January 31, 2025 • 32 mins
This week we review a recent ACTION NETWORK project comparing outcomes of children with CHD who undergo VAD support in comparison to those children with VAD's who do not have CHD. How do morbidity and mortality rates compare between these two cohorts? Why might there be differences in outcomes between these two groups? How are heart failure and transplant cardiologists going to improve outcomes in complex CHD VAD patients in the future? How can all cardiologists make more timely referrals to heart failure and transplant cardiologists for their congenital heart patients? Dr. Shahnawaz Amdani of the Cleveland Clinic provides many insights this week.
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Episode Transcript
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Speaker 1 (00:16):
Welcome to Pediheart Pediatric Cardiology Today. My name is doctor
Robert Pass and I'm the host of this podcast. I'm
Professor of Pediatrics at the Icons School of Medicine at
Mount SINAI thank you for joining me for this three
hundred and twenty eighth episode of Petiheart. I hope everybody
enjoyed last week's episode, in which we turned back the
clock to speak with doctor Jennifer Cohne about corrected transposition
(00:37):
in fetal life. For those of you interested in this
important topic, i'd recommend you to take a listen to
last week's discussion of a Fetal Heart Society Project episode
three hundred and twenty seven. As I say every week,
if you'd like to get in touch with me, my
email is easy to remember. It's Ptiheart at gmail dot com.
This week we move into the world of heart failure
and transplantation. The title of the work we'll be reviewing
(00:59):
is out Comes for Children with Congenital heart Disease undergoing
Ventricular assist Device Implantation and Action Registry Analysis. The first
author of this work is Shahnawaz Amdani and the senior
author Matthew J. O'Connor and doctor Amdani comes to us
from the Cleveland Clinic Children's Hospital in Cleveland, Ohio. When
we're done reviewing this paper, doctor Amdani has graciously agreed
(01:21):
to join us to discuss it. Therefore, let's get straight
onto the article and then a conversation with its first author.
The authors begin this week's work by commenting on how
our successes in the treatment of children with congenital heart
disease have resulted in growing numbers of pediatric patients with
congenital heart disease who are being evaluated for heart failure
related emergencies and hospitalizations, and how some children in that
(01:43):
circumstance will lead advanced heart failure therapies, including ventricular assist
devices and heart transplantation, and they reference pdmax data that
support this statement. They then comment on the technical challenges
of putting a VAD and a child with congenital heart disease,
and mention how there's only one month as I Center
trial assessing outcomes of VADs in congenital heart disease patients
(02:04):
from twenty twelve to twenty seventeen that showed that they
had a substantially higher POSTVAD mortality than non congenital heart
patients was actually a threefold difference in mortality. The authors
and explained how the Action Network or the Advanced Cardiac
Therapies Improving Outcomes Network, has resulted in many improvements in
the care of children on mechanical support and so with
(02:25):
these improvements, it's important to reassess outcomes for children undergoing
ventricular syst devices in the present era. With this as
a background, the authors state that the primary aim of
this study was to evaluate differences in POSTVAD implantation morbidity
immortality for children with congenital heart disease compared to those
who do not. The secondary aims were to evaluate differences
(02:46):
POSTVAD implantation morbidity and mortality among bivententricular congeneral heart disease
patients and univentricular congeneral heart disease patients, and also to
look at pre implantation differences in clinical characteristics between those
with a m without congenital heart disease as well as
those with and without univentricular congenital heart disease. This was
a retrospective study using the Action Network database for subjects
(03:09):
enrolled from April twenty eighteen to February twenty twenty three,
and it included all patients under nineteen years of age
with congeneral heart disease or those who did not have
it who had a VAD placed in that timeframe. As
would be expected, tons of data regarding demographic and clinical
details were recorded, as well as post operative clinical course
and adverse events. The authors explain how there are data
(03:31):
quality members of the action group to ensure that the
information recorded is robust and very accurate, and on page
four there is a very nice detailed description of the
statistical techniques that are used and for those interested in
large data set analysis for certain I'd recommend you to
take a look at this part of the paper and
all to the results. There were a total of nine
hundred and sixty six pediatric VAD implants in the time period,
(03:54):
of which thirty six percent had congenital heart disease and
sixty four percent did not. Age of the cohort was
four point four years and the majority, or fifty six
percent were male and fifty four percent were white. Thirty
one percent of patients were listed as intermax IE and
fifty three percent intermax TWIO. For those unfamiliar, Intermax one
represents a significantly more critical advanced heart failure compared to
(04:18):
intermax two, meaning patients classified as intermax one are considered
the most severe symptomatic and they require immediate, intensive life
saving interventions, while intermax two patients still have significant deterioration
but are not as acutely ill and may require high
doses of etotropes to main stability. Essentially, intermax one patients
are considered the sickest and most likely to require immediate
(04:40):
mechanical circulatary support like a VAD. Figure one is a
great barograph showing that from twenty eighteen to now, the
numbers of congenital heart disease patients has gotten larger and
larger steadily, while the non congenital heart disease patient group
has seemed fairly stable larger than the congenital heart disease group,
but with time the difference in numbers in patients is
smaller and smaller. Perhaps not surprisingly, the majority of these
(05:04):
patients who did not have congenital heart disease had dilated
cardimiopathy eighty one percent, and the medium follow up in
this work was two point six months. And so how
did the patients who got VADs and had congenital heart
disease compared to those who did not have congenital heart disease. Well,
congenital heart disease patients were younger, with a median age
of one point two years compared with nine point four
(05:25):
years in the non congenital heart disease cohort, and were
more likely white sixty four percent versus forty eight percent,
and they were generally sicker, with a higher percentage being
intermax one patients thirty six percent versus twenty nine percent.
They also had a higher billy rubin lower GFR, and
were more likely on mechanical ventilation or dialysis prior to device.
(05:47):
Ekmo was also used more commonly in the congenital heart
disease patients prior to a VAD thirty three percent versus
twenty one percent. Perhaps not surprisingly given the differences in age,
the congenital heart disease patients were more we were likely
to have a Berlin heart forty five percent or a
PDMAG or centrimag thirty three percent, and less likely to
have a HeartWare VAD or heart mate three used in
(06:09):
just eleven percent of congenital heart disease patients univentricular congenital
heart disease patients were generally younger by nearly a year
one versus one point seven versus their two ventricle comparison group,
and were more likely hispanic twenty three point five percent
versus twelve point three percent, and were more likely to
have a lower GFR and more likely to have a
(06:30):
VAD as a bridge to transplant or listed within twenty
four hours. However, there was no difference in pre implant
intermax level, mechanical ventilation or echmo use, or the type
of device in comparing the biventricular to univentricular patients. And
what about after the VAD, how did the congenital and
non congenital heart patients compare well. Generally, congenital heart disease
(06:52):
patients fared worse with more stroke bleeding, renal dysfunction, and
liver dysfunction, as well as more infections as well as
device XME malfunctions and respiratory failure. However, when comparing the
single to two ventricle VAD patients after the VAD, there
was more bleeding in the single ventrical patients, but rate
of stroke confections renual or hepatic dysfunction. Device malfunctions or
(07:14):
respiratory failure rates were similar in the two congenital heart
disease groups, and here is perhaps one of the most
important questions. How did mortality rates differ between those with
and without congenital heart disease and AVAD Well In a
multi variable adjusted analysis, those who had congenital heart disease
had significantly higher POSTVAD mortality comparing to non congenital heart
(07:36):
disease patients with a hazard ratio of two point zero five.
Speaker 2 (07:40):
Fig.
Speaker 1 (07:41):
Two is a very nice visual demonstration of the difference
in POSTVAD survival comparing those with congenital heart disease and
those without, showing a very significant divergence in lines in survival,
with survival on the Y axis and time of VAD
support on the X axis. The other risk factor for
death were use of EKMO before the use of a
VAD with a hasard ratio of one point nine to nine,
(08:02):
use of a para corporeal continuous device with a hazard
ratio of two point zho nine, and an elevated billyrubin
and lower GFR. In their discussion, the author's state and
I quote in this analysis of contemporary outcomes for children
undergoing bad implantation. There were several important findings. One, the
number of children with congel heart disease undergoing bad implantation,
(08:22):
particularly those with univentricular congenital heart disease, is rising. Two
Compared to children with non congenital heart disease, those with
congel heart disease are undergoing bad implantation at a more
advanced heart failure stage, more likely to be intermatics I
and non mechanical ventilations, dialysis and ECHMO. Three Compared with
children who do not have congel heart disease, those with
(08:45):
congel heart disease were significantly more likely to experience major
adverse events post bad implantation, stroke, bleeding, infection, renal dysfunction,
had paddock dysfunction, and respiratory failure. Four. In the current era,
children with congel heart disease, compared to those who do
not have congel heart disease, are twice as likely to
die post VAT implantation, and five, there were no differences
(09:07):
in post implantation's survival for those with univentricular and biventricular
congel heart disease undergoing VAD implantation. The authors then make
a few general observations about how the numbers of patients
getting VADs in the setting of congel heart disease is
rising rapidly within near tenfold rise in congelal heart disease
VAD implants in the most recent era reviewed in this report,
(09:27):
and also how two thirds of the congel heart disease
patients getting VADs are those who have a single ventricle,
highlighting for us the importance of this work and this
growing tsunami of challenging single ventrical patients. They reference the
single ventrical reconstructive trial which we've previously highlighted on the
padcast over the last seven years, demonstrating nearly forty percent
of children with single ventrical palliation dying or needing a
(09:51):
heart transplant in the six years after initial reconstructive operation.
The authors then make one of the most important points
of this work, namely that children with congect all heart
disease and heart failure are being referred late, which means
they are sicker at presentation, and they reference a prior
work on font Haines sent for transplantation evaluation, where it
was demonstrated that forty percent of these patients needed either
(10:12):
iotropes VADs or listing for transplant within only thirty days
of referral, and they review how this work was similar,
with the congenal heart disease patients in this work being
more commonly intermax one and having many other commobidities at
higher rates than the non congenital heart disease children in
this work. The author's reference the Action Networks recently released
guidelines for pediatric cardiologists caring for fontane patients with advanced
(10:37):
heart failure, which was written to try and improve referring
at a more optimal time, and I think this is
for sure something we'll discuss with doctor Amdani. The authors
speak about improving that outcomes and congenlal heart disease patients,
again touting earlier implantation and also the Action Network which
is famous for rapidly disseminating best practices amongst centers to
rapidly improve in all the centers providing these therapies. They
(11:00):
mentioned the finding that VAD single and two v patients
did similarly in this work and that this was somewhat
different from prior literature on this and they offer one
or two possibilities as to why, but ultimately suggest that
more work on this to confirm these observations is needed.
In regard to limitations, they point to the registry based
nature of the database, the absence of socioeconomic variables like
(11:23):
residential zip code, household income, or parental education or income,
and so this database largely misses social determinants of health
that have been shown in other works, some reviewed in
this podcast to be of importance. The authors also mention
how NONVAD surgery for those with congelal heart disease is
not included in the database, and so in some of
the congenal heart disease patients, the referral may not have
(11:45):
been late at all, but instead have been used as
a rescue following congeneral heart surgery that did not go well,
and so they conclude children with congenital heart disease, particularly
those with univentricular congeneral heart disease, are increasingly undergoing VAD implantation.
Children with congeneral heart disease are often in more advanced
heart failure compared to those with non congenital heart disease
(12:05):
at the time of bad implantation. Subsequently, such children with
congenital heart disease are twice as likely to die and
significantly more likely to experience major adverse events POSTVAD implantation
compared to those who do not have congenital heart disease.
There was no significant difference in POSTVAD implantation's survival for
those with UNI or bibin tricular congeneral heart disease. Timely
(12:27):
referral of congenal heart disease patients with heart failure for
bad implantation has the potential to improve outcomes for this
important cohort of children who at increased risk for experiencing
heart failure.
Speaker 3 (12:37):
Well.
Speaker 1 (12:38):
This is a very interesting work demonstrating that the numbers
of congeneral heart disease patients needingvads is growing rapidly, and
also that outcomes thus far are substantially inferior to those
without congenital heart disease. This is not entirely shocking given
what may be later referrals, as well as greater complexity
of placing a VAD in this complex and heterogeneous patient group.
(12:59):
I am interested to learn more about how the Action
network thinks that general cardiologists can be encouraged to send
their cases earlier to heart failure doctors and what it
is that we should be looking for. I also wonder
what doctor Amdani believes will be the factors that will
most improve these outcomes into the future. Thus, at this time,
I think we shall move forward with our conversation with
the works. First author joining us now to discuss this
(13:21):
week's work is the work's first author, Doctor Shahnawaz Amdani.
Doctor Amdani is the medical director of the VAD program
as well as Fontine Multidisciplinary Program and Cardio Oncology Program
at Cleveland Clinic Children's Hospital. Doctor Amdani is a graduate
of SMT NHL Municipal Medical College in Ahmedabad, India, followed
by a residency at Lincoln Medical Center in the Bronx,
(13:43):
New York. He completed fellowship in cardiology at Children's Hospital
of Michigan and Detroit, followed by heart failure and Transplantation
training at Saint Louis Children's Hospital. It is a pleasure
to have him join us this week. Welcome doctor Amdani
to Pdheart.
Speaker 3 (13:58):
I'm here now with doctor Amdani of the Clinic. Doctor Amdonie,
thank you very much for joining us this week on
the podcast.
Speaker 2 (14:04):
It's great to be here, doctor past real.
Speaker 3 (14:06):
Pleasure to have you, you know very much enjoyed this
very important work that you and your colleagues at the
Action Network have performed. One of the most important lessons
of the work, I think is that congeneral heart disease
patients were likely referred later in their heart failure course
than patients who do not have congenital heart disease, despite
being much younger. And I'm wondering what you believe are
(14:29):
the most important reasons that congeneral heart disease cases are
generally referred for advanced therapies like VADs or transplant later
than other pediatric patients who have heart failure, like, for example,
a dilated cardimotopathy patient.
Speaker 2 (14:45):
Yeah, I mean, that's a really important question.
Speaker 4 (14:47):
I think that there are several factors that contribute to
the later referm of congenital heart disease patients, And you know,
the first being the clinical complexity and the surgical history.
As we know, congeneral patients, specifically those with univentricular circulation,
have undergone multiple palliative surgeries, including stage valiations such as
the Fontaine procedure, and this creates a more intricate hemodynamic
(15:11):
and anatomic substrate that often leads to therapeutic hesitation regarding
advanced heart filler therapies. The second thing I'll say is
that there is delayed recognition.
Speaker 2 (15:20):
Of heart failure.
Speaker 4 (15:21):
We put out an AHA statement that specifically addresses that
it is more insiduous and a little bit more difficult
to determine progression of heart failure in those with congeneral
heart disease because of adapted physiologic baselines. So, to give
you a concrete example, they have lower oxygen saturations and
(15:42):
chronic exposure to systemic venous hypertension and low credit output,
they may mass the severity of the progression. The third
is really the perceived highsohyasurgical risk. Congeneral patients have more
challenging anatomic considerations for bad implant and or transplant, which
may lead to reluctance in early referraled by their primary
cardologists or surgical teams. The fourth is the limited VAD options. Unfortunately,
(16:07):
the available AADs, particularly the durable continuous flow gads that
have become ubiquitous in the adult world, are not well
suited for complex and general anatomy, making referral pathways less
defined compared to those for non congeneral vations. And the
final aspect is the institutional and provider availability. As we know,
some centers have limited experience in managing congeneral patients on
(16:30):
a ad or on their journey to a transplant, and
this may lead to differences in referral patterns and thresholds
for escalation of care. So there are numerous factors here.
Speaker 3 (16:39):
I see. Well, that was great, very clear, Thank you
very much, doctor m JOHNI I'm going to go out
of order the questions since you just referenced the Action
Network's guidelines for addressing the timing of referral for fonts
hand patients or who have heart failure who in order
to be evaluated by heart failure and transplant doctors. You
(17:00):
hinted a little bit at this already, Doctor of Donnie.
But I'm wondering if you could share with us some
of the more important principles that you and your colleagues
from Action have suggested in that document that we, as
more general cardiologists could learn to better inform our referral practices.
Speaker 2 (17:17):
Yeah.
Speaker 4 (17:17):
Sure, So the actual Learning network has really been at
the forefront of managing advanced heart failure in pediatric patients,
both in general and non congenital. And so one of
the things that we did was to put out a
comprehensive guideline that emphasizes earlier and proactive refutal of funtent patients.
Speaker 2 (17:37):
And so it has a couple.
Speaker 4 (17:38):
Of I'll give you some broad teams or important teams
from that paper. The first was early recognition of Fontaine failure,
and this guideline really stresses the importance of identifying early
signs of content failure, including you know, pl blastic bronchitis
and organ dysfunction and something as subtle as declining exercise tolerance.
The second aspect is routine surveillance and multidisciplinary care is key.
(18:03):
So structured surveillance strategy that incorporates biomarket analysis, CEPET testing,
liver imaging, hemodynamic assessments is recommended to identify patients who
may otherwise look good. And as we saw in the
paper that be published in JACK, they often end up
in the Advanced heart Failure Therapy REALM when their intermax one,
(18:23):
which is essentially critical cardiogenic shocks, so we want to
avoid that. The third aspect is understanding the optimal window
for bad consideration. So rather than waiting until a fontent
patient is in severe decompensation or an extremists. The guideline
suggests that patients with worstening fontent physiology, whether it's documented
by worsening societies, worstening and organ dysfunction, or something even
(18:46):
less ominous such as recurrent plural fusions or admissions for
just album and replacement, should be considered for bad evaluation
before severe and organ.
Speaker 2 (18:54):
Dysfunction and she ensues.
Speaker 4 (18:56):
And the final aspect is early referral for transplant evaluation.
Given that many fontent patients develop complications such as type need, ASK, resistence,
or get or sensitization from their crid operations and exposure
to blood products, early engagement re transplant centers and transplant
teams ensures a smoother transition through listing in eventual transplantation.
Speaker 3 (19:17):
I see, And for those in the audience, you should
be aware that doctor Amdonie not only is an expert
in heart failure and transplant, but also directs the fontane
clinic at the Cleveland Clinics. So I think they found
the right man for that job to really make that
transition and surveillance of those patients optimal. I'm sure, and
I'm sure you encounter failing fontane patients with some regularity.
Speaker 5 (19:42):
Yeah.
Speaker 3 (19:43):
Well, I was wondering, doctor Amdonnie, why you think that
patients with conjelal heart disease in general have such worse outcomes.
Is it all just late referral? Is it that a
lot of them are being referred as rescue from a
surgery that went bad and so are in just lousy
shape from that? Or do you believe there are other
(20:03):
factors that explain the majority of the very substantial enhanced
morbidity immortality that you described in your paper.
Speaker 4 (20:11):
Yeah, and I think you know what later. Perral certainly
plays a significant role. It is one piece of a
multifaceted challenge, and the other key contributors here are one
greater preoperative acuity are aslready highlighted that congeneral patients are
form far more likely to be intermax one at the
time of bad implant, requiring mechanical.
Speaker 2 (20:31):
Ventilation, dialysis and ekma.
Speaker 4 (20:33):
This severely ill preoperative state predisposes them to worse post
implant outcomes. As we demonstrated in our paper, It's not
only about survival. They have a increased risk for almost
all major adverse events after they have a bad implant.
Speaker 2 (20:48):
This includes stroke, infection, bleeding, and.
Speaker 4 (20:50):
Organ dysfunction compared to those non congeneral patients, and many
of these complications stem not only from their higher preoperative acuity,
but from their prior surgical alterations, the chronic exposure as
I mentioned before, to venus hypertension, and the higher incidence
really of the thromba embolic and the hamorrhagic complications that
those have with in general heart disease.
Speaker 2 (21:11):
The third is you know they have chronic end augment
dysfunction that is often not.
Speaker 4 (21:15):
As is often subtle and may manifest itself in a
more direct way after they have a bad implant, and
that certainly plays a role into all the post operative
complications that we see. The last two comments I'll make
is one is the ventricular morphology and pump limitation.
Speaker 2 (21:33):
So many con general patients, particularly.
Speaker 4 (21:35):
Those with univentrical physiology required as we do in our center,
and I'm sure you are in other major con general centers,
require non standard can relation strategies and have sub optimum
ventricular mechanics that affects, honestly in the long run, pump
efficiency and increases the thrombo embolic complications that we see
and then nationally, you know, over the last few years
(21:56):
since the unfortunately since the COVID pandemic, we're seeing the
long beaitless time that these patients in general have to
go through. And these bids were never designed for durability
in the long term, you know. And then they're getting
blood transfusions, they're getting more sensitized, and we really need
optimal size, matched anatomic compatible donors, which.
Speaker 2 (22:19):
Are scarce unfortunately in the current area.
Speaker 4 (22:21):
So there are multiple reasons why they are more predisposed
and inclined to have worse post and planned outcomes.
Speaker 2 (22:26):
Interesting.
Speaker 3 (22:27):
I never really thought about the fact that these devices
are not intended to be as long used. They're not
like destination therapy as some of the continuous devices are
in the adult world. So I guess that's a very
very important point. Thank you well for those in the audience.
I got Tuctor Aremdanni right in the middle of his
very busy day at the Cleveland clinic, and so I'm
(22:48):
going to take advantage of the fact that we have
them and ask them one or two questions now that
have nothing really to do with his paper, but sort
of peripherally because we have an expert here in heart
failure and transplant and device support. I was wondering, as
a highly experienced heart failure and transplant expert, what factors
are innovations going forward do you think will more positively
(23:09):
impact the outcomes of congenital heart patients who need a AD,
so that the outcomes start to approach the generally excellent
ones that you have demonstrated, those who don't have congenital
heart disease, and even more, I'm going to just take
advantage of the fact that we have you here. How
far are we from a durable total artificial heart and
kids or even zeno transplantation so we don't have to
(23:31):
think about these VADs anymore. Just pop in a new
Jarvik twenty nine and ready to go for the next
seventy years.
Speaker 4 (23:39):
Yeah, I mean, this is such a critical aspect of
our jobs and certainly keeps me up at nine, as
I know of many others in the field around the
country and around the world. But I'd say a couple
of things here, and I think there's several key advancements
that could improve outcomes for this really vulnerable cohort of
congenital patients who're experiencing advanced heart failure. The first is,
you know, we need base specific bad cannulation and flow strategies,
(24:03):
and I know that there's a lot of ongoing working
optimizing cannulation strategies specifically for those with univentricular hearts and
improving flow dynamics and smaller patients that will be critical
I think in reducing throw bymbolic and hemo dynamic complications
that we see. The second aspect is really you know,
we've been forgotten in the pediatric realm. You know most
(24:24):
of the bads have been designed for adults, and why
we've gotten really good at successfully putting them in kids
using novel techniques. I think what we need is miniaturization
and design that is specific for pediatric pasions and more
specific for conngineral heart disease patients. The development of these
smaller CONTAINUS flow devices is going to be key and
(24:46):
is a major area of need for US. And I
say the third point here is that I think we
should we're gonna eventually get really good at hybrid support
strategies so that c mode of AD transition as we
define in our paper. There are a lot of gentle patients
end up on more first, So how do you bridge
them these critically ill rdiogenic shock patients with congeneral heart
disease from echmore to durable support while optimizing.
Speaker 2 (25:08):
Their end organ function. I think is going to be key.
Speaker 4 (25:11):
And I think the Akpella strategy, the ecmobilis embella strategy
specifically for the older pediatric patients, has now been tested
and I could see that being transitioned into the congeneral population.
I think specific to bads, i'd say biologic modifications start
are needed to improve the hemodynamic the sorry the hemo
compatibility related adverse events, to reduce it from bartybars, to
(25:34):
reduce inflammation POSTWAD. That could improve the outcomes. And i'd
say specific to artificial hearts, you know the total artificial
heart is not really optimized for the pediatric congeneral patients.
But I know that the newer iterations the Bible Core
tool artificial heart is showing some promise overall in the
adult world. But I could if you had asked me now,
(25:57):
I would say, certainly pediatric uses is a few years,
if not a decade away. As far as xeno transplantation,
as we've seen in the last years, recent basements in
genetically engineered big arts have demonstrated some early peace ability,
but rejection durability.
Speaker 2 (26:14):
To main major concerns.
Speaker 4 (26:16):
You know, Zeno transplants started with Leonard Bailey, didn't transplant.
Speaker 2 (26:20):
Forty years ago.
Speaker 4 (26:21):
A yeah, and you know it's interesting that we're still
sort of in the infancy stages, but clinical application is
still I would say several years, if not decades away,
But Certaly would be a dream of mine.
Speaker 2 (26:33):
And I know many of my colleagues are on there.
Speaker 3 (26:35):
I see. Yeah, well that was wonderful. You know, I'm
very dear friends with Gouvil Ferruki, who's been working on
imaging approaches to optimize VAT placement and congenital patients. But
despite her brilliance and the effort she's doing, I think
you're right that the devices themselves were sort of trying
(26:57):
to put a size seven foot and a size for
a shoe or something along those lines. Well, doctor Amdannie,
I can't thank you enough. I want to thank you
so much for joining us this week on the podcast,
and I want to congratulate you in all the many
many centers and co authors on this very important project.
I would encourage everybody to go read the paper. It's
(27:18):
a lot of important points made. Thank you so much.
Speaker 2 (27:22):
Thank you so much, Doctor Best for having me. It's
an honor.
Speaker 4 (27:24):
And I just wanted to give a shout out to
the Action Learning Network for really leading the way here.
Speaker 3 (27:28):
Thank you, thank you so much.
Speaker 2 (27:30):
Well.
Speaker 1 (27:30):
As I'm apt to say, when the guest is good,
there's often not much to add. Doctor Amdanie offered a
number of reasons he thinks that congeneral heart disease patients
do worse with AADs, and I think that when thinking
about all of these many factors, one could make the
argument that the outcomes reported here are remarkable, but clearly
have a way to go in reaching the outstanding results
that we expect in the present era for patients who
(27:52):
do not have congenital heart disease. I found his comments
about timing of congeneral heart referrals to be of great
interest and was also interested to hear his thoughts on
how we can improve ADS in the future to be
better suited to our congenital heart patients. I am most
appreciative to him for taking time out of his very
busy schedule right in the middle of his day to
speak with us this week on the podcast. To conclude
(28:14):
this three hundred and twenty eighth episode of Peedhart Pediatric Carreology,
Today we hear the wonderful lyrico spinto Bulgarian soprano Rena Kabavanska.
Miss Kabovanska was born in Burgis, Bulgaria, and she started
in Sofia and made her professional opera debut at the
Bulgarian National Opera in nineteen fifty seven. Shortly thereafter, she
(28:35):
began performing in Italy, where she had many gigantic successes
and where she was known for her wonderful voice married
to her authentic acting skills. Miss Kabovanska sang at the
funeral of her co star Luciano Pavarotti in two thousand
and seven, and she works today still as a professor
in Siena, Italy, as well as at the New Bulgarian
(28:55):
University in Sofia in Bulgaria. Today we hear her in
one of the roles for which she was quite famous,
singing Tosca's Visitiarte from Actu Aupuccini's masterpiece. Thank you so
much for joining me for this three hundred and twenty
eighth episode, and thanks once again to our guest doctor.
Speaker 6 (29:12):
I'mdani. I hope I'll have a good week ahead.
Speaker 5 (30:00):
Oh, sign.
Speaker 7 (30:17):
The chain.
Speaker 5 (30:23):
On, lady. He saw me and nobody saw me sign
for the racle.
Speaker 7 (30:35):
Chains didn't really joined the com Ball.
Speaker 5 (31:32):
Signs. Say ask me a h had you the recy
(32:31):
gol
Welcome to Pediheart Pediatric Cardiology Today. My name is doctor
Robert Pass and I'm the host of this podcast. I'm
Professor of Pediatrics at the Icons School of Medicine at
Mount SINAI thank you for joining me for this three
hundred and twenty eighth episode of Petiheart. I hope everybody
enjoyed last week's episode, in which we turned back the
clock to speak with doctor Jennifer Cohne about corrected transposition
(00:37):
in fetal life. For those of you interested in this
important topic, i'd recommend you to take a listen to
last week's discussion of a Fetal Heart Society Project episode
three hundred and twenty seven. As I say every week,
if you'd like to get in touch with me, my
email is easy to remember. It's Ptiheart at gmail dot com.
This week we move into the world of heart failure
and transplantation. The title of the work we'll be reviewing
(00:59):
is out Comes for Children with Congenital heart Disease undergoing
Ventricular assist Device Implantation and Action Registry Analysis. The first
author of this work is Shahnawaz Amdani and the senior
author Matthew J. O'Connor and doctor Amdani comes to us
from the Cleveland Clinic Children's Hospital in Cleveland, Ohio. When
we're done reviewing this paper, doctor Amdani has graciously agreed
(01:21):
to join us to discuss it. Therefore, let's get straight
onto the article and then a conversation with its first author.
The authors begin this week's work by commenting on how
our successes in the treatment of children with congenital heart
disease have resulted in growing numbers of pediatric patients with
congenital heart disease who are being evaluated for heart failure
related emergencies and hospitalizations, and how some children in that
(01:43):
circumstance will lead advanced heart failure therapies, including ventricular assist
devices and heart transplantation, and they reference pdmax data that
support this statement. They then comment on the technical challenges
of putting a VAD and a child with congenital heart disease,
and mention how there's only one month as I Center
trial assessing outcomes of VADs in congenital heart disease patients
(02:04):
from twenty twelve to twenty seventeen that showed that they
had a substantially higher POSTVAD mortality than non congenital heart
patients was actually a threefold difference in mortality. The authors
and explained how the Action Network or the Advanced Cardiac
Therapies Improving Outcomes Network, has resulted in many improvements in
the care of children on mechanical support and so with
(02:25):
these improvements, it's important to reassess outcomes for children undergoing
ventricular syst devices in the present era. With this as
a background, the authors state that the primary aim of
this study was to evaluate differences in POSTVAD implantation morbidity
immortality for children with congenital heart disease compared to those
who do not. The secondary aims were to evaluate differences
(02:46):
POSTVAD implantation morbidity and mortality among bivententricular congeneral heart disease
patients and univentricular congeneral heart disease patients, and also to
look at pre implantation differences in clinical characteristics between those
with a m without congenital heart disease as well as
those with and without univentricular congenital heart disease. This was
a retrospective study using the Action Network database for subjects
(03:09):
enrolled from April twenty eighteen to February twenty twenty three,
and it included all patients under nineteen years of age
with congeneral heart disease or those who did not have
it who had a VAD placed in that timeframe. As
would be expected, tons of data regarding demographic and clinical
details were recorded, as well as post operative clinical course
and adverse events. The authors explain how there are data
(03:31):
quality members of the action group to ensure that the
information recorded is robust and very accurate, and on page
four there is a very nice detailed description of the
statistical techniques that are used and for those interested in
large data set analysis for certain I'd recommend you to
take a look at this part of the paper and
all to the results. There were a total of nine
hundred and sixty six pediatric VAD implants in the time period,
(03:54):
of which thirty six percent had congenital heart disease and
sixty four percent did not. Age of the cohort was
four point four years and the majority, or fifty six
percent were male and fifty four percent were white. Thirty
one percent of patients were listed as intermax IE and
fifty three percent intermax TWIO. For those unfamiliar, Intermax one
represents a significantly more critical advanced heart failure compared to
(04:18):
intermax two, meaning patients classified as intermax one are considered
the most severe symptomatic and they require immediate, intensive life
saving interventions, while intermax two patients still have significant deterioration
but are not as acutely ill and may require high
doses of etotropes to main stability. Essentially, intermax one patients
are considered the sickest and most likely to require immediate
(04:40):
mechanical circulatary support like a VAD. Figure one is a
great barograph showing that from twenty eighteen to now, the
numbers of congenital heart disease patients has gotten larger and
larger steadily, while the non congenital heart disease patient group
has seemed fairly stable larger than the congenital heart disease group,
but with time the difference in numbers in patients is
smaller and smaller. Perhaps not surprisingly, the majority of these
(05:04):
patients who did not have congenital heart disease had dilated
cardimiopathy eighty one percent, and the medium follow up in
this work was two point six months. And so how
did the patients who got VADs and had congenital heart
disease compared to those who did not have congenital heart disease. Well,
congenital heart disease patients were younger, with a median age
of one point two years compared with nine point four
(05:25):
years in the non congenital heart disease cohort, and were
more likely white sixty four percent versus forty eight percent,
and they were generally sicker, with a higher percentage being
intermax one patients thirty six percent versus twenty nine percent.
They also had a higher billy rubin lower GFR, and
were more likely on mechanical ventilation or dialysis prior to device.
(05:47):
Ekmo was also used more commonly in the congenital heart
disease patients prior to a VAD thirty three percent versus
twenty one percent. Perhaps not surprisingly given the differences in age,
the congenital heart disease patients were more we were likely
to have a Berlin heart forty five percent or a
PDMAG or centrimag thirty three percent, and less likely to
have a HeartWare VAD or heart mate three used in
(06:09):
just eleven percent of congenital heart disease patients univentricular congenital
heart disease patients were generally younger by nearly a year
one versus one point seven versus their two ventricle comparison group,
and were more likely hispanic twenty three point five percent
versus twelve point three percent, and were more likely to
have a lower GFR and more likely to have a
(06:30):
VAD as a bridge to transplant or listed within twenty
four hours. However, there was no difference in pre implant
intermax level, mechanical ventilation or echmo use, or the type
of device in comparing the biventricular to univentricular patients. And
what about after the VAD, how did the congenital and
non congenital heart patients compare well. Generally, congenital heart disease
(06:52):
patients fared worse with more stroke bleeding, renal dysfunction, and
liver dysfunction, as well as more infections as well as
device XME malfunctions and respiratory failure. However, when comparing the
single to two ventricle VAD patients after the VAD, there
was more bleeding in the single ventrical patients, but rate
of stroke confections renual or hepatic dysfunction. Device malfunctions or
(07:14):
respiratory failure rates were similar in the two congenital heart
disease groups, and here is perhaps one of the most
important questions. How did mortality rates differ between those with
and without congenital heart disease and AVAD Well In a
multi variable adjusted analysis, those who had congenital heart disease
had significantly higher POSTVAD mortality comparing to non congenital heart
(07:36):
disease patients with a hazard ratio of two point zero five.
Speaker 2 (07:40):
Fig.
Speaker 1 (07:41):
Two is a very nice visual demonstration of the difference
in POSTVAD survival comparing those with congenital heart disease and
those without, showing a very significant divergence in lines in survival,
with survival on the Y axis and time of VAD
support on the X axis. The other risk factor for
death were use of EKMO before the use of a
VAD with a hasard ratio of one point nine to nine,
(08:02):
use of a para corporeal continuous device with a hazard
ratio of two point zho nine, and an elevated billyrubin
and lower GFR. In their discussion, the author's state and
I quote in this analysis of contemporary outcomes for children
undergoing bad implantation. There were several important findings. One, the
number of children with congel heart disease undergoing bad implantation,
(08:22):
particularly those with univentricular congenital heart disease, is rising. Two
Compared to children with non congenital heart disease, those with
congel heart disease are undergoing bad implantation at a more
advanced heart failure stage, more likely to be intermatics I
and non mechanical ventilations, dialysis and ECHMO. Three Compared with
children who do not have congel heart disease, those with
(08:45):
congel heart disease were significantly more likely to experience major
adverse events post bad implantation, stroke, bleeding, infection, renal dysfunction,
had paddock dysfunction, and respiratory failure. Four. In the current era,
children with congel heart disease, compared to those who do
not have congel heart disease, are twice as likely to
die post VAT implantation, and five, there were no differences
(09:07):
in post implantation's survival for those with univentricular and biventricular
congel heart disease undergoing VAD implantation. The authors then make
a few general observations about how the numbers of patients
getting VADs in the setting of congel heart disease is
rising rapidly within near tenfold rise in congelal heart disease
VAD implants in the most recent era reviewed in this report,
(09:27):
and also how two thirds of the congel heart disease
patients getting VADs are those who have a single ventricle,
highlighting for us the importance of this work and this
growing tsunami of challenging single ventrical patients. They reference the
single ventrical reconstructive trial which we've previously highlighted on the
padcast over the last seven years, demonstrating nearly forty percent
of children with single ventrical palliation dying or needing a
(09:51):
heart transplant in the six years after initial reconstructive operation.
The authors then make one of the most important points
of this work, namely that children with congect all heart
disease and heart failure are being referred late, which means
they are sicker at presentation, and they reference a prior
work on font Haines sent for transplantation evaluation, where it
was demonstrated that forty percent of these patients needed either
(10:12):
iotropes VADs or listing for transplant within only thirty days
of referral, and they review how this work was similar,
with the congenal heart disease patients in this work being
more commonly intermax one and having many other commobidities at
higher rates than the non congenital heart disease children in
this work. The author's reference the Action Networks recently released
guidelines for pediatric cardiologists caring for fontane patients with advanced
(10:37):
heart failure, which was written to try and improve referring
at a more optimal time, and I think this is
for sure something we'll discuss with doctor Amdani. The authors
speak about improving that outcomes and congenlal heart disease patients,
again touting earlier implantation and also the Action Network which
is famous for rapidly disseminating best practices amongst centers to
rapidly improve in all the centers providing these therapies. They
(11:00):
mentioned the finding that VAD single and two v patients
did similarly in this work and that this was somewhat
different from prior literature on this and they offer one
or two possibilities as to why, but ultimately suggest that
more work on this to confirm these observations is needed.
In regard to limitations, they point to the registry based
nature of the database, the absence of socioeconomic variables like
(11:23):
residential zip code, household income, or parental education or income,
and so this database largely misses social determinants of health
that have been shown in other works, some reviewed in
this podcast to be of importance. The authors also mention
how NONVAD surgery for those with congelal heart disease is
not included in the database, and so in some of
the congenal heart disease patients, the referral may not have
(11:45):
been late at all, but instead have been used as
a rescue following congeneral heart surgery that did not go well,
and so they conclude children with congenital heart disease, particularly
those with univentricular congeneral heart disease, are increasingly undergoing VAD implantation.
Children with congeneral heart disease are often in more advanced
heart failure compared to those with non congenital heart disease
(12:05):
at the time of bad implantation. Subsequently, such children with
congenital heart disease are twice as likely to die and
significantly more likely to experience major adverse events POSTVAD implantation
compared to those who do not have congenital heart disease.
There was no significant difference in POSTVAD implantation's survival for
those with UNI or bibin tricular congeneral heart disease. Timely
(12:27):
referral of congenal heart disease patients with heart failure for
bad implantation has the potential to improve outcomes for this
important cohort of children who at increased risk for experiencing
heart failure.
Speaker 3 (12:37):
Well.
Speaker 1 (12:38):
This is a very interesting work demonstrating that the numbers
of congeneral heart disease patients needingvads is growing rapidly, and
also that outcomes thus far are substantially inferior to those
without congenital heart disease. This is not entirely shocking given
what may be later referrals, as well as greater complexity
of placing a VAD in this complex and heterogeneous patient group.
(12:59):
I am interested to learn more about how the Action
network thinks that general cardiologists can be encouraged to send
their cases earlier to heart failure doctors and what it
is that we should be looking for. I also wonder
what doctor Amdani believes will be the factors that will
most improve these outcomes into the future. Thus, at this time,
I think we shall move forward with our conversation with
the works. First author joining us now to discuss this
(13:21):
week's work is the work's first author, Doctor Shahnawaz Amdani.
Doctor Amdani is the medical director of the VAD program
as well as Fontine Multidisciplinary Program and Cardio Oncology Program
at Cleveland Clinic Children's Hospital. Doctor Amdani is a graduate
of SMT NHL Municipal Medical College in Ahmedabad, India, followed
by a residency at Lincoln Medical Center in the Bronx,
(13:43):
New York. He completed fellowship in cardiology at Children's Hospital
of Michigan and Detroit, followed by heart failure and Transplantation
training at Saint Louis Children's Hospital. It is a pleasure
to have him join us this week. Welcome doctor Amdani
to Pdheart.
Speaker 3 (13:58):
I'm here now with doctor Amdani of the Clinic. Doctor Amdonie,
thank you very much for joining us this week on
the podcast.
Speaker 2 (14:04):
It's great to be here, doctor past real.
Speaker 3 (14:06):
Pleasure to have you, you know very much enjoyed this
very important work that you and your colleagues at the
Action Network have performed. One of the most important lessons
of the work, I think is that congeneral heart disease
patients were likely referred later in their heart failure course
than patients who do not have congenital heart disease, despite
being much younger. And I'm wondering what you believe are
(14:29):
the most important reasons that congeneral heart disease cases are
generally referred for advanced therapies like VADs or transplant later
than other pediatric patients who have heart failure, like, for example,
a dilated cardimotopathy patient.
Speaker 2 (14:45):
Yeah, I mean, that's a really important question.
Speaker 4 (14:47):
I think that there are several factors that contribute to
the later referm of congenital heart disease patients, And you know,
the first being the clinical complexity and the surgical history.
As we know, congeneral patients, specifically those with univentricular circulation,
have undergone multiple palliative surgeries, including stage valiations such as
the Fontaine procedure, and this creates a more intricate hemodynamic
(15:11):
and anatomic substrate that often leads to therapeutic hesitation regarding
advanced heart filler therapies. The second thing I'll say is
that there is delayed recognition.
Speaker 2 (15:20):
Of heart failure.
Speaker 4 (15:21):
We put out an AHA statement that specifically addresses that
it is more insiduous and a little bit more difficult
to determine progression of heart failure in those with congeneral
heart disease because of adapted physiologic baselines. So, to give
you a concrete example, they have lower oxygen saturations and
(15:42):
chronic exposure to systemic venous hypertension and low credit output,
they may mass the severity of the progression. The third
is really the perceived highsohyasurgical risk. Congeneral patients have more
challenging anatomic considerations for bad implant and or transplant, which
may lead to reluctance in early referraled by their primary
cardologists or surgical teams. The fourth is the limited VAD options. Unfortunately,
(16:07):
the available AADs, particularly the durable continuous flow gads that
have become ubiquitous in the adult world, are not well
suited for complex and general anatomy, making referral pathways less
defined compared to those for non congeneral vations. And the
final aspect is the institutional and provider availability. As we know,
some centers have limited experience in managing congeneral patients on
(16:30):
a ad or on their journey to a transplant, and
this may lead to differences in referral patterns and thresholds
for escalation of care. So there are numerous factors here.
Speaker 3 (16:39):
I see. Well, that was great, very clear, Thank you
very much, doctor m JOHNI I'm going to go out
of order the questions since you just referenced the Action
Network's guidelines for addressing the timing of referral for fonts
hand patients or who have heart failure who in order
to be evaluated by heart failure and transplant doctors. You
(17:00):
hinted a little bit at this already, Doctor of Donnie.
But I'm wondering if you could share with us some
of the more important principles that you and your colleagues
from Action have suggested in that document that we, as
more general cardiologists could learn to better inform our referral practices.
Speaker 2 (17:17):
Yeah.
Speaker 4 (17:17):
Sure, So the actual Learning network has really been at
the forefront of managing advanced heart failure in pediatric patients,
both in general and non congenital. And so one of
the things that we did was to put out a
comprehensive guideline that emphasizes earlier and proactive refutal of funtent patients.
Speaker 2 (17:37):
And so it has a couple.
Speaker 4 (17:38):
Of I'll give you some broad teams or important teams
from that paper. The first was early recognition of Fontaine failure,
and this guideline really stresses the importance of identifying early
signs of content failure, including you know, pl blastic bronchitis
and organ dysfunction and something as subtle as declining exercise tolerance.
The second aspect is routine surveillance and multidisciplinary care is key.
(18:03):
So structured surveillance strategy that incorporates biomarket analysis, CEPET testing,
liver imaging, hemodynamic assessments is recommended to identify patients who
may otherwise look good. And as we saw in the
paper that be published in JACK, they often end up
in the Advanced heart Failure Therapy REALM when their intermax one,
(18:23):
which is essentially critical cardiogenic shocks, so we want to
avoid that. The third aspect is understanding the optimal window
for bad consideration. So rather than waiting until a fontent
patient is in severe decompensation or an extremists. The guideline
suggests that patients with worstening fontent physiology, whether it's documented
by worsening societies, worstening and organ dysfunction, or something even
(18:46):
less ominous such as recurrent plural fusions or admissions for
just album and replacement, should be considered for bad evaluation
before severe and organ.
Speaker 2 (18:54):
Dysfunction and she ensues.
Speaker 4 (18:56):
And the final aspect is early referral for transplant evaluation.
Given that many fontent patients develop complications such as type need, ASK, resistence,
or get or sensitization from their crid operations and exposure
to blood products, early engagement re transplant centers and transplant
teams ensures a smoother transition through listing in eventual transplantation.
Speaker 3 (19:17):
I see, And for those in the audience, you should
be aware that doctor Amdonie not only is an expert
in heart failure and transplant, but also directs the fontane
clinic at the Cleveland Clinics. So I think they found
the right man for that job to really make that
transition and surveillance of those patients optimal. I'm sure, and
I'm sure you encounter failing fontane patients with some regularity.
Speaker 5 (19:42):
Yeah.
Speaker 3 (19:43):
Well, I was wondering, doctor Amdonnie, why you think that
patients with conjelal heart disease in general have such worse outcomes.
Is it all just late referral? Is it that a
lot of them are being referred as rescue from a
surgery that went bad and so are in just lousy
shape from that? Or do you believe there are other
(20:03):
factors that explain the majority of the very substantial enhanced
morbidity immortality that you described in your paper.
Speaker 4 (20:11):
Yeah, and I think you know what later. Perral certainly
plays a significant role. It is one piece of a
multifaceted challenge, and the other key contributors here are one
greater preoperative acuity are aslready highlighted that congeneral patients are
form far more likely to be intermax one at the
time of bad implant, requiring mechanical.
Speaker 2 (20:31):
Ventilation, dialysis and ekma.
Speaker 4 (20:33):
This severely ill preoperative state predisposes them to worse post
implant outcomes. As we demonstrated in our paper, It's not
only about survival. They have a increased risk for almost
all major adverse events after they have a bad implant.
Speaker 2 (20:48):
This includes stroke, infection, bleeding, and.
Speaker 4 (20:50):
Organ dysfunction compared to those non congeneral patients, and many
of these complications stem not only from their higher preoperative acuity,
but from their prior surgical alterations, the chronic exposure as
I mentioned before, to venus hypertension, and the higher incidence
really of the thromba embolic and the hamorrhagic complications that
those have with in general heart disease.
Speaker 2 (21:11):
The third is you know they have chronic end augment
dysfunction that is often not.
Speaker 4 (21:15):
As is often subtle and may manifest itself in a
more direct way after they have a bad implant, and
that certainly plays a role into all the post operative
complications that we see. The last two comments I'll make
is one is the ventricular morphology and pump limitation.
Speaker 2 (21:33):
So many con general patients, particularly.
Speaker 4 (21:35):
Those with univentrical physiology required as we do in our center,
and I'm sure you are in other major con general centers,
require non standard can relation strategies and have sub optimum
ventricular mechanics that affects, honestly in the long run, pump
efficiency and increases the thrombo embolic complications that we see
and then nationally, you know, over the last few years
(21:56):
since the unfortunately since the COVID pandemic, we're seeing the
long beaitless time that these patients in general have to
go through. And these bids were never designed for durability
in the long term, you know. And then they're getting
blood transfusions, they're getting more sensitized, and we really need
optimal size, matched anatomic compatible donors, which.
Speaker 2 (22:19):
Are scarce unfortunately in the current area.
Speaker 4 (22:21):
So there are multiple reasons why they are more predisposed
and inclined to have worse post and planned outcomes.
Speaker 2 (22:26):
Interesting.
Speaker 3 (22:27):
I never really thought about the fact that these devices
are not intended to be as long used. They're not
like destination therapy as some of the continuous devices are
in the adult world. So I guess that's a very
very important point. Thank you well for those in the audience.
I got Tuctor Aremdanni right in the middle of his
very busy day at the Cleveland clinic, and so I'm
(22:48):
going to take advantage of the fact that we have
them and ask them one or two questions now that
have nothing really to do with his paper, but sort
of peripherally because we have an expert here in heart
failure and transplant and device support. I was wondering, as
a highly experienced heart failure and transplant expert, what factors
are innovations going forward do you think will more positively
(23:09):
impact the outcomes of congenital heart patients who need a AD,
so that the outcomes start to approach the generally excellent
ones that you have demonstrated, those who don't have congenital
heart disease, and even more, I'm going to just take
advantage of the fact that we have you here. How
far are we from a durable total artificial heart and
kids or even zeno transplantation so we don't have to
(23:31):
think about these VADs anymore. Just pop in a new
Jarvik twenty nine and ready to go for the next
seventy years.
Speaker 4 (23:39):
Yeah, I mean, this is such a critical aspect of
our jobs and certainly keeps me up at nine, as
I know of many others in the field around the
country and around the world. But I'd say a couple
of things here, and I think there's several key advancements
that could improve outcomes for this really vulnerable cohort of
congenital patients who're experiencing advanced heart failure. The first is,
you know, we need base specific bad cannulation and flow strategies,
(24:03):
and I know that there's a lot of ongoing working
optimizing cannulation strategies specifically for those with univentricular hearts and
improving flow dynamics and smaller patients that will be critical
I think in reducing throw bymbolic and hemo dynamic complications
that we see. The second aspect is really you know,
we've been forgotten in the pediatric realm. You know most
(24:24):
of the bads have been designed for adults, and why
we've gotten really good at successfully putting them in kids
using novel techniques. I think what we need is miniaturization
and design that is specific for pediatric pasions and more
specific for conngineral heart disease patients. The development of these
smaller CONTAINUS flow devices is going to be key and
(24:46):
is a major area of need for US. And I
say the third point here is that I think we
should we're gonna eventually get really good at hybrid support
strategies so that c mode of AD transition as we
define in our paper. There are a lot of gentle patients
end up on more first, So how do you bridge
them these critically ill rdiogenic shock patients with congeneral heart
disease from echmore to durable support while optimizing.
Speaker 2 (25:08):
Their end organ function. I think is going to be key.
Speaker 4 (25:11):
And I think the Akpella strategy, the ecmobilis embella strategy
specifically for the older pediatric patients, has now been tested
and I could see that being transitioned into the congeneral population.
I think specific to bads, i'd say biologic modifications start
are needed to improve the hemodynamic the sorry the hemo
compatibility related adverse events, to reduce it from bartybars, to
(25:34):
reduce inflammation POSTWAD. That could improve the outcomes. And i'd
say specific to artificial hearts, you know the total artificial
heart is not really optimized for the pediatric congeneral patients.
But I know that the newer iterations the Bible Core
tool artificial heart is showing some promise overall in the
adult world. But I could if you had asked me now,
(25:57):
I would say, certainly pediatric uses is a few years,
if not a decade away. As far as xeno transplantation,
as we've seen in the last years, recent basements in
genetically engineered big arts have demonstrated some early peace ability,
but rejection durability.
Speaker 2 (26:14):
To main major concerns.
Speaker 4 (26:16):
You know, Zeno transplants started with Leonard Bailey, didn't transplant.
Speaker 2 (26:20):
Forty years ago.
Speaker 4 (26:21):
A yeah, and you know it's interesting that we're still
sort of in the infancy stages, but clinical application is
still I would say several years, if not decades away,
But Certaly would be a dream of mine.
Speaker 2 (26:33):
And I know many of my colleagues are on there.
Speaker 3 (26:35):
I see. Yeah, well that was wonderful. You know, I'm
very dear friends with Gouvil Ferruki, who's been working on
imaging approaches to optimize VAT placement and congenital patients. But
despite her brilliance and the effort she's doing, I think
you're right that the devices themselves were sort of trying
(26:57):
to put a size seven foot and a size for
a shoe or something along those lines. Well, doctor Amdannie,
I can't thank you enough. I want to thank you
so much for joining us this week on the podcast,
and I want to congratulate you in all the many
many centers and co authors on this very important project.
I would encourage everybody to go read the paper. It's
(27:18):
a lot of important points made. Thank you so much.
Speaker 2 (27:22):
Thank you so much, Doctor Best for having me. It's
an honor.
Speaker 4 (27:24):
And I just wanted to give a shout out to
the Action Learning Network for really leading the way here.
Speaker 3 (27:28):
Thank you, thank you so much.
Speaker 2 (27:30):
Well.
Speaker 1 (27:30):
As I'm apt to say, when the guest is good,
there's often not much to add. Doctor Amdanie offered a
number of reasons he thinks that congeneral heart disease patients
do worse with AADs, and I think that when thinking
about all of these many factors, one could make the
argument that the outcomes reported here are remarkable, but clearly
have a way to go in reaching the outstanding results
that we expect in the present era for patients who
(27:52):
do not have congenital heart disease. I found his comments
about timing of congeneral heart referrals to be of great
interest and was also interested to hear his thoughts on
how we can improve ADS in the future to be
better suited to our congenital heart patients. I am most
appreciative to him for taking time out of his very
busy schedule right in the middle of his day to
speak with us this week on the podcast. To conclude
(28:14):
this three hundred and twenty eighth episode of Peedhart Pediatric Carreology,
Today we hear the wonderful lyrico spinto Bulgarian soprano Rena Kabavanska.
Miss Kabovanska was born in Burgis, Bulgaria, and she started
in Sofia and made her professional opera debut at the
Bulgarian National Opera in nineteen fifty seven. Shortly thereafter, she
(28:35):
began performing in Italy, where she had many gigantic successes
and where she was known for her wonderful voice married
to her authentic acting skills. Miss Kabovanska sang at the
funeral of her co star Luciano Pavarotti in two thousand
and seven, and she works today still as a professor
in Siena, Italy, as well as at the New Bulgarian
(28:55):
University in Sofia in Bulgaria. Today we hear her in
one of the roles for which she was quite famous,
singing Tosca's Visitiarte from Actu Aupuccini's masterpiece. Thank you so
much for joining me for this three hundred and twenty
eighth episode, and thanks once again to our guest doctor.
Speaker 6 (29:12):
I'mdani. I hope I'll have a good week ahead.
Speaker 5 (30:00):
Oh, sign.
Speaker 7 (30:17):
The chain.
Speaker 5 (30:23):
On, lady. He saw me and nobody saw me sign
for the racle.
Speaker 7 (30:35):
Chains didn't really joined the com Ball.
Speaker 5 (31:32):
Signs. Say ask me a h had you the recy
(32:31):
gol
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