And like I said, sometimes that person is just the family member, right?
01:01:52
Speaker
The parent in most cases who has been dealing with that since their child was born.
01:01:57
Speaker
And so they know firsthand what's realistic and what's not because they've had so many conversations about it.
01:02:03
Speaker
And so I do think that that's really important to kind of establish and to not conclude that the chronic,
01:02:11
Speaker
illness equals acute or irreversible disease, which then condemns them to no quality of life.
01:02:19
Speaker
Excellent.
01:02:20
Speaker
I think we've had a wonderful discussion.
01:02:22
Speaker
And like you mentioned earlier, Cameron, this is a topic that could go on for hours and hours.
01:02:28
Speaker
So I think that this is a perfect place to stop the clinical discussion.
01:02:32
Speaker
We like to close the podcast with a couple of questions that are unrelated to the clinical topic.
01:02:36
Speaker
Would that be okay?
01:02:38
Speaker
Absolutely.
01:02:39
Speaker
So the first question is about books.
01:02:41
Speaker
And have there been a book or books that have influenced you significantly or a book that you have often gifted to other people?
01:02:49
Speaker
Yeah, so I mean, I thought about this and, you know, there's a lot of books I love.
01:02:53
Speaker
But I have to be honest with you, the book that I've read, the only book I've ever read eight now going on nine times is the Bible.
01:03:01
Speaker
The only book I have read
01:03:04
Speaker
given out to, you know, a hundred people or more is the Bible.
01:03:09
Speaker
So yeah, it's the book that's influencing me the most.
01:03:12
Speaker
I mean, it's, it's,
01:03:14
Speaker
book that I read every single day of my life.
01:03:17
Speaker
So definitely it.
01:03:18
Speaker
And I would say that without going deep into religious discussions, I have read many holy books from different religions.
01:03:29
Speaker
I mean, not the whole thing, but I've ventured in reading different things.
01:03:32
Speaker
And I think there's value and learning in all these traditions, right?
01:03:37
Speaker
And I agree.
01:03:38
Speaker
I mean, I think that at the end of the day, the idea of this portion is to get to know people a little bit more as individuals, right?
01:03:45
Speaker
And this is obviously an important part of who you are.
01:03:47
Speaker
So thanks for sharing that.
01:03:49
Speaker
For sure.
01:03:50
Speaker
The second question is, could you share something you changed your mind about over the last couple of years?
01:03:56
Speaker
Yeah.
01:03:56
Speaker
So, and, you know, when we say business,
01:03:58
Speaker
over the last couple of years is probably more like over the last 15 years.
01:04:03
Speaker
Um, but, uh, you know, I have to say that, you know, growing up as a, as a man and, you know, within kind of, uh, you know, I was in a fraternity in college and, you know, I played sports and a lot of locker room stuff.
01:04:18
Speaker
And, um, I always considered that,
01:04:21
Speaker
things like pornography and, you know, sleeping around, you know, and even prostitution to be essentially like victimless crimes.
01:04:33
Speaker
Right.
01:04:33
Speaker
And I just thought to myself, like, you know, who cares?
01:04:36
Speaker
It's consensual.
01:04:37
Speaker
It's between two adults, you know, that nobody's business.
01:04:40
Speaker
Right.
01:04:41
Speaker
And I've definitely changed my mind about that.
01:04:43
Speaker
I think some of it is my religious inclinations, you know, which came on later in adulthood.
01:04:49
Speaker
But I think more than that,
01:04:51
Speaker
It's kind of grasping what's going on in the, particularly in the porn industry with human trafficking and for that matter, prostitution with human trafficking and what has happened to women across the world that has just horrified me and, you know, made me very regretful of my attitude.
01:05:15
Speaker
And I think that the reason why those things happen is because there's a market, right?
01:05:21
Speaker
There is a complicit population that is fostering that.
01:05:27
Speaker
And I think it's definitely worth, I mean, deep reflection.
01:05:31
Speaker
So absolutely.
01:05:32
Speaker
Well, and having been part of that complicit, you know, population, let me say that I did it without thinking about it.
01:05:38
Speaker
Right.
01:05:38
Speaker
Meaning like I didn't think through what was, what was all this leading to.
01:05:44
Speaker
Right.
01:05:45
Speaker
And so that's, that's why I've changed my mind so radically is because now I've kind of seen some of that.
01:05:51
Speaker
Excellent.
01:05:51
Speaker
And the last question is, what would you want every person listening to our podcast today to know?
01:05:57
Speaker
Could be a quote or fact or just a departing thought.
01:06:01
Speaker
You know, I love the idea that, you know,
01:06:08
Speaker
attributed to different folks, but essentially the idea that discovery is an accident meeting a prepared mind.
01:06:15
Speaker
And, you know, it's the thought that as an intensivist, you know, we start a million different therapies and we have the benefit in the ICU of seeing the physiologic response and getting testing multiple times a day and things like that.
01:06:29
Speaker
And so we are able to very quickly see a reaction to something that we've done or prescribed that
01:06:38
Speaker
And I think if your mind is prepared, not just for the expected outcome, but for what are some potential explanations for alternative outcomes, that's when you start to make diagnoses that are, you know, potentially missed by others and can really change the course of the patient's disease.
01:06:56
Speaker
having that underlying knowledge to then be able to see the outcome and judge it and discern what was the result of that.
01:07:04
Speaker
And then adjust your therapies and diagnoses in keeping with that.
01:07:09
Speaker
Perfect.
01:07:10
Speaker
And I think this is a good place to stop.
01:07:12
Speaker
Thank you so much, Cameron, for sharing your expertise and being so generous with your time.
01:07:17
Speaker
I definitely enjoyed, I mean, learning from you on this fascinating topic.
01:07:22
Speaker
I hope to have you back on the podcast to discuss other topics related to critical care.
01:07:27
Speaker
Thanks a lot.
01:07:27
Speaker
I really appreciate your invitation, Sergio.
01:07:29
Speaker
And it's been great talking with you as well.
01:07:32
Speaker
Thank you for listening to Critical Matters, a sound podcast.
01:07:36
Speaker
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01:07:42
Speaker
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01:07:46
Speaker
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