October 17, 2025 • 30 mins
This week we review an episode from 3 years back and delve into the world of adult congenital heart disease when we review a recent work from the ACHD team at UCSF that assesses the impact of BMI on clinical outcomes in the single ventricle adult Fontan patient. What is the cause of elevation in BMI in some Fontan patients? Is obesity the only explanation? Is BMI a modifiable risk factor for our Fontan patients and should exercise be 'prescribed' for these patients? If so prescribed, what type of exercise is best for the Fontan patient? This week's work's senior author, Dr. Anushree Agarwal, Assistant Professor of Medicine at UCSF, shares her insights into this important topic.
DOI: 10.1161/JAHA.122.026732
Also mentioned in today's episode is episode #222 with Dr. Dan Halpern of NYU medical center (https://www.stitcher.com/show/pediheartpediatric-cardiology-today/episode/pediheart-podcast-222-impact-of-cardiac-rehab-on-exercise-tolerance-in-the-achd-patient-206781483)
DOI: 10.1161/JAHA.122.026732
Also mentioned in today's episode is episode #222 with Dr. Dan Halpern of NYU medical center (https://www.stitcher.com/show/pediheartpediatric-cardiology-today/episode/pediheart-podcast-222-impact-of-cardiac-rehab-on-exercise-tolerance-in-the-achd-patient-206781483)
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Transcript
Episode Transcript
Available transcripts are automatically generated. Complete accuracy is not guaranteed.
Speaker 1 (00:00):
Hello everybody. This is Rob Pass, the host of the
(00:01):
Pdhart podcast. This week, we'll be taking a step back
in time two years to hear doctor anu Agarwal of
the University of California in San Francisco speak about the
impact of BMI and outcomes of fontane patients. I'll see
you next week with a brand new episode. Welcome to
(00:34):
Pdhart Pediatric Cardiology Today. My name is doctor Robert Pass
and I'm the host of this podcast. I am Professor
of Pediatrics at the Icon School of Medicine at Mount
SINAI thank you very much for joining me for this
two hundred and forty ninth episode of the podcast. I
hope everybody enjoyed last week's co branded episode with the
SADS Foundation, in which we replayed an episode from two
years ago starring doctor Peter Schwartz, the eminent authority on
(00:57):
long QT syndrome. For those of you with an interest
and channelopathy and long QT syndrome, I definitely recommend you
to take a listen to last week's episode one hundred
and fifty with doctor Peter Schwartz. As I say most weeks,
if you'd like to get in touch with me, it's
easy to remember my email it's Pdheart at gmail dot com.
This week we move on to the world of adult
congenital heart disease as well as single ventricles and the
(01:19):
title of the work we'll be reviewing is Association of
body mass indexed with clinical features and outcomes and Adults
with fontine palliation. The first author of this work is
Yudouche Yogi Swarin and the senior author Anushri Agerwal. And
this work comes to us mostly from the University of
California in San Francisco. After we're done reviewing this paper,
(01:39):
doctor Agerwaal has kindly agreed to speak with us about it. Therefore,
let's get straight onto the article and then a conversation
with its senior author, doctor Nushri Agerwal. This week's work
begins with some comments about the fontane operation, with some
historical comments and reference to the fact that, as is
the case in patients who do not have single ventrical physiology,
the numbers ofts who have obesity is rapidly rising in
(02:02):
the single ventrical community. And though we have a growing
mountain of evidence suggesting the evils of being overweight, for
the non congenital heart population, there is relatively little on
the impact of obesity in the fontan patient, and this
is the rationale for this week's work. The authors speak
of elevations and CVP seen in the fontan patient and
how so many studies have demonstrated the enhanced risks associated
(02:25):
with a fontan circulation, such as a rhythmia, heart failure,
cirrhosis of the liver, thromboembolism, pl and even death, and
they note that many of these factors are only marginally
modifiable and are true results of the circulation itself. They
mention how there is growing interest in modifiable risk factors
such as obesity and physical activity and how these may
(02:47):
positively affect varied cardiovascar conditions, and though there is substantial
evidence for the impact of these factors on acquired cardiovascer disease,
the impact on congenital cardiovascar disease is less well established.
The authors stated and I quote that further understanding of
the association between elevated BMI and fontane related morbidity will
(03:08):
provide important information to clinicians about managing this complex patient population.
With this as a background, the authors chose to perform
a retrospective study of their adult fontane patients with a
goal of assessing the impact of BMI on clinical characteristics
and outcomes, with a hypothesis that elevation in the BMI
would be associated with an increased prevalence of adverse outcomes
(03:30):
in the fontane adult patient. This was a retrospective cross
sectional study of all adult patients greater than are equal
to eighteen years of age who were survivors of single
ventrical physiology and fontan managed at UCSF's AHD program over
a nearly twenty year period from January one, two thousand
to July first, twenty nineteen. All sorts of data about
(03:53):
these patients was obtained, including all the anatomical detail, the
physiological details, all testing results, and the type of fontanine
as well. They looked at New York Heart Association classifications
and many many details, with the most important for this
work being anthropomorphic data such as heightened weight. In order
that BMI was obtained, again, all sorts of data, including
(04:13):
CPET data and echo data were obtained for this work.
Adverse clinical outcomes were defined as arrhythmia, pacemaker or ICD implantation,
liver cirrhosis, pl heart failure, hospitalization, thrombombolic complications or death cirrhosis.
For this work was defined as imaging suggestive of this
(04:34):
diagnosis with or without portal hypertension, splendi, megaly ascietes of
varises and all to the results. There were a total
of one hundred and ninety adult fontane patients in this work,
though twenty seven were removed as BMI data was not
available on these patients, and so the cohort studied had
one hundred and sixty three patients with a mean age
of twenty nine point nine years with a BMI of
(04:56):
twenty four but with thirty seven percent or a bit
more than the third having a BMI over twenty five
kilograms per meter squared. The underlying conditions were trecuspitetresion thirty
six percent double left ventricle and twenty percent, hypoplastic left
heart syndrome in eighteen percent double outlid righte ventricle and
eight percent, pulmonary treasure with intact septim and six percent,
(05:18):
and then a smattering of other patients with things like
heterotaxi syndrome or Ebstein's. The medium time since fontane was
twenty eight years, with a mean age at fontine being
six point six years. It's interesting that patients with a
BMI over twenty five kilograms per meter squared were older
and had a higher prevalence of depression and anxiety. And
what were the main findings? Well, there are a few,
(05:40):
but I would certainly encourage that all read the paper.
But here are some of the more important ones. First,
on multivariable analysis, for every standard deviation increase in BMI,
there was an independent increase in fontane pressure and wedge pressure,
meaning that BMI directly correlated with worse human dynamic measurements. Second,
for every standard deviation increase in BMI, there was a
(06:03):
decrease in PK two consumption. On univariate analysis, Third, and
perhaps most important, BMI over twenty five was independently associated
with heart failure hospitalization with an adjusted ODDS ratio of
ten point two, as well as thromboembola complications with an
adjusted ODDS ratio of two point seventy nine. In their discussion,
(06:24):
the author state and I quote our study of one
hundred and sixty three adult patients with font hanne at
a tertiary center demonstrated the association of elevated BMI with
unfavorable hemo dynamics and adverse clinical outcomes. One in three
patients with font hanne had a BMI over twenty five
kilograms per meter squared, and an increase in BMI was
(06:45):
associated with significantly lower exercise capacity and after adjustments for covariance,
with higher font hane and wedge pressures, fontan patients who
had a BMI over twenty five kilograms per meter squared
had a higher prevalence of heart failure, hospitals Z and
thrombonebalic complications. The authors state that this is the first
study in fontane patients showing that obesity was associated with
(07:08):
fontane failure and heart failure, and they re emphasize how
many of the patients in this group thirty seven percent
had a BMI over twenty five. They suggest that the
weight gain being seen in these adults starts in childhood
and wonder if efforts to address this would be helpful
in childhood. They suggest that exercise prescription for this patient
(07:28):
group would be very important. They mentioned that obesities association
with worse outcomes means that it could be viewed as
a modifiable risk factor for the fontane patient and also
might represent a Heman dynamic problem for this patient group.
They remind us that elevated weight in BMI is associated
in all patients with elevations in in diystolic pressure, and
how this could be particularly diletarious in a single vetrical patient.
(07:53):
The authors also review the finding that there was an
elevated risk for thromboonbala complications in this patient group and
explain that obesity is a systemic inflammatory condition that may
in itself activate pro thrombotic signaling, and given the already
well known enhance stress for thrombosis in this patient group,
this would clearly be potentially dangerous. The authors also note
(08:15):
of the association of obesity in this group and anxiety
or depression and how these factors need to be addressed,
which is a common theme we've explored previously on the
podcast in all congenital heart disease patients, and so the
authors conclude in this tertiary care center experience of adults
with Fontanne palliation, elevated BMI was significantly associated with poor
(08:36):
human dynamics such as higher fontane and filling pressures and
poor clinical outcomes such as heart failure, hospitalization, and thrombo
mbolic complications. This suggests that elevated BMI could be a
consequence of poor outcomes among adults with fontanne palliation, but
needs to be further established in prospective cohort studies. Clinicians
managing this complex group of patients should be vigilant about
(08:59):
this association because it could impact their evaluation and management
of adults with fontine palliation.
Speaker 2 (09:06):
Well.
Speaker 1 (09:06):
I think this is clearly an important work. It would
seem obvious that obesity is bad for a fontane patient
as it is bad for nonfontan patients, and the importance
of maintaining a healthy BMI with the incorporation of aerobic
and muscular strength training seems to be rising in all
of our consciousness daily. This is a concept we discussed
with doctor Dan Halpern of NYU just a few months
(09:28):
ago on episode two hundred and twenty two when he
spoke about the impact of rehabilitation on the ACHD patient.
I think the clear importance of this work is the
demonstration that this growing epidemic of obesity is particularly bad
for our fontane patients, and I am increasingly convinced that
consideration of having a formalized exercise prescriptive program for these
(09:48):
patients is crucial, though I admittedly am unsure how this
might work. Though some may wish to hear about doctor
Halpern's episode in this and it's reviewed in episode two
hundred and twenty two, which I'll linked to in the
show notes. In any event, this work clearly sounds the
alarm for all of us caring for single metrical patients
who are overweight. At this point, I think we should
(10:09):
move forward to speak with the works author. Doctor Agawah,
a New Shri Agurwah is a cardiologist who specializes in
non invasive cardiology and adult congenital heart disease, including taking
care of women with heart disease during childbearing years. Doctor
Agerwal's research interests include eco cardiography, pregnancy and heart disease,
and adult congenital heart disease. Doctor Agawal earned her medical
(10:31):
degree from the Indira Gandhi Government Medical College and Hospital,
and she completed a residency in internal medicine at the
Southern Illinois University School of Medicine. She then completed a
fellowship in cardiology at Aurora Healthcare, which is affiliated with
the University of Wisconsin School of medicine and public health.
She also completed a fellowship in Adult congenital Cardiology at UCSF.
(10:52):
It is indeed a great pleasure and honor to have
her join us this week on the podcast. Welcome doctor Aguwal.
Speaker 3 (10:57):
I'm here now at doctor anu Agarwal, who's the senior
author of this week's work. Doctor Arkawahal, thank you so
much for joining us this week on the podcast.
Speaker 4 (11:05):
Doctor pas It's my pleasure. I'm nice to be here
and talk about our paper.
Speaker 3 (11:10):
Thank you, Thank you very much, doctor Argowahll, very interesting
and important work. Congratulations to you and all of your
co investigators. Elevated BMI seemed very strongly associated with bad
outcomes in the fontane adult patient in your work. But
one thing that struck me was or a question that
came to me was what do you think came first?
(11:31):
Was it elevated BMI or the heart failure that we
often see in our fontane patients. Is this the chicken
or the egg.
Speaker 4 (11:38):
That's a really great question and that's exactly the question
that came to our mind as we were doing this research.
You know, based on austerdy we found that elevated BMI
was associated with variables that suggest fontent failure or heart
failure hospital heart failure hospitalization in these patients, such as
higher fontaine pressures, high permonary vocabulary wedge pressures, and higher
(12:03):
heart failure hospitalizations. In fact, we found that patients with
elevated BMI had ten fold higher odds of heart failure
hospitalizations in those with BMI less than twenty five kilogram
perimeter square. However, it is difficult to really determine whether
obesity is the cause or the marker of heart failure
in this patients, especially due to the cross section design
(12:26):
of our study, and we need more research to explore
this question further. But it's also important, in my opinion
to note that defining healthy weight status using BMI in
this population is really challenging because of the high prevalence
of lean mass deficiency, which can mask the presence of
(12:46):
increase fat mass when BMI is used as a surrogate
for adiposity in these patients. Therefore, in reference measures of
lean and fat masses, lightly better markers define obesity. I
also wanted to mention it's important to consider the role
of physical activity in these patients. You know, historically many
(13:09):
patients fontan patients have been cautioned against moderate through vigorous
physical activity, which could then lead to further adiposity as
they grow older. Moreover, there is increasing evidence that there
is significant elevations of insulin resistance among font tent patients
compared to controls, and this insulent resistance continues to increase
(13:32):
with age. So it's you know, it's not yet clear
whether this incident resistance is due to impaired glucose metabolism
related to their FONT and associated liver disease, or as
there is anotherteology. But you know, there are many factors
as we saw, that could contribute to adipocity and lower
skeleton mass in our font tent population. And ariposity, as
(13:55):
we know, is very well known to be associated with
structural changes in the heart and adverse effects on human
dynamics and any person, not only FONT tematis. So, in summary,
while ME and MI may be associated with heart failure outcomes,
if we need more research to determine the causal relationship
we BMI and heart failure, and then we need most
(14:16):
ready to define the healthy weight status in this population.
Speaker 3 (14:19):
Well, I will tell you one thing. You know, a
lot of FONT hand patients will tell you that they
were cautioned against exercise. I have to be honest, I've
been practicing for twenty five years. I have never known
any cardiologists to recommend that to any font AT patients.
So while it might be true thirty or forty years ago,
I do believe that that is something that FONTAN patients
(14:41):
tell their adult congenital providers. But I am not at
all convinced that any pediatric cardiologists ever tell the font
HAD patient that they were not able to exercise. I
just wanted to set that point out there. Now that
I'm a little older, I could tell you I was
there at the beginning. My brain is still working well
enough that I remember what happens on five years ago,
and for certain I don't think that that's true. But
(15:04):
there are, of course many many reasons why FOTTANT patients
are not very physically active on average, which have nothing
at all to do with their desire to be active.
But anyway, I just thought just mentioned that.
Speaker 4 (15:20):
I totally agree with you, and I person agree with that,
and I also think there is some element of fear
patients can they exercise that.
Speaker 3 (15:30):
I definitely believe yes, and I can tell you I've
even seen this. When I've tried to do stress tests,
on metabolic stress tests, I've had the experience more than
once where a family member at the stress test wanted
to stop the study because they were concerned. So I
do think there is some truth to the fact that
(15:51):
people have told font tant patients not to exercise, but
I don't actually think it's coming from the medical professionals.
I think it's probably more from their families, To be
honest with you, at least that's been my experience. You know,
I was wondering, how do you deal with the elevated
be at my adult adult congenital patient. I know that,
as you just very nicely explained to us, this isn't
(16:12):
only about obesity, but I think obesity clearly plays a
role in a good percentage of these patients. We on
the podcast had Dan Halpern a couple of months ago,
and he talked with us from NYU about having the
wonderful resources of the russ Institute with a rehab center
right there affiliated with his program. Do you have an
(16:33):
associated exercise program for your adult fontine patients? Do you
have dietitians on call for you for these patients? How
do you practically make interventions in this issue.
Speaker 4 (16:45):
Yeah, that's great, and I feel like more and more
centers should incorporate this amazing option of having a dedicated
exercise and IDTY program. We currently do not have a
dedicated exercise program just for our FONT time patient population. Personally,
I spent a significant amount of time with my all
(17:06):
my ADHD patients who are bees, counseling them extensively on lifestyle.
But you know, it is critical as we talked about
in the FONT and patients more so. And you know,
there is there is significant research that have shown, as
we know, that exercise training is most effective non invasive
(17:27):
therapy for improving aerobic capacity. And and I think the
and I think the most important aspect I really counsel
patients on is the resistance training because increasing the muscle
mass is so important for our quontent patients, like just
mentioned earlier, how they struggle with low early mass. So
(17:49):
you know, I uh, just like you do, doctor Pass,
I also often do an exercise test testing, especially the
cardiopulmonary exercise test testing, mainly to assess my PAGs fitness
levels and reassure them and then also use that to
motivate them to stay active because, as I mentioned, a
lot of medicis have concerns that they don't want to
stress their heart, yes, you know, in terms of it
(18:12):
by exercising. So this is this, This has become a
very important part of my counseling. Again, as I mentioned,
resistance training is important to I, you know, usually tell
them to do it as much as possible on a
regular basis four to five times a week at least,
if not at least two times a week. You know,
I just like, really, yeah, create that a lot in
(18:34):
my counseling, in addition to the aerobic exercises.
Speaker 3 (18:39):
It's interesting. It's interesting because I've been reading this book
called Outlive by the author Peter Attia, who's very into
longevity and quality of life, and he makes the argument
that resistance training is very under represented in recommendations and
that it's his belief, I think, based on his reading
(18:59):
of the letters, that for people who do not have
font Hanes and that that probably consistent forms of this
type of isometric exercise is particularly important in terms of
maintaining functionality. And I think probably and we all know
that Jack Rerichick for a number of years now has
been recommending particularly lower extremity strength training in our Fontante patient.
(19:22):
So that's very much in line with what you've just
told this doctor Agwall, So yeah, I certainly agree. You know,
one of the things that we're a little frightening in
your work was that throm bombola complications were more common
in those who had an elevated BMI and were a
font hande. Can you share with the audience what your
thoughts are on the mechanism for this and also if
you do anything differently in regards to anti coagulation in
(19:45):
a high bmi Fontanne patient versus let's say a normal
weight or a normal bmi Fontanne patient.
Speaker 4 (19:53):
Yeah, and that's that was a bit of surprise for
us as well. Although you know, if you think of
the underlying mechanism, not that I can be exactly sure,
but we know that obesity is known to be associated
with thrombosis, and this has been extensively studied in in
previous literature in case of the Fontaine patients. In addition
(20:15):
to the prothrombotic effect of adiposity and obesity by itself,
there is you know, there is other factors that contribute,
such as you know, stasis of blood flow, prosthetic material
that these patients have was arrhythmias and then the chronic
you know, this adds to the chronic informatory state of
(20:36):
the obesity to activate the protraumatics signaling, and I think
the combination could likely increase the traumatlinic risks in these patients.
You know, in terms of anti cooigalation, it is it
is very hard to counsel patients or you know, even
(20:57):
recommend patients stuggling on anti cooigualation just based on their
BMI and also just based on this one's sturdy because
you know, we have to take into a consideration the
risks us benefits, and I think we need more sturdies
to really determine whether the risks of anti coagulation outweighs
or outrage the benefits in other words, or you know,
some kind of a risk going system that includes BMI
(21:20):
in these patients. Right now, as you know, anti coagulation
is a class one ACC recommendations and fontine population. But
that is also a level of evidence c which is
expert opinion among patients who have a rhythm as an
you know, previous wrong membody complications. So I think we
need some risk modeling to understand whether this is important
(21:40):
but more important as we talked about as counseling.
Speaker 3 (21:43):
Yeah, I definitely think that idea of coming up with
a risk model would be very useful in managing these
patients because we still I don't think have the definitive
answer on this. Well, for those in the audience, it's
right in the middle of doctor Agerworld's day actually is
probably in the beginning of her day, in the middle
of my day. So I'm gonna wrap this up with
(22:04):
one final question. So you, of course are an adult
congenital specialist, see lots and lots of adults who have
congel heart disease. A lot of adult fonts hands not
as involved obviously with pediatric patients before they reach your
transition clinic. But what kind of interventions would you suggest
to us as pediatric cardiologists in pediatric clinic prior to
(22:28):
reaching ADHD transition to maybe stave off or mitigate this
issue in adulthood.
Speaker 4 (22:38):
You know, I think pediatric cardiologists have done an amazing
job in our front and population with like you know,
the surgeries and counseling and all of this is I'm
nobody to suggest pediatric carriology. But I think the routine
counseling that goes on with exercise, I think the I
think just stressing patients and like you said, you know,
(22:59):
maybe identifying if they have fears related to their exercise
capacity and motivating them more and more. And I think
we all know that exercise is the most non invisive
and the best known effective intervention, not only for the
mortality benefits, but also quality of life. And you know
that I think sometimes has become so important for our
(23:21):
patients when we counsel them on how it could improve
your day to day well be yeah, you know, whether
it's mental, physical, or just you know this quality of
life measures in general.
Speaker 3 (23:32):
So yeah, I think I guess it's the same battle
all doctors have with their patients, and maybe the same
battle we have with ourselves and reminding themselves the exercise
we do the right things. Well, doctor Aghawalla, I want
to congratulate you again and all of your co investigators,
and I'm very appreciative that you're spending a few minutes
(23:54):
with us this week to discuss your paper. Thank you
so much.
Speaker 4 (23:57):
Thank you for having me here.
Speaker 3 (23:59):
Great pleasure.
Speaker 2 (24:00):
Thank you.
Speaker 1 (24:00):
Well, I think you'll agree that doctor Ackerwall very nicely
fleshed out her paper for us and offered to us
a number of intriguing ideas regarding why the BMI of
these patients was elevated and how it might not always
be due to obesity. I thought her comments on the
reasons for thrombombola complications and elevated BMI patients to be
of interest, and also her thoughts on how we might
(24:22):
one day figure out what sort of anti coagulation is
best for the fontine patient. Once again this week, I think,
as we discussed in past episodes, we've again learned of
the potent impact of exercise for all patients with congenital
heart disease, and I am increasingly convinced of the extremely
important role for this in our patients. This may prove
(24:43):
important enough to warrant true exercise prescription for our patients,
an investment in a full time resource for this patient group,
which is something that I can certainly imagine, may very
well be more common, as it is increasingly clear that
both strength training and cartivacer exercise may have similar, if
not more potent impact on our ADHD patients than any
(25:04):
lycenteprel tablet or Dosa ferosa mind. I am most appreciative
to doctor Aguaal for spending time with us this week
on the podcast. To conclude this replay episode, we hear
the wonderful Mongolian operatic baritone a Martushin Ankbot singing the
todayum at the end of the first act of Puccini's
Tosca in a live performance from just a few years ago.
(25:27):
Mister Ankbat was born and raised in Mongolia and studied
opera initially at the State University of Arts and Culture
in Mongolia. Following this, he has won many different awards
and operatic singing competitions, with one of the most notable
being the Dame Jones Sutherland Audience Prize in twenty fifteen
at the BBC Cardiff Singer of the World competition, and
(25:48):
more recently, in twenty twenty four he won the Premio
et Torre Bastianini Award. And I'm sure you'll understand well
why when you hear him in this live performance. Thank
you for listening to this week's repat Play episode and
thanks once more to doctor oker Wall. I'll see you
next week with a brand new.
Speaker 5 (26:05):
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ah hush.
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Be christ Watcher say, I'm talking is so and looking disus.
Speaker 2 (27:16):
Something a sparty, a h spot.
Speaker 7 (27:34):
I'll be ding lost in anybody in the Cappings, Yeah.
Speaker 2 (28:09):
Sh I guess you, Scar.
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Candy s
Speaker 3 (29:09):
S st
Hello everybody. This is Rob Pass, the host of the
(00:01):
Pdhart podcast. This week, we'll be taking a step back
in time two years to hear doctor anu Agarwal of
the University of California in San Francisco speak about the
impact of BMI and outcomes of fontane patients. I'll see
you next week with a brand new episode. Welcome to
(00:34):
Pdhart Pediatric Cardiology Today. My name is doctor Robert Pass
and I'm the host of this podcast. I am Professor
of Pediatrics at the Icon School of Medicine at Mount
SINAI thank you very much for joining me for this
two hundred and forty ninth episode of the podcast. I
hope everybody enjoyed last week's co branded episode with the
SADS Foundation, in which we replayed an episode from two
years ago starring doctor Peter Schwartz, the eminent authority on
(00:57):
long QT syndrome. For those of you with an interest
and channelopathy and long QT syndrome, I definitely recommend you
to take a listen to last week's episode one hundred
and fifty with doctor Peter Schwartz. As I say most weeks,
if you'd like to get in touch with me, it's
easy to remember my email it's Pdheart at gmail dot com.
This week we move on to the world of adult
congenital heart disease as well as single ventricles and the
(01:19):
title of the work we'll be reviewing is Association of
body mass indexed with clinical features and outcomes and Adults
with fontine palliation. The first author of this work is
Yudouche Yogi Swarin and the senior author Anushri Agerwal. And
this work comes to us mostly from the University of
California in San Francisco. After we're done reviewing this paper,
(01:39):
doctor Agerwaal has kindly agreed to speak with us about it. Therefore,
let's get straight onto the article and then a conversation
with its senior author, doctor Nushri Agerwal. This week's work
begins with some comments about the fontane operation, with some
historical comments and reference to the fact that, as is
the case in patients who do not have single ventrical physiology,
the numbers ofts who have obesity is rapidly rising in
(02:02):
the single ventrical community. And though we have a growing
mountain of evidence suggesting the evils of being overweight, for
the non congenital heart population, there is relatively little on
the impact of obesity in the fontan patient, and this
is the rationale for this week's work. The authors speak
of elevations and CVP seen in the fontan patient and
how so many studies have demonstrated the enhanced risks associated
(02:25):
with a fontan circulation, such as a rhythmia, heart failure,
cirrhosis of the liver, thromboembolism, pl and even death, and
they note that many of these factors are only marginally
modifiable and are true results of the circulation itself. They
mention how there is growing interest in modifiable risk factors
such as obesity and physical activity and how these may
(02:47):
positively affect varied cardiovascar conditions, and though there is substantial
evidence for the impact of these factors on acquired cardiovascer disease,
the impact on congenital cardiovascar disease is less well established.
The authors stated and I quote that further understanding of
the association between elevated BMI and fontane related morbidity will
(03:08):
provide important information to clinicians about managing this complex patient population.
With this as a background, the authors chose to perform
a retrospective study of their adult fontane patients with a
goal of assessing the impact of BMI on clinical characteristics
and outcomes, with a hypothesis that elevation in the BMI
would be associated with an increased prevalence of adverse outcomes
(03:30):
in the fontane adult patient. This was a retrospective cross
sectional study of all adult patients greater than are equal
to eighteen years of age who were survivors of single
ventrical physiology and fontan managed at UCSF's AHD program over
a nearly twenty year period from January one, two thousand
to July first, twenty nineteen. All sorts of data about
(03:53):
these patients was obtained, including all the anatomical detail, the
physiological details, all testing results, and the type of fontanine
as well. They looked at New York Heart Association classifications
and many many details, with the most important for this
work being anthropomorphic data such as heightened weight. In order
that BMI was obtained, again, all sorts of data, including
(04:13):
CPET data and echo data were obtained for this work.
Adverse clinical outcomes were defined as arrhythmia, pacemaker or ICD implantation,
liver cirrhosis, pl heart failure, hospitalization, thrombombolic complications or death cirrhosis.
For this work was defined as imaging suggestive of this
(04:34):
diagnosis with or without portal hypertension, splendi, megaly ascietes of
varises and all to the results. There were a total
of one hundred and ninety adult fontane patients in this work,
though twenty seven were removed as BMI data was not
available on these patients, and so the cohort studied had
one hundred and sixty three patients with a mean age
of twenty nine point nine years with a BMI of
(04:56):
twenty four but with thirty seven percent or a bit
more than the third having a BMI over twenty five
kilograms per meter squared. The underlying conditions were trecuspitetresion thirty
six percent double left ventricle and twenty percent, hypoplastic left
heart syndrome in eighteen percent double outlid righte ventricle and
eight percent, pulmonary treasure with intact septim and six percent,
(05:18):
and then a smattering of other patients with things like
heterotaxi syndrome or Ebstein's. The medium time since fontane was
twenty eight years, with a mean age at fontine being
six point six years. It's interesting that patients with a
BMI over twenty five kilograms per meter squared were older
and had a higher prevalence of depression and anxiety. And
what were the main findings? Well, there are a few,
(05:40):
but I would certainly encourage that all read the paper.
But here are some of the more important ones. First,
on multivariable analysis, for every standard deviation increase in BMI,
there was an independent increase in fontane pressure and wedge pressure,
meaning that BMI directly correlated with worse human dynamic measurements. Second,
for every standard deviation increase in BMI, there was a
(06:03):
decrease in PK two consumption. On univariate analysis, Third, and
perhaps most important, BMI over twenty five was independently associated
with heart failure hospitalization with an adjusted ODDS ratio of
ten point two, as well as thromboembola complications with an
adjusted ODDS ratio of two point seventy nine. In their discussion,
(06:24):
the author state and I quote our study of one
hundred and sixty three adult patients with font hanne at
a tertiary center demonstrated the association of elevated BMI with
unfavorable hemo dynamics and adverse clinical outcomes. One in three
patients with font hanne had a BMI over twenty five
kilograms per meter squared, and an increase in BMI was
(06:45):
associated with significantly lower exercise capacity and after adjustments for covariance,
with higher font hane and wedge pressures, fontan patients who
had a BMI over twenty five kilograms per meter squared
had a higher prevalence of heart failure, hospitals Z and
thrombonebalic complications. The authors state that this is the first
study in fontane patients showing that obesity was associated with
(07:08):
fontane failure and heart failure, and they re emphasize how
many of the patients in this group thirty seven percent
had a BMI over twenty five. They suggest that the
weight gain being seen in these adults starts in childhood
and wonder if efforts to address this would be helpful
in childhood. They suggest that exercise prescription for this patient
(07:28):
group would be very important. They mentioned that obesities association
with worse outcomes means that it could be viewed as
a modifiable risk factor for the fontane patient and also
might represent a Heman dynamic problem for this patient group.
They remind us that elevated weight in BMI is associated
in all patients with elevations in in diystolic pressure, and
how this could be particularly diletarious in a single vetrical patient.
(07:53):
The authors also review the finding that there was an
elevated risk for thromboonbala complications in this patient group and
explain that obesity is a systemic inflammatory condition that may
in itself activate pro thrombotic signaling, and given the already
well known enhance stress for thrombosis in this patient group,
this would clearly be potentially dangerous. The authors also note
(08:15):
of the association of obesity in this group and anxiety
or depression and how these factors need to be addressed,
which is a common theme we've explored previously on the
podcast in all congenital heart disease patients, and so the
authors conclude in this tertiary care center experience of adults
with Fontanne palliation, elevated BMI was significantly associated with poor
(08:36):
human dynamics such as higher fontane and filling pressures and
poor clinical outcomes such as heart failure, hospitalization, and thrombo
mbolic complications. This suggests that elevated BMI could be a
consequence of poor outcomes among adults with fontanne palliation, but
needs to be further established in prospective cohort studies. Clinicians
managing this complex group of patients should be vigilant about
(08:59):
this association because it could impact their evaluation and management
of adults with fontine palliation.
Speaker 2 (09:06):
Well.
Speaker 1 (09:06):
I think this is clearly an important work. It would
seem obvious that obesity is bad for a fontane patient
as it is bad for nonfontan patients, and the importance
of maintaining a healthy BMI with the incorporation of aerobic
and muscular strength training seems to be rising in all
of our consciousness daily. This is a concept we discussed
with doctor Dan Halpern of NYU just a few months
(09:28):
ago on episode two hundred and twenty two when he
spoke about the impact of rehabilitation on the ACHD patient.
I think the clear importance of this work is the
demonstration that this growing epidemic of obesity is particularly bad
for our fontane patients, and I am increasingly convinced that
consideration of having a formalized exercise prescriptive program for these
(09:48):
patients is crucial, though I admittedly am unsure how this
might work. Though some may wish to hear about doctor
Halpern's episode in this and it's reviewed in episode two
hundred and twenty two, which I'll linked to in the
show notes. In any event, this work clearly sounds the
alarm for all of us caring for single metrical patients
who are overweight. At this point, I think we should
(10:09):
move forward to speak with the works author. Doctor Agawah,
a New Shri Agurwah is a cardiologist who specializes in
non invasive cardiology and adult congenital heart disease, including taking
care of women with heart disease during childbearing years. Doctor
Agerwal's research interests include eco cardiography, pregnancy and heart disease,
and adult congenital heart disease. Doctor Agawal earned her medical
(10:31):
degree from the Indira Gandhi Government Medical College and Hospital,
and she completed a residency in internal medicine at the
Southern Illinois University School of Medicine. She then completed a
fellowship in cardiology at Aurora Healthcare, which is affiliated with
the University of Wisconsin School of medicine and public health.
She also completed a fellowship in Adult congenital Cardiology at UCSF.
(10:52):
It is indeed a great pleasure and honor to have
her join us this week on the podcast. Welcome doctor Aguwal.
Speaker 3 (10:57):
I'm here now at doctor anu Agarwal, who's the senior
author of this week's work. Doctor Arkawahal, thank you so
much for joining us this week on the podcast.
Speaker 4 (11:05):
Doctor pas It's my pleasure. I'm nice to be here
and talk about our paper.
Speaker 3 (11:10):
Thank you, Thank you very much, doctor Argowahll, very interesting
and important work. Congratulations to you and all of your
co investigators. Elevated BMI seemed very strongly associated with bad
outcomes in the fontane adult patient in your work. But
one thing that struck me was or a question that
came to me was what do you think came first?
(11:31):
Was it elevated BMI or the heart failure that we
often see in our fontane patients. Is this the chicken
or the egg.
Speaker 4 (11:38):
That's a really great question and that's exactly the question
that came to our mind as we were doing this research.
You know, based on austerdy we found that elevated BMI
was associated with variables that suggest fontent failure or heart
failure hospital heart failure hospitalization in these patients, such as
higher fontaine pressures, high permonary vocabulary wedge pressures, and higher
(12:03):
heart failure hospitalizations. In fact, we found that patients with
elevated BMI had ten fold higher odds of heart failure
hospitalizations in those with BMI less than twenty five kilogram
perimeter square. However, it is difficult to really determine whether
obesity is the cause or the marker of heart failure
in this patients, especially due to the cross section design
(12:26):
of our study, and we need more research to explore
this question further. But it's also important, in my opinion
to note that defining healthy weight status using BMI in
this population is really challenging because of the high prevalence
of lean mass deficiency, which can mask the presence of
(12:46):
increase fat mass when BMI is used as a surrogate
for adiposity in these patients. Therefore, in reference measures of
lean and fat masses, lightly better markers define obesity. I
also wanted to mention it's important to consider the role
of physical activity in these patients. You know, historically many
(13:09):
patients fontan patients have been cautioned against moderate through vigorous
physical activity, which could then lead to further adiposity as
they grow older. Moreover, there is increasing evidence that there
is significant elevations of insulin resistance among font tent patients
compared to controls, and this insulent resistance continues to increase
(13:32):
with age. So it's you know, it's not yet clear
whether this incident resistance is due to impaired glucose metabolism
related to their FONT and associated liver disease, or as
there is anotherteology. But you know, there are many factors
as we saw, that could contribute to adipocity and lower
skeleton mass in our font tent population. And ariposity, as
(13:55):
we know, is very well known to be associated with
structural changes in the heart and adverse effects on human
dynamics and any person, not only FONT tematis. So, in summary,
while ME and MI may be associated with heart failure outcomes,
if we need more research to determine the causal relationship
we BMI and heart failure, and then we need most
(14:16):
ready to define the healthy weight status in this population.
Speaker 3 (14:19):
Well, I will tell you one thing. You know, a
lot of FONT hand patients will tell you that they
were cautioned against exercise. I have to be honest, I've
been practicing for twenty five years. I have never known
any cardiologists to recommend that to any font AT patients.
So while it might be true thirty or forty years ago,
I do believe that that is something that FONTAN patients
(14:41):
tell their adult congenital providers. But I am not at
all convinced that any pediatric cardiologists ever tell the font
HAD patient that they were not able to exercise. I
just wanted to set that point out there. Now that
I'm a little older, I could tell you I was
there at the beginning. My brain is still working well
enough that I remember what happens on five years ago,
and for certain I don't think that that's true. But
(15:04):
there are, of course many many reasons why FOTTANT patients
are not very physically active on average, which have nothing
at all to do with their desire to be active.
But anyway, I just thought just mentioned that.
Speaker 4 (15:20):
I totally agree with you, and I person agree with that,
and I also think there is some element of fear
patients can they exercise that.
Speaker 3 (15:30):
I definitely believe yes, and I can tell you I've
even seen this. When I've tried to do stress tests,
on metabolic stress tests, I've had the experience more than
once where a family member at the stress test wanted
to stop the study because they were concerned. So I
do think there is some truth to the fact that
(15:51):
people have told font tant patients not to exercise, but
I don't actually think it's coming from the medical professionals.
I think it's probably more from their families, To be
honest with you, at least that's been my experience. You know,
I was wondering, how do you deal with the elevated
be at my adult adult congenital patient. I know that,
as you just very nicely explained to us, this isn't
(16:12):
only about obesity, but I think obesity clearly plays a
role in a good percentage of these patients. We on
the podcast had Dan Halpern a couple of months ago,
and he talked with us from NYU about having the
wonderful resources of the russ Institute with a rehab center
right there affiliated with his program. Do you have an
(16:33):
associated exercise program for your adult fontine patients? Do you
have dietitians on call for you for these patients? How
do you practically make interventions in this issue.
Speaker 4 (16:45):
Yeah, that's great, and I feel like more and more
centers should incorporate this amazing option of having a dedicated
exercise and IDTY program. We currently do not have a
dedicated exercise program just for our FONT time patient population. Personally,
I spent a significant amount of time with my all
(17:06):
my ADHD patients who are bees, counseling them extensively on lifestyle.
But you know, it is critical as we talked about
in the FONT and patients more so. And you know,
there is there is significant research that have shown, as
we know, that exercise training is most effective non invasive
(17:27):
therapy for improving aerobic capacity. And and I think the
and I think the most important aspect I really counsel
patients on is the resistance training because increasing the muscle
mass is so important for our quontent patients, like just
mentioned earlier, how they struggle with low early mass. So
(17:49):
you know, I uh, just like you do, doctor Pass,
I also often do an exercise test testing, especially the
cardiopulmonary exercise test testing, mainly to assess my PAGs fitness
levels and reassure them and then also use that to
motivate them to stay active because, as I mentioned, a
lot of medicis have concerns that they don't want to
stress their heart, yes, you know, in terms of it
(18:12):
by exercising. So this is this, This has become a
very important part of my counseling. Again, as I mentioned,
resistance training is important to I, you know, usually tell
them to do it as much as possible on a
regular basis four to five times a week at least,
if not at least two times a week. You know,
I just like, really, yeah, create that a lot in
(18:34):
my counseling, in addition to the aerobic exercises.
Speaker 3 (18:39):
It's interesting. It's interesting because I've been reading this book
called Outlive by the author Peter Attia, who's very into
longevity and quality of life, and he makes the argument
that resistance training is very under represented in recommendations and
that it's his belief, I think, based on his reading
(18:59):
of the letters, that for people who do not have
font Hanes and that that probably consistent forms of this
type of isometric exercise is particularly important in terms of
maintaining functionality. And I think probably and we all know
that Jack Rerichick for a number of years now has
been recommending particularly lower extremity strength training in our Fontante patient.
(19:22):
So that's very much in line with what you've just
told this doctor Agwall, So yeah, I certainly agree. You know,
one of the things that we're a little frightening in
your work was that throm bombola complications were more common
in those who had an elevated BMI and were a
font hande. Can you share with the audience what your
thoughts are on the mechanism for this and also if
you do anything differently in regards to anti coagulation in
(19:45):
a high bmi Fontanne patient versus let's say a normal
weight or a normal bmi Fontanne patient.
Speaker 4 (19:53):
Yeah, and that's that was a bit of surprise for
us as well. Although you know, if you think of
the underlying mechanism, not that I can be exactly sure,
but we know that obesity is known to be associated
with thrombosis, and this has been extensively studied in in
previous literature in case of the Fontaine patients. In addition
(20:15):
to the prothrombotic effect of adiposity and obesity by itself,
there is you know, there is other factors that contribute,
such as you know, stasis of blood flow, prosthetic material
that these patients have was arrhythmias and then the chronic
you know, this adds to the chronic informatory state of
(20:36):
the obesity to activate the protraumatics signaling, and I think
the combination could likely increase the traumatlinic risks in these patients.
You know, in terms of anti cooigalation, it is it
is very hard to counsel patients or you know, even
(20:57):
recommend patients stuggling on anti cooigualation just based on their
BMI and also just based on this one's sturdy because
you know, we have to take into a consideration the
risks us benefits, and I think we need more sturdies
to really determine whether the risks of anti coagulation outweighs
or outrage the benefits in other words, or you know,
some kind of a risk going system that includes BMI
(21:20):
in these patients. Right now, as you know, anti coagulation
is a class one ACC recommendations and fontine population. But
that is also a level of evidence c which is
expert opinion among patients who have a rhythm as an
you know, previous wrong membody complications. So I think we
need some risk modeling to understand whether this is important
(21:40):
but more important as we talked about as counseling.
Speaker 3 (21:43):
Yeah, I definitely think that idea of coming up with
a risk model would be very useful in managing these
patients because we still I don't think have the definitive
answer on this. Well, for those in the audience, it's
right in the middle of doctor Agerworld's day actually is
probably in the beginning of her day, in the middle
of my day. So I'm gonna wrap this up with
(22:04):
one final question. So you, of course are an adult
congenital specialist, see lots and lots of adults who have
congel heart disease. A lot of adult fonts hands not
as involved obviously with pediatric patients before they reach your
transition clinic. But what kind of interventions would you suggest
to us as pediatric cardiologists in pediatric clinic prior to
(22:28):
reaching ADHD transition to maybe stave off or mitigate this
issue in adulthood.
Speaker 4 (22:38):
You know, I think pediatric cardiologists have done an amazing
job in our front and population with like you know,
the surgeries and counseling and all of this is I'm
nobody to suggest pediatric carriology. But I think the routine
counseling that goes on with exercise, I think the I
think just stressing patients and like you said, you know,
(22:59):
maybe identifying if they have fears related to their exercise
capacity and motivating them more and more. And I think
we all know that exercise is the most non invisive
and the best known effective intervention, not only for the
mortality benefits, but also quality of life. And you know
that I think sometimes has become so important for our
(23:21):
patients when we counsel them on how it could improve
your day to day well be yeah, you know, whether
it's mental, physical, or just you know this quality of
life measures in general.
Speaker 3 (23:32):
So yeah, I think I guess it's the same battle
all doctors have with their patients, and maybe the same
battle we have with ourselves and reminding themselves the exercise
we do the right things. Well, doctor Aghawalla, I want
to congratulate you again and all of your co investigators,
and I'm very appreciative that you're spending a few minutes
(23:54):
with us this week to discuss your paper. Thank you
so much.
Speaker 4 (23:57):
Thank you for having me here.
Speaker 3 (23:59):
Great pleasure.
Speaker 2 (24:00):
Thank you.
Speaker 1 (24:00):
Well, I think you'll agree that doctor Ackerwall very nicely
fleshed out her paper for us and offered to us
a number of intriguing ideas regarding why the BMI of
these patients was elevated and how it might not always
be due to obesity. I thought her comments on the
reasons for thrombombola complications and elevated BMI patients to be
of interest, and also her thoughts on how we might
(24:22):
one day figure out what sort of anti coagulation is
best for the fontine patient. Once again this week, I think,
as we discussed in past episodes, we've again learned of
the potent impact of exercise for all patients with congenital
heart disease, and I am increasingly convinced of the extremely
important role for this in our patients. This may prove
(24:43):
important enough to warrant true exercise prescription for our patients,
an investment in a full time resource for this patient group,
which is something that I can certainly imagine, may very
well be more common, as it is increasingly clear that
both strength training and cartivacer exercise may have similar, if
not more potent impact on our ADHD patients than any
(25:04):
lycenteprel tablet or Dosa ferosa mind. I am most appreciative
to doctor Aguaal for spending time with us this week
on the podcast. To conclude this replay episode, we hear
the wonderful Mongolian operatic baritone a Martushin Ankbot singing the
todayum at the end of the first act of Puccini's
Tosca in a live performance from just a few years ago.
(25:27):
Mister Ankbat was born and raised in Mongolia and studied
opera initially at the State University of Arts and Culture
in Mongolia. Following this, he has won many different awards
and operatic singing competitions, with one of the most notable
being the Dame Jones Sutherland Audience Prize in twenty fifteen
at the BBC Cardiff Singer of the World competition, and
(25:48):
more recently, in twenty twenty four he won the Premio
et Torre Bastianini Award. And I'm sure you'll understand well
why when you hear him in this live performance. Thank
you for listening to this week's repat Play episode and
thanks once more to doctor oker Wall. I'll see you
next week with a brand new.
Speaker 5 (26:05):
Episode on miss as a lush name for sunny they
(26:37):
ah hush.
Speaker 6 (26:51):
Be christ Watcher say, I'm talking is so and looking disus.
Speaker 2 (27:16):
Something a sparty, a h spot.
Speaker 7 (27:34):
I'll be ding lost in anybody in the Cappings, Yeah.
Speaker 2 (28:09):
Sh I guess you, Scar.
Speaker 7 (28:33):
Candy s
Speaker 3 (29:09):
S st
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