September 5, 2025 • 51 mins
This week we review a landmark paper that came out this month on the topic of ACHD. How common is neurocognitive dysfunction amongst the ACHD patient population? What factors are associated with worse or better such outcomes? What interventions should be considered to mitigate these issues? What is the relationship between mental illness and neurocognitive dysfunction? Dr. Scott Cohen of The Medical College of Wisconsin and Dr. Ali Zaidi of The Icahn School of Medicine at Mount Sinai share their insights into their recent multicenter study.
doi: 10.1016/j.jacc.2025.06.051
Also featured after the article is a brief description of the upcoming Mount Sinai ACHD Conference called: Kawasaki Disease Across The Ages: From Childhood Onset To Adult Outcomes - The 4th Mount SInai ACHD Symposium. We speak with Dr. Nadine Choueiter who is the Course Director of this conference about the goals and objectives of the conference. Dr. Zaidi is the course co-director. For those interested in signing up, please go to this webpage:
https://physicians.mountsinai.org/news/register-today-for-the-fourth-annual-mount-sinai-adult-congenital-heart-disease-symposium
doi: 10.1016/j.jacc.2025.06.051
Also featured after the article is a brief description of the upcoming Mount Sinai ACHD Conference called: Kawasaki Disease Across The Ages: From Childhood Onset To Adult Outcomes - The 4th Mount SInai ACHD Symposium. We speak with Dr. Nadine Choueiter who is the Course Director of this conference about the goals and objectives of the conference. Dr. Zaidi is the course co-director. For those interested in signing up, please go to this webpage:
https://physicians.mountsinai.org/news/register-today-for-the-fourth-annual-mount-sinai-adult-congenital-heart-disease-symposium
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Transcript
Episode Transcript
Available transcripts are automatically generated. Complete accuracy is not guaranteed.
Speaker 1 (00:16):
Welcome to PD Heart 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 here in New York City. Thank
you for joining me for this three hundred and fifty
fourth episode of p D Heart. I hope everybody enjoyed
last week's extraordinary episode, in which we spoke with the
wonderful doctor Terry King about his life in times, specifically
(00:40):
the time surrounding his landmark intervention of closing an ASD
and a human for the first time. For those of
you interested in the history of medicine as well as mentorship,
i'd certainly recommend you take a listen to last week's
episode three hundred and fifty three live from the PICKS
meeting in Chicago. As I say most weeks, if you'd
like to get in touch with me, my email is
(01:00):
easy to remember. It's Pdheart at gmail dot com. This
week we venture into the world of adult congeneral heart disease.
The title of the work we'll be reviewing is Prevalence
and Predictors of Neurocognitive Dysfunction in Adults with congenital heart
disease results from the Mind's ADHD study. The first author
of this work is Scott Cohne and the senior author
(01:21):
Ali Zadi, and this work comes to us from the
PHN and aar CC investigators. Doctor Zaidi comes to us
from the Department of Medicine and Pediatrics at the Icon
School of Medicine here at Mount Sinai, where he is
the director of the Adult Congenital Program, and doctor Scott
Cone comes from the Department of Medicine and Pediatrics at
the Medical College of Wisconsin in Milwaukee, Wisconsin. When we're
(01:44):
done reviewing this landmark paper, doctors Cone and doctor Zadi
have graciously agreed to speak with me about it. Therefore,
let's move straight onto this article and then a conversation
with doctor Cone and doctor Zaidi. The work starts with
comments about the large and growing population of adult conj
general heart disease patients and how roughly three hundred thousand
adults in the United States have what would be categorized
(02:06):
as moderate or complex congeneral heart disease, and the authors
also mention the present evidence showing that both children and
adults with congeneral heart disease, have unique neurodevelopmental patterns that
are associated with higher than usual rates of psychiatric disorders,
as well as patterns that may negatively impact upon neurocognitive
abilities and what the author's term self management skills. They
(02:30):
then review how there is substantial evidence of neurocognitive issues
affecting children with congenital heart disease and how these negatively
impact on quality of life when these patients reach adulthood.
They comment on how some will need educational or psychosocial
support and how this will potentially affect education, employment, insurance coverage,
and quality of life in the adult congenital patient population.
(02:54):
The authors then remind that on average, adult congeneral heart
disease patients are at an enhanced risk for comorbidity like
atrial fibrillation, hypertension, or diabetes, and they explain that all
of these factors are associated with higher rates of mild
cognitive impairment or dementia in patients who do not have
congenital heart disease. They then bemoan the absence of specific
(03:14):
guidelines for screening or treatment of neurocognitive defects in adults
with congenital heart disease, but They suggest that timely identification
and referral for treatment or support services might prevent care gaps,
which might improve quality of life and possibly reduce mortality
and morbidity in the adult congenital population. With this as
a background, this study, the so called Multi Institutional Neurocognitive
(03:37):
Discovery Study and Adult Congenital Heart Disease or MINDS ADHD study,
aim to assess neurocognitive functioning in young adults with moderate
to severely complex congenital heart disease in order to better
understand the scope of this problem by identifying the prevalence
of neurocognitive disorders and the need for cognitive remediation programs.
(03:59):
They state that the primary aim of the study was
to investigate neurocognitive functioning of eighteen to thirty year olds
with moderate to severe complexity congenital heart disease, with the
first hypothesis being that these adults would score lower than
a standardization sample on the so called NIH Toolbox Cognition battery,
and the second hypothesis was that the Adult Congenital Heart
(04:20):
Disease cohort would have lower neuroquality of life Item Bank
Version two cognitive function scores than the standardized sample. A
secondary aim of this work was to explore the association
of neurocognitive dysfunction with demographic and clinical factors. This was
a multi center, cross sectional pilot study of individuals aged
eighteen to thirty years with moderate to severely complex congenital
(04:43):
heart disease and was conducted via the phn NHLBI in
alliance with the Adult Research and Congeneral Cardiology Group and
involved fourteen ADHD centers in North America. And this study
was conducted from October twenty twenty to May of twenty
twenty Freves. The participants had to complete questionnaires and provide
biospecimens during a single visit or within twelve months of
(05:06):
a clinic visit. And the authors review the many inclusion
and exclusion criteria and I would encourage that listeners take
a look at the paper with the link in the
show notes. Patients who had known genetic syndromes were not
excluded unless they met other exclusion criteria. The authors review
the NIH toolbox cognitive battery and what it includes and
the validation for it that was used in this study.
(05:27):
They also created a neuro quality of life instrument with
twenty eight questions to self report neurocognitive function including memory, attention,
decision making, and daily task performance. If NIH toolbox respondents
fell one or more standard deviations from the normal population mean,
indicating possible neurocognitive problems, Recommendations were made for further clinical
(05:50):
evaluation of cognitive symptoms to the different minds AHD sites.
Tons of clinical and demographic data were obtained, including details
of the medical history as well as things like age, sex,
educational level, race, ethnicity, occupation, and socioeconomic status, amongst many others,
and DAHNTA the results. Initially, there were nearly twenty six
(06:11):
hundred patients eligible for this work in the fourteen participating centers,
and twenty one hundred and thirty were approached and of
these five hundred and one consented to participate, so basically
less than twenty five percent of eligible patients. And of
these five hundred and one, four hundred and sixty seven
participants completed all the study procedures and so were the
(06:32):
focus of this work. The patients were grouped into four
main groups. Detransposition of the Great Arteries, of which there
were eighty two participants, to Trilogy of philoh of which
there were one hundred and twenty three participants, single ventricle
patients one hundred and thirty two participants, and other moderate
or severe congenital heart disease one hundred and thirty participants.
(06:52):
When looking at the demographics, the average age was about
twenty five years, with fifty six percent being women, eighty
one percent white, and s seventy eight percent were in
New York Heart Association Functional Class one. The four groups
of patients were demographically the same, with the only difference
being that the DTGA group had a higher proportion of
men at fifty two percent. Almost one quarter of the
(07:14):
group were in school, with thirty nine percent having a
college degree or postgraduate degree. The single ventrical group was
different than the other congenital heart disease groups in that
there was a higher proportion of high school or lower
grade level achievement thirty three percent. Fifty five percent of
the group were employed full time, with sixteen point five
percent part time, and sixty five percent of the group
(07:36):
were single. There were a number of comorbidities in These
included hypertension in nine point six percent, diabetes in one
point three percent, congestive heart failure in five percent, seizures
in three point six percent, atrial or ventricular arrhythmiasm, seventeen percent,
and importantly thirty three percent were obese, and these findings
(07:56):
were similar in the four groups. The prevalences of mental
health disorders were similar between the four groups as well.
When looking at the neurocognitive findings using the objective NIH
toolbox testing, thirty four percent of the participants scored more
than or equal to one standard deviation below the normative mean,
with the score of forty four in comparison to fifty
(08:18):
for the normative mean. The mean cognitive function score was
significantly lower in tetrology, single ventrical and other moderate to
severe congel heart disease populations, but not so in the
DTGA group. The lowest scores were seen in the single
ventrical group and highest in the DTGA patients. Among the
single ventrical patients, fully forty one percent scored at least
(08:40):
one standard deviation below the mean, followed by tetrology of
below in which thirty seven percent were below one standard
deviation from the mean, and the mixed group of thirty
two percent, and finally the DTGA group at twenty percent
Figure one on page six hundred and forty six of
this document nicely demonstrates the cognitive function compass. It scores
(09:01):
and shows graphically how tetrology, single ventrical and other moderate
to severe coan joal heart disease patients scored statistically significantly
below the normal average, whereas the DTGA patients were in
the normal range. Using the subjective neuroquality of life item
bank testing, twenty two percent scored more than are equal
to one standard deviation below normative levels? And what were
(09:24):
the predictors of dysfunction? In the objective nih toolbox testing?
On multivariable analysis, the worst performance was linked to a
lower New York Heart Association functional class, with lower being
worse and lower educational attainment. When looking at the more
subjective neuroquality of life testing, worse outcomes were associated with
lower educational level, higher BMI, presence of atrial arrhythmias, and
(09:48):
symptoms of depression and anxiety. In their discussion, the author's
state and I quote our findings indicate that objective neurocognitive
dysfunction was identified in nearly one third or thre thirty
four percent of the cohort, correlating most strongly with level
of education. Subjective neurocognitive dysfunction was observed in one fifth,
(10:08):
or twenty two percent of the cohort, also linked to
educational level, but negatively associated with BMI atrilarrhythmias and underlying
psychiatric conditions such as depression and anxiety. These deficits may
persist throughout the lifespan and affect various neurocognitive subdomains, including
executive functioning, information processing, psychomotor speed, reaction time, attention, fine
(10:33):
motor skills, working memory, and visiospatial abilities, particularly in individuals with
complex congenital heart disease. If I were to summarize the
main points of their discussion, it would be a restating
of this being the largest such study assessing objective and
subjective neurocognitive dysfunction in adults congenital heart disease patients. They
use these data to suggest that there is a need
(10:56):
to integrate neurocognitive assessments into routine care for the achd
PA patient and wonder if early detection of these issues
can help guide the development of clinical guidelines and targeted
interventions to improve outcomes in quality of life. The authors
speak of the overrepresentation of neurocognitive dysfunction amongst single ventrical patients,
contrasting this finding to the DTGA patients, in whom the
(11:18):
prevalence of these problems was similar to the general population.
They suggest that this may be because of the rapid
establishment of normal cardiovascular hemodynamics in transposition patients in contrast
to single ventrical patients who are exposed to syinosis long term,
as well as heart failure and arrhythmias. A lot of
discussion about quality of life is made in this work,
(11:39):
trying to parse out what the factors are to explain
it and how neurocognitive dysfunction can play a role. Lower employment,
for example, is common amongst ADHD patients, and they explain
how this fact can be affected by so many different factors,
with educational achievement being an important one that is clearly
lower in the ADHD patient group, perhaps due to the
(12:00):
neurocognitive dysfunction described in this work. However, is this an
example of a situation of the chicken in the egg,
which problem causes which effect. They do posit that if
more focus on educational planning were done earlier in children's
lives with congel heart disease, perhaps this might be an
opportunity to improve later achievement and overall quality of life
(12:20):
in the ADHD patient group. The author's comment on the
relation of subjective measures of neurocognitive dysfunction and anxiety and
depression and feel that this should highlight the vital role
of mental health in the overall health of the ADHD
patient and the potential critical role for mental health professionals
and caring for this group of patients. They bemoan the
insufficient allocation of resources to address neurocognitive problems in this
(12:45):
patient group, which coupled with societal stigma in addressing neurocognitive
dysfunction associated disabilities, means that this is indeed a very
hard problem to address because of the complex and frankly
expensive solutions that that are required. Feel that this work
sounds the alarm for the need for dedicated resources to
screen ACHD patients for neurocognitive dysfunction because of the profound
(13:09):
impact neurocognitive dysfunction can have an overall health and quality
of life, and they again speak about how important they
believe early identification and referral would be to try and
meaningfully improve outcomes in this regard. The authors speak of
the limitations of this work, including what could be a
substantial sampling bias, given that less than one quarter of
patients queried actually participated in the study and the fact
(13:31):
that more than eighty percent were white, with the predominance
of females and more educated New York Heart Class Association
IE patience. All of these may significantly affect generalizability of
these data to all adult congenital patients. The authors also
point to some limitations of the NIH toolbox test that
was used, as well as the neuro Quality of Life test,
(13:54):
and so they conclude our findings reveal that objective neurocognitive
dysfunction we're identified in one third or thirty four percent
of the cohort, showing a correlation with education and New
York Heart Association functional classification. Similarly, subjective neurocognitive dysfunction were
present in one fifth or twenty two percent of the participants,
(14:16):
with links to educational level and negative associations with atril
arrhythmias BMI and underlying psychiatric conditions such as depression and anxiety.
These results underscore the critical need for integrating neurocognitive assessments
into the routine care of adults with congenital heart disease.
By identifying these deficits early, we can inform clinical practice
(14:40):
guidelines aimed at proactive detection and management of neurocognitive dysfunction.
Addressing these challenges paves the way for targeted interventions that
can not only improve cognitive outcomes, but also improve the
overall quality of life for ADHD patients. Accompanying this article
as an editorial comment by doctors shaf Pavandi and ROBERTA.
(15:01):
Williams from UCSF and USC, respectively. The authors of this
commentary review the major findings of the work that we
have just reviewed. They emphasize that this work has demonstrated
a very high burden of neurocognitive defects or dysfunction, more
than one third, and how this means that in the
US alone, we're speaking of over one hundred thousand patients
(15:22):
with these problems, and they suggest to use their terminology
that these findings are jarring. The author's comment on the
finding of educational level being a major factor in the
multi variable model for both objective and subjective cognitive measures,
and they state that this quote begs the question of
which came first poor neurocognitive function making it harder to
(15:43):
succeed in school? Or poor school participation and persistence driving
worse neurocognitive outcomes, and they suggest that the work cannot,
unfortunately ferret out this relationship. They re emphasize the paper's
demonstration that mental health problems were highly associated with neurocos
cognitive dysfunction, and again re emphasize the important role that
(16:03):
mental health support should play in the care of the
adult congenital patient. The authors essentially feel that this work
is a call to action and stress the need for
embedding neurodevelopmental and mental health care early in childhood and
extending this throughout adulthood for patients with significant congenital heart
disease so that, in the author's words, our patients can
(16:24):
achieve their full potential in life.
Speaker 2 (16:27):
Well.
Speaker 1 (16:27):
This is clearly an interesting and important work demonstrating the
very high prevalence of neurocognitive dysfunction amongst ADHD patients with
moderate to severe congenital heart disease. There are a number
of methodological limitations of this work and biases that might
be skewing these data, and most of these patients were
born in the twentieth century, and so it might be
hard to say if these data reflect what a baby
(16:49):
today will be like in twenty more years. But certainly,
as doctors Paivandi and William's right, this is indeed a
call to action not only for the mental health and
neurocognit and of support of ADHD patients, but also pediatric patients.
How this would practically work in pediatric cardiology practice and
how it would be paid for are questions that are
(17:10):
perhaps above this podcast or pediatric cardiologists pay grade. But
certainly this work puts into stark relief the critical nature
of these problems and really should leave us all wondering
how we can intercede to improve our patients' lives. To me,
this work is saying that we should no longer view
ourselves only as people who save lives, but also as
(17:31):
doctors and care providers who wish to improve lives. How
do we do that is likely something that will become
more clear in the coming decades based on data from
works like this week's. In the interest of time, I
think we'll move forward to our conversation with the work's
authors Jennings now to discuss this week's work is its
first and senior author, Doctor Scott Cohene is an ACHD
(17:53):
specialist at the University of Wisconsin. He received his medical
degree from Saint Louis University School of Medicine and completed
rests and fellowship in cardiology at Brown University. Following these,
he went on to perform an ADHD fellowship at the
Children's Hospital of Wisconsin and he is an ADHD specialist
both at Children's Wisconsin and also Freudtert Hospital. Doctor Ali
(18:15):
Zadi is a graduate of the Aga Khan University in Karachi, Pakistan.
He completed his combined internship and residency in internal medicine
and pediatrics at the Penn State University Hershey Medical Center.
He did chief residency in internal medicine at Penn State
before pursuing cardiology training at Columbus, Ohio as well as
adult congenital training. He is a professor of pediatrics and
(18:37):
Medicine at Mount Sinai where he is the director of
the adultkin General Heart Disease Center at Mount Sinai as
well as a co director of our Children's Hearts Center.
It is a delight and pleasure to welcome my friend
doctor Zaidi and doctor Cohne to Pediheart. Welcome doctor Cohne
and doctor Zaidi. I'm here now with doctor Scott Cone
and doctor Ali Zaidi. Guys, thank you very much for
(18:57):
joining me this week on Pdheart.
Speaker 3 (18:59):
Yeah, thanks for the really, thanks Rob, thanks for having us.
Speaker 1 (19:02):
Real pleasure. Really enjoyed it. You know, I'll leave before
we get going. I'm wondering if you could share with us, like,
how did you come to this idea of doing this
project and what exactly did it involve? It seemed like
it was extremely involved, So how did it get going?
Speaker 3 (19:19):
Yeah?
Speaker 2 (19:19):
Thanks, thanks Rob and Scott, thanks for being here. So
so Rob, this started back if you believe it, in
twenty and seventeen or somewhere right around there. It was
I'll take just thirty seconds to explain how this came along.
It was quite honestly all of us in ADHD, and
it was Scott. It was Scott's idea to sort of
look at neurocognitive functioning and just just sort of see,
(19:41):
you know, what's happening in our clinics, you know, sort
of do some sort of some sort of testing. And
it turned out that there was no valid sort of
questionnaire that we could just pull out of our pockets
in our clinics and say, hey, listen, let's let's look
at neurocognitive functioning and AHD. That's how started in twenty
seventeen and Quantoti, we got ARC involved, which is this
(20:04):
large consortium of ADHD clinical investigators, and then we went
to the PHN because we needed funding, and thankfully the
PHN funded it through the NHLBI and we got enough
funding to then use fourteen.
Speaker 3 (20:19):
Centers across North America.
Speaker 2 (20:20):
That was back in twenty and seventeen and eighteen, and
we started looking at this. The way we did this
rob was actually it was a learning experience for all
of us. What we'd learned was that we can't just
really test neurocognitive function in our piic.
Speaker 3 (20:34):
We didn't know how.
Speaker 2 (20:36):
And what we found out was that we had to
involve neuropsychologists that were two part of Minds that were
housed as a core at MCW and they helped us
sort of define which questionnaires needed to be done.
Speaker 3 (20:50):
They gave us those questionnaires and then.
Speaker 2 (20:52):
We did this over three years and we recruited five
hundred patients over when we started, I think was twenty twenty.
Speaker 3 (21:01):
I think that's when we started re suiting patients right YEP.
Speaker 2 (21:04):
And then COVID happened, and we pivoted through that, and
then we went through that and we ended up getting
five hundred patients. It took quite a bit, quite a
bit of effort.
Speaker 3 (21:13):
So that's how we that's how we got here.
Speaker 1 (21:16):
And so the folks, the neuropsychologists in Wisconsin had identified
the appropriate questions or a questionnaire to be used. Is
that how that works? Gone?
Speaker 4 (21:27):
Yeah?
Speaker 5 (21:27):
Correct, So we wanted to assess both objective and subjective
neurocontent functioning, and they helped us identify which questionnaires we
could use. For objective functioning, we use the NH Toolbox
and for the subjective neurocontent functioning, we used the NEUROQOL survey.
Speaker 1 (21:49):
I see right, Well, thank you for that. That's very interesting.
So we're really looking at the efforts of a very
long effort, and congratulations again.
Speaker 3 (21:57):
To both of you on that.
Speaker 1 (21:59):
Just the stick to it miss alone is worthy of congratulations. Scott,
since you're the first author, I wondered, you know, sometimes
I'll start these interviews off and ask the authors of
the work if maybe they could summarize what they believe,
maybe are the four or five most important takeaways from
this work this week? Maybe you could share your insights
into it.
Speaker 3 (22:20):
Yeah. Sure.
Speaker 5 (22:20):
So, as Ali mentioned, we ended up recruiting nearly five
hundred patients for this study. In nationality, it came out
to be four hundred and sixty seven patients with moderate
or severely complex congeneral heart disease age between eighteen and
thirty years across the fourteen sites.
Speaker 3 (22:40):
In the US and Canada.
Speaker 5 (22:42):
We chose this age range because we really were to
focus in on the nervokognitive function in young adults at
the stage of transition where they're going from pediatric to
adult healthcare, where they may be leaving to go to
college or starting a new job, new relationships. And what
we found is and the total cohort, thirty four percent
(23:03):
of these patients scored at least one Stanard deviation below
the normative mean on our objective neurocognctive function. Twenty two
percent scored at least one Seren deviation below the normative
mean for the subjective neuroconnactive function. We found that educational
level and NYHA classification were both independent predictors of objective
(23:27):
neurocognitive function and educational level BMI history of atrilarrhythmias and
history of mental health disorders such as anxiety and depression
were independent predictors of subjective neuroconnactive function. And the last
thing I want to call attention to is just the
characteristics of our cohort.
Speaker 3 (23:47):
During screening.
Speaker 5 (23:48):
We had about more than half of the patients we
approached for consent actually declined consenting for our study, and
so we came out with a population that were very
high functioning, well educated individuals. Nearly eighty percent of our
cohort where NYHA Class one nearly eighty percent had at
(24:08):
least some college experience or an associate degree, Nearly seventy
percent worth either full or part time employed. And so
we are actually likely under estimating the true prevalence of
neurocogncin functioning in our adult in general heart disease population.
Speaker 1 (24:26):
Wow, that's a very important point. So this is really
the best case scenario basically, So what you're.
Speaker 3 (24:32):
Saying, yeah, yeah, I think So that's a.
Speaker 1 (24:34):
Little disappointing, I guess, But as a doctor Pavante suggested,
this is a call to action. So I'm wondering, Scott.
You know, educational achievement, as you mentioned, a moment ago
was highly associated with neurocognitive dysfunction, with lower levels of
education being associated with worse levels of function. In this
(24:57):
regard the office of the editorial, I'll ask this question,
and so I thought I would ask you which came first,
the neurocognitive dysfunction or the lower educational achievement. I mean,
is that even the right question to ask, like which
is causing which or what's their relationship there?
Speaker 5 (25:14):
Yeah, I think that's a really tough question that may
not be able to answer. Based on our cross sectional study,
there's clearly a bi directional relationship between neurocogctive function and
educational attainment, such that those with higher neurocogctant function will
likely reach a higher level of educational attainment. But we
(25:35):
could also think that those that have access to better,
higher quality education may also be able to have improved
neurocognctive function. I think that this relationship is very complicated
and probably future longitudinal studies are needed to better answer
that the question of what that relationship means looking at
(25:58):
whether more or better higher educational attainment can actually improve
neuroconcert function. But at this point in time, I think
the best thing we can do is really focus on
improving access to high quality education for our young conngenital
heart disease patients and really optimizing their educational attainment by
(26:19):
developing appropriate IEPs, assessing the need for accommodations in middle
school and high school, and then really encouraging these patients
to continue the awareness of what they needed and what
kind of combinations they needed in middle school and high
school into their next stage of education, whether that be
college or vocational school or beyond.
Speaker 3 (26:40):
Yeah.
Speaker 2 (26:41):
Wrong than just jumping real quick, I think you hit
the nail on the head as the editorial, because it's
really the question is the chicken and the egg, right
which comes first?
Speaker 3 (26:50):
It's and it's tricky.
Speaker 2 (26:53):
It's very tricky because in essence, we are showing a
bi directional relationship, so that you know, if you have
a bad neural, are going to functioning that bad education?
Or is it bad education leading to the others. So
it is a very complex relationship, and as Scott said,
I think we need to delve into this maybe perhaps
even in the earlier years, you know, not in this
(27:13):
school ward per se.
Speaker 1 (27:15):
Well, you know, it's interesting the patient group you guys
studied were relatively young ADHD patients by intention, As you
explained Scott eighteen to thirty. You know, it's interesting. Doctor
Gilbernowski posted on LinkedIn when I wrote that we were
going to be discussing this this week, and he said,
(27:37):
let's remember all of these patients or the majority, were
born twenty or thirty years ago, and he liked he
said they were ball born in the twentieth century, trying
to make it seem like we're very advanced today. I'm
wondering if you think that the findings that we're finding
that you've identified today in the ADHD young population are
(27:58):
going to be applicable to children and who are infants
today twenty years from now.
Speaker 3 (28:03):
Yeah, that's a good question.
Speaker 5 (28:05):
I think the study, the mind study, reflects the longitudinal
experiences that our cohort had over their eighteen to thirty
years of their lifetime and identified risk factors of objective
and subjective neuroconnactive deficits within those experiences. But for today's infants,
I think there's been achievements and advancements and care models
(28:27):
that may lead to better neuroconnactive outcomes for our young
adult cogeneral heartsease patients.
Speaker 3 (28:34):
So I think.
Speaker 5 (28:35):
There's a growing awareness of neurodevelopmental outcomes and children with
congenital heart disease and that continued issue with neuroconnactive deficits
and our ADHD patients. This awareness, I think, was demonstrated
in a updated American Heart Association scientific statement Neurodevelopmental Outcomes
and Individuals with Congenital Heart Disease that was published by
(28:56):
Erica Sud and others just last year.
Speaker 3 (28:59):
This statement gave.
Speaker 5 (28:59):
Up updates on topics such as risk assessment for a
poor neural developmental outcomes, potential genetic basis or perinatal factors
that impact neural developmental outcomes, and neuroprotective strategies at times
of surgery for kids born with congole heart disease. So
I think, overall, with continued collaborative research in the pediatric
(29:21):
cardiology realm and continued awareness of this issue as it
also moves into adults living with congenital heart disease, my
hope is that we'll see better neurocognitive function in our
young ADHD patients.
Speaker 3 (29:32):
In twenty years.
Speaker 1 (29:33):
Yeah. Well, I certainly think that the efforts of all
of those investigators, as well as the editorialist, doctor Pivandi,
is at the forefront of this. Doctor Jane Neuberger and
many others certainly give us cause for optimism. You know,
I'm wondering if you guys might be able to speak
about the relationship of mental illness to neurocardiac disfunction cocognitive
(29:55):
dysfunction and how should we think about this, Ali, I
know this is a particular interest of yours.
Speaker 2 (30:00):
Yes, yeah, thanks, thanks Robin and it Scott. Thanks for
taking the question to mental health.
Speaker 3 (30:06):
Rob It was.
Speaker 2 (30:07):
It was fascinating because we did look at a questionnaire
looking at mental health, both anxiety and depression, and we
looked across the board and what we found was that
approximately one third of this cohort was had sort of
scored in the mental health spectrum which means either anxiety
or depression. And that's one third, that's about thirty three
(30:27):
thirty five percent of patients. And what we showed was
a clear association with one quality of life and two
it was an independent predictor for subjective neurocognitive functioning. So
if you had anxiety or depression, that was an independent
predictor for subjective neurocognitive functioning.
Speaker 3 (30:44):
So again that makes you.
Speaker 2 (30:45):
Go back and take a look and say, well, hang on,
displays a big part in neurocognition mental health play is
a big part in it. And then the next question
that comes into our mind as we all look at it.
These kids are growing up and they're growing up with
in essence anxiety, PTSD and things along those lines. So
what can we do better? This is one where Rob
(31:07):
we're actually going to do a sub study from the
Mind's cohort and we're going to delve into this even
more and try to see what other factors we can find.
That's one and two. Can we then try to identify
resources that can sort of mitigate this early on so
that we can then help from a neurocognitive functioning standpoint,
(31:28):
So I e. Catch them when they're eight, nine, ten,
eleven years old, mitigate the anxiety as much as we can,
and then see what happens down Thereaw, and now can
we prove this from minds it's a cross sexual study.
We cannot, but I think that's sort of the next
step to take.
Speaker 1 (31:44):
I see, well, both of you guys are also proud
pediatric cardiologists, So I'm going to ask you to put
on your pediatric hats here and ask you know, Scott,
you hinted at this earlier, maybe Ali, you want to
take this question. You know, what's stuff could pediatric cardiology
providers take to interfere with this process of neurocognitive dysfunction.
(32:06):
You just hinted at it, Ali in terms of the
role that we could have in managing mental health problems.
You know what's needed in the early years to improve
the sequelite of this problem that you've identified. I've always
been interested that the adult congenital clinics have psychological and
psychiatric support sort of embedded in them, but we don't
(32:29):
do that at all in pediatric cardiology are very minimally so,
at least routinely. So I'm wondering what what sort of
recommendations do you think you would you would make.
Speaker 2 (32:40):
I can take the first part of this, and that's
got I'm going to take you. Let you let you
take the next part of this. But so, Robbie're absolutely correct.
I mean, this is a spectrum that sort of stems
from pediatric crdology and sort of goes into adult in
genital and quite honestly, there is there is a there's
a there's a big body of work that is Jane
Neuberger Gilbernowski. You said, Michelle Gerbertz, who is a co
(33:01):
investigator of Fowers on this study. Michelle has done some
great work from Boston Children's too, and all the folks
that I mentioned that several more in the country have
worked on this, and what they're trying to say is
that can you catch this earlier on? Is there a
way you can actually do the screening in these kids
when they're younger?
Speaker 3 (33:21):
And there are actually some.
Speaker 2 (33:22):
Scientific statements that have come out from the AHA, again
led by doctor Newberger and doctor gil Bernowski, saying that
if you catch it early, can you mitigate it?
Speaker 3 (33:34):
Now?
Speaker 2 (33:34):
What does that mean? Does that mean mitigation from either
or techniques? Is there something that needs to be done
early on from a developmental standpoint?
Speaker 3 (33:42):
So all those things come into play. That's early on.
Speaker 2 (33:46):
As a pediatric cardiologist, we're getting them when they are
in their twenties, So can we do this and can
we mitigate this by the time they get in their twenties?
So I think so there are techniques in their ways.
There's Scott if you want to add to that. The
other part Rob that I'm going to add is that
we did not get into the forty fifty sixty year
(34:07):
old cohort there's a whole plethora there that may or
may not be affected by acquired disease spectrums that we
didn't even touch, right, right.
Speaker 5 (34:17):
Yeah, I agree with everything Ali mentioned. I would also
add that a good transition program would be essential to
allow or help lower the risk of gaps and care
as these young adults transition from pediatric care into adult
co general heart disease care, so that us as adult
(34:38):
conjeneral heart disease providers can continue the work that was
started in the pediatric realm, and then emphasizing continued neurocognitive
evaluations and referrals to specialists as needed.
Speaker 6 (34:52):
And I think going back to the statement about ADHD
clinics having mental health or SOL workers embedded in their clinics,
I think there's a fair degree of a spectrum in
terms of how much ADHD clinics actually have social workers
or mental health providers within their centers are dedicated within
(35:14):
their centers. I think there are a fair number of
centers where the social workers often split between ADHD and
other specialties and may not always have the bandwidth to accommodate.
Speaker 3 (35:26):
The ADHD team.
Speaker 5 (35:28):
And I think the same could be said for mental
health providers especially in centers that maybe in rural areas
or in the so called ADHD deserts where there really
aren't any major ADHD centers of excellence within their region.
So I think from an ADHD standpoint, when they get
to us as ADHD providers, I think starting to enact
(35:50):
widespread mental health and neuroconnective screening in the outpatient setting
would be a good first step.
Speaker 3 (35:57):
That's easier said than done.
Speaker 5 (35:58):
Of course, we don't really know exactly what screening tool
would be most efficient from a time perspective or a
sensitivity specificity aspect, but I think having that screening in
some way would be a good first step. And then
if you're going to be screening, you have to know
what resources you have available for those individuals at screen positives.
(36:20):
So for those centers that don't have embedded mental health
providers or neuropsychologists, knowing in the community what other centers
may have mental health providers that you could refer to,
and establishing a good referral program or relationship with those
providers I think would be really necessary. And then one
(36:42):
last thought I had was having a check or a
follow up program for those that you do refer to
mental health providers or neuropsychologists, because a lot of these
patients that have neuroconc at dysfunction may not be able
to easily call and set up a point means for
intake with these providers and may get lost to follow
(37:03):
up even though you've put the referral in. And I
think same could be said for individuals with mental health concerns.
So having a good follow up system and a good
Claudter referral process by all providers would really benefit these patients.
Speaker 1 (37:17):
Those are all very interesting ideas, Scott. It's really even
as simple as administrative support is very important for this, Ali.
Speaker 3 (37:26):
I'm wondering.
Speaker 1 (37:28):
You know, we're already hitting on my final question, which is,
you know, in a perfect world, what else would you
want in an ADHD clinic to address these issues?
Speaker 3 (37:38):
Scott?
Speaker 1 (37:38):
I think really nicely summarized the notion of, you know,
making having someone to check that there's adequate follow through.
You can make all the recommendations in the world if
no one follows them. Any other things that in a
perfect world, Ali, you would like to see routinely in clinics.
Speaker 3 (37:56):
So thank you. You know, there's so much to do here.
Speaker 2 (37:59):
I mean, there's this has been quite a learning experience
for all of us in ADHD, this whole neurocognitive journey.
The two things on highlight, it's kind of alluded to
transition clinic, this, that and the other. But the one
thing I learned is that we can screen and we
can make a diagnosis, and we can come up with
and you know that patients can have neurocognitive dysfunction, but
we then need to get them to a specialist, which
(38:20):
is a neurocognitive or neuropsychologist, somebody who specializes in that realm.
And I'll be honest with you, when we did this,
we actually struggled, even in the fourteenth centers we were
working with if they had dedicated people that we could
then refer these patients to. So that resource structure in
(38:40):
itself is few and far between. So we've just screened,
made a diagnosis, but then the intervention itself is a
resource structure, and that is quite that I think is
going to be a heavy lift. I think that to
your point, I mean, would we want that as part
of ADHD absolulutely, but I think that that is a
(39:01):
key metric.
Speaker 3 (39:02):
I don't know if I have the right answer to
them Rob.
Speaker 2 (39:04):
The one other thing I was going to add to
what your question initially and is as far as pediatric
cardiology goes, if I recall it, I think it's twenty
ten or twenty twelve. There is a scientific statement Jane Neuburger,
Brad Marino, and they've done some work in screening and children.
So I just want the viewers to know that our
cohort was adults. It was all adults, so we really
(39:26):
didn't look into the sort of the pediatric side of them,
although we did have some variables that we looked at.
Speaker 1 (39:34):
Wow, well, obviously it seems like we just hit the
tip of a massive iceberg, and I think there's a
lot more that we are going to need to learn
about this. But your work, extraordinary work, is really a
wonderful first step in at least identifying the problem or
(39:54):
the magnitude of the problem. I want to congratulate you,
doctor Cone, Doctor's for a really wonderful work. Also congratulate
all your many many co investigators, all who work very
hard on this project. And I want to thank you
both for spending time with us this week to discuss
the work on pd heart Great.
Speaker 2 (40:13):
Yeah, thank you, thank you Ron, Thanks for having us,
and a shout out to all the patients for letting
us do this over seven years.
Speaker 3 (40:19):
I mean it's without them it would be not doable.
But thank you absolutely well.
Speaker 1 (40:24):
As I'm fond of saying, when the guest is good,
there really is not much to discuss. Doctors Cone and
Zeti were uncommonly clear and offered a lot of important
context for this week's work, and I think the way
in which they made us all aware of these data
representing the best case scenario is important. As both authors
suggested to us that the bias and ascertainment of subjects
(40:45):
in this work may have actually caused the group to
overemphasize the better moderate to complex congenle hard patients. I
found their comments about ways to potentially intercede and improve
outcomes to be important and thoughtful, and think that doctor
Cohne's comments about the importance of transition in avoiding gaps
in care and resources was particularly important among the many
(41:07):
important comments that both authors shared with us. I'd once
again like to thank doctor Cohne and doctor Zadi for
taking time from their very busy schedules to speak with
us this week about this very important work on Pdhart
Before we get to the end of the podcast, I
wanted to mention a very exciting up and coming conference
that we'll be holding at Mount Sinai entitled the Kawasaki
(41:28):
Disease Across the Ages from Childhood Onset to Adult Outcomes,
the fourth Mount Sinai Adult Congenital Heart Disease Symposium. This
conference will be occurring at Mount Sinai from Friday September twelfth,
eight a m. To Saturday September thirteen, five pm and
will be held at the Leon and Norma Hes Center
for Science and Medicine Davis Auditorium here at Mount Sinai.
(41:51):
This event will bring together leading experts in pediatric and
adult cardiology to address the evolving needs of Kawasaki disease
patients across the lifespan. In order to better understand this
conference and what exciting things will be coming next week,
I've invited one of the course code directors, doctor Nadine Schwader,
who's Professor of Pediatrics here at Mount Sinai, to speak
briefly about it. Therefore, let's move straight on to our
(42:14):
brief conversation with Nadine Schwater. I'm here now with doctor
Nadine Schwader, who is the course director of the upcoming
Kawasaki Disease Across the Ages from Childhood Onset to Adult
Outcomes course here at Mount SINAI. Interestingly enough, our guest
that we just hung up the phone with doctor Zaidi
is the co director of this course. Nadine, Welcome to Pedheart.
Speaker 4 (42:37):
Thank you, Rob, Thank you for having me.
Speaker 1 (42:39):
Great Nadine, I explained to you that I really am
very excited by this upcoming course. I'm wondering if you
could share with the audience what the goals are of
this Kawasaki conference and then maybe share with us some
of the sessions that you're particularly looking forward to.
Speaker 4 (42:56):
Thanks Bob, Well, we're very excited about this meeting the uh.
The meeting plans to bring together world experts in Kawasaki disease,
both pediatric and adult cardiologists, to address how Kawasaki disease
affects patients through their lifetime and the you know, the
(43:17):
aims of the meeting is to bridge pediatric and adult
discipline in this particular disease, because we know that Kawasaki
disease is the leading cause of acquired heart disease and
children in North America and with advanced in therapy, more
of these children are surviving. As these patients transition into adulthood,
(43:37):
adult carreologists are now seeing complications like thrombosis, ischemia, acute
coronary syndrome, and right now is the time to educate
across specialties so we can ensure continuity of care and
optimal outcomes.
Speaker 3 (43:51):
So sessions.
Speaker 4 (43:52):
I'm particularly you know, excited about our sessions that bring
both pediatric and adult careologists and adult con genital heart
disease specialists to sit down and talk about how we
can partner to care for these patients, how corinaty archery
(44:12):
disease and children differ from corinaty archy disease and adults
and etherosculotic disease. We're also interested in learning about the
outcomes of Kasaki disease and adults from in Japan. It's
a large surveillance study that will be presented for the
first time. The data will be presented for the first time.
(44:34):
And finally, we have a live scanning echo workshop on
the next day of the meeting that teaches the attendees
how to image the cordinary arteries by echo in children
with Kawasaki disease, since this is the modality that we
use to determine the extent of the disease and the
(44:55):
corinaty archery involvement.
Speaker 1 (44:57):
Well, I know that traditionally when we ever you have
hands on echo sessions, that's always some of the most
desired and the fun parts of the conference. I'm sure
people are going to really enjoy that. On the second
day of the conference. For those in the audience, I'm
going to leave a link to the sign up page
if you want to attend this conference. And this is
(45:19):
an only in person conference, is that right, Nadine.
Speaker 4 (45:22):
Actually, we do have a virtual option. There's a virtual
registration option for it will be live zoom streaming with
ability for the participants and the attendees to ask questions online.
Yet the workshop will not be streamed live because it's
a hands on workshop.
Speaker 1 (45:43):
Okay, great, thanks for clarifying that. So again, maybe you
could just share once more with the audience, Nadine, when
the conferences and where it is.
Speaker 4 (45:53):
So the conference will be held next week September twelve
to thirteen, at the i Can's School of Medicine at
Mount Sinai in the Davis auditorium at the Health Science Center.
Speaker 1 (46:09):
Okay, great, and again for those interested in this very
exciting conference which is being co hosted by doctor Zaidi,
our adult congenital expert, and of course doctor Schweider, who's
world renowned for her expertise in Kawasaki disease. There'll be
a link in the show notes this week for those
of you who are interested to learn about it, and
I hope some of you will come to Mount Sinai.
(46:31):
It's a wonderful time in the year to visit New
York City. The weather is great, lots of things to
do outside of the conference, so learning and fun altogether. Nadine,
thank you very much for coming on and thank you
also for being a sometimes co host of the podcast.
Speaker 3 (46:45):
It's always great to have you on. Thanks again, Rob,
thank you.
Speaker 1 (46:49):
To conclude this episode of p D Heart Pediatric Cardiology,
today we end with one of the great tenors of
the past one hundred years, the wonderful Luciano Pavarotti. Pavarotti
was born and then Modina, Italy and went on to
have one of the largest and most successful of all
operatic careers, and he is a rarity in the field
and that he was not only loved by opera lovers
(47:10):
in the world, but also by all fans of music
because of his very popular concerts that he performed in
sold out gigantic arenas. Today we hear him in a
performance from over thirty years ago of Larze Liana's e
La Sulita storia from Act two of Chilea's opera. This
opera is rarely performed today, but this particular tana aria
(47:31):
is a favorite of many, and you will hear why
when sung by the master Pavarotti with his lovely tone,
timber and diction. Thank you very much for joining me
for this episode, and thanks once again to our wonderful guests.
I hope I'll have a good week ahead.
Speaker 7 (47:47):
Ela sasco.
Speaker 8 (48:00):
Ah so baa cont so don't let me last a
(48:32):
lot of the oh ny a, but order me reps.
Speaker 2 (49:04):
As on.
Speaker 9 (49:06):
All auto.
Speaker 8 (49:12):
Loma a.
Speaker 9 (49:15):
T what aca.
Speaker 8 (49:36):
This monest.
Speaker 3 (49:41):
Feel said you.
Speaker 7 (49:49):
You all listen, send me labouchital, Yes, said.
Speaker 9 (50:24):
Walk scor.
Speaker 7 (50:40):
I said nynacord on anything.
Speaker 9 (50:54):
Be t don't
Speaker 2 (51:00):
Me
Welcome to PD Heart 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 here in New York City. Thank
you for joining me for this three hundred and fifty
fourth episode of p D Heart. I hope everybody enjoyed
last week's extraordinary episode, in which we spoke with the
wonderful doctor Terry King about his life in times, specifically
(00:40):
the time surrounding his landmark intervention of closing an ASD
and a human for the first time. For those of
you interested in the history of medicine as well as mentorship,
i'd certainly recommend you take a listen to last week's
episode three hundred and fifty three live from the PICKS
meeting in Chicago. As I say most weeks, if you'd
like to get in touch with me, my email is
(01:00):
easy to remember. It's Pdheart at gmail dot com. This
week we venture into the world of adult congeneral heart disease.
The title of the work we'll be reviewing is Prevalence
and Predictors of Neurocognitive Dysfunction in Adults with congenital heart
disease results from the Mind's ADHD study. The first author
of this work is Scott Cohne and the senior author
(01:21):
Ali Zadi, and this work comes to us from the
PHN and aar CC investigators. Doctor Zaidi comes to us
from the Department of Medicine and Pediatrics at the Icon
School of Medicine here at Mount Sinai, where he is
the director of the Adult Congenital Program, and doctor Scott
Cone comes from the Department of Medicine and Pediatrics at
the Medical College of Wisconsin in Milwaukee, Wisconsin. When we're
(01:44):
done reviewing this landmark paper, doctors Cone and doctor Zadi
have graciously agreed to speak with me about it. Therefore,
let's move straight onto this article and then a conversation
with doctor Cone and doctor Zaidi. The work starts with
comments about the large and growing population of adult conj
general heart disease patients and how roughly three hundred thousand
adults in the United States have what would be categorized
(02:06):
as moderate or complex congeneral heart disease, and the authors
also mention the present evidence showing that both children and
adults with congeneral heart disease, have unique neurodevelopmental patterns that
are associated with higher than usual rates of psychiatric disorders,
as well as patterns that may negatively impact upon neurocognitive
abilities and what the author's term self management skills. They
(02:30):
then review how there is substantial evidence of neurocognitive issues
affecting children with congenital heart disease and how these negatively
impact on quality of life when these patients reach adulthood.
They comment on how some will need educational or psychosocial
support and how this will potentially affect education, employment, insurance coverage,
and quality of life in the adult congenital patient population.
(02:54):
The authors then remind that on average, adult congeneral heart
disease patients are at an enhanced risk for comorbidity like
atrial fibrillation, hypertension, or diabetes, and they explain that all
of these factors are associated with higher rates of mild
cognitive impairment or dementia in patients who do not have
congenital heart disease. They then bemoan the absence of specific
(03:14):
guidelines for screening or treatment of neurocognitive defects in adults
with congenital heart disease, but They suggest that timely identification
and referral for treatment or support services might prevent care gaps,
which might improve quality of life and possibly reduce mortality
and morbidity in the adult congenital population. With this as
a background, this study, the so called Multi Institutional Neurocognitive
(03:37):
Discovery Study and Adult Congenital Heart Disease or MINDS ADHD study,
aim to assess neurocognitive functioning in young adults with moderate
to severely complex congenital heart disease in order to better
understand the scope of this problem by identifying the prevalence
of neurocognitive disorders and the need for cognitive remediation programs.
(03:59):
They state that the primary aim of the study was
to investigate neurocognitive functioning of eighteen to thirty year olds
with moderate to severe complexity congenital heart disease, with the
first hypothesis being that these adults would score lower than
a standardization sample on the so called NIH Toolbox Cognition battery,
and the second hypothesis was that the Adult Congenital Heart
(04:20):
Disease cohort would have lower neuroquality of life Item Bank
Version two cognitive function scores than the standardized sample. A
secondary aim of this work was to explore the association
of neurocognitive dysfunction with demographic and clinical factors. This was
a multi center, cross sectional pilot study of individuals aged
eighteen to thirty years with moderate to severely complex congenital
(04:43):
heart disease and was conducted via the phn NHLBI in
alliance with the Adult Research and Congeneral Cardiology Group and
involved fourteen ADHD centers in North America. And this study
was conducted from October twenty twenty to May of twenty
twenty Freves. The participants had to complete questionnaires and provide
biospecimens during a single visit or within twelve months of
(05:06):
a clinic visit. And the authors review the many inclusion
and exclusion criteria and I would encourage that listeners take
a look at the paper with the link in the
show notes. Patients who had known genetic syndromes were not
excluded unless they met other exclusion criteria. The authors review
the NIH toolbox cognitive battery and what it includes and
the validation for it that was used in this study.
(05:27):
They also created a neuro quality of life instrument with
twenty eight questions to self report neurocognitive function including memory, attention,
decision making, and daily task performance. If NIH toolbox respondents
fell one or more standard deviations from the normal population mean,
indicating possible neurocognitive problems, Recommendations were made for further clinical
(05:50):
evaluation of cognitive symptoms to the different minds AHD sites.
Tons of clinical and demographic data were obtained, including details
of the medical history as well as things like age, sex,
educational level, race, ethnicity, occupation, and socioeconomic status, amongst many others,
and DAHNTA the results. Initially, there were nearly twenty six
(06:11):
hundred patients eligible for this work in the fourteen participating centers,
and twenty one hundred and thirty were approached and of
these five hundred and one consented to participate, so basically
less than twenty five percent of eligible patients. And of
these five hundred and one, four hundred and sixty seven
participants completed all the study procedures and so were the
(06:32):
focus of this work. The patients were grouped into four
main groups. Detransposition of the Great Arteries, of which there
were eighty two participants, to Trilogy of philoh of which
there were one hundred and twenty three participants, single ventricle
patients one hundred and thirty two participants, and other moderate
or severe congenital heart disease one hundred and thirty participants.
(06:52):
When looking at the demographics, the average age was about
twenty five years, with fifty six percent being women, eighty
one percent white, and s seventy eight percent were in
New York Heart Association Functional Class one. The four groups
of patients were demographically the same, with the only difference
being that the DTGA group had a higher proportion of
men at fifty two percent. Almost one quarter of the
(07:14):
group were in school, with thirty nine percent having a
college degree or postgraduate degree. The single ventrical group was
different than the other congenital heart disease groups in that
there was a higher proportion of high school or lower
grade level achievement thirty three percent. Fifty five percent of
the group were employed full time, with sixteen point five
percent part time, and sixty five percent of the group
(07:36):
were single. There were a number of comorbidities in These
included hypertension in nine point six percent, diabetes in one
point three percent, congestive heart failure in five percent, seizures
in three point six percent, atrial or ventricular arrhythmiasm, seventeen percent,
and importantly thirty three percent were obese, and these findings
(07:56):
were similar in the four groups. The prevalences of mental
health disorders were similar between the four groups as well.
When looking at the neurocognitive findings using the objective NIH
toolbox testing, thirty four percent of the participants scored more
than or equal to one standard deviation below the normative mean,
with the score of forty four in comparison to fifty
(08:18):
for the normative mean. The mean cognitive function score was
significantly lower in tetrology, single ventrical and other moderate to
severe congel heart disease populations, but not so in the
DTGA group. The lowest scores were seen in the single
ventrical group and highest in the DTGA patients. Among the
single ventrical patients, fully forty one percent scored at least
(08:40):
one standard deviation below the mean, followed by tetrology of
below in which thirty seven percent were below one standard
deviation from the mean, and the mixed group of thirty
two percent, and finally the DTGA group at twenty percent
Figure one on page six hundred and forty six of
this document nicely demonstrates the cognitive function compass. It scores
(09:01):
and shows graphically how tetrology, single ventrical and other moderate
to severe coan joal heart disease patients scored statistically significantly
below the normal average, whereas the DTGA patients were in
the normal range. Using the subjective neuroquality of life item
bank testing, twenty two percent scored more than are equal
to one standard deviation below normative levels? And what were
(09:24):
the predictors of dysfunction? In the objective nih toolbox testing?
On multivariable analysis, the worst performance was linked to a
lower New York Heart Association functional class, with lower being
worse and lower educational attainment. When looking at the more
subjective neuroquality of life testing, worse outcomes were associated with
lower educational level, higher BMI, presence of atrial arrhythmias, and
(09:48):
symptoms of depression and anxiety. In their discussion, the author's
state and I quote our findings indicate that objective neurocognitive
dysfunction was identified in nearly one third or thre thirty
four percent of the cohort, correlating most strongly with level
of education. Subjective neurocognitive dysfunction was observed in one fifth,
(10:08):
or twenty two percent of the cohort, also linked to
educational level, but negatively associated with BMI atrilarrhythmias and underlying
psychiatric conditions such as depression and anxiety. These deficits may
persist throughout the lifespan and affect various neurocognitive subdomains, including
executive functioning, information processing, psychomotor speed, reaction time, attention, fine
(10:33):
motor skills, working memory, and visiospatial abilities, particularly in individuals with
complex congenital heart disease. If I were to summarize the
main points of their discussion, it would be a restating
of this being the largest such study assessing objective and
subjective neurocognitive dysfunction in adults congenital heart disease patients. They
use these data to suggest that there is a need
(10:56):
to integrate neurocognitive assessments into routine care for the achd
PA patient and wonder if early detection of these issues
can help guide the development of clinical guidelines and targeted
interventions to improve outcomes in quality of life. The authors
speak of the overrepresentation of neurocognitive dysfunction amongst single ventrical patients,
contrasting this finding to the DTGA patients, in whom the
(11:18):
prevalence of these problems was similar to the general population.
They suggest that this may be because of the rapid
establishment of normal cardiovascular hemodynamics in transposition patients in contrast
to single ventrical patients who are exposed to syinosis long term,
as well as heart failure and arrhythmias. A lot of
discussion about quality of life is made in this work,
(11:39):
trying to parse out what the factors are to explain
it and how neurocognitive dysfunction can play a role. Lower employment,
for example, is common amongst ADHD patients, and they explain
how this fact can be affected by so many different factors,
with educational achievement being an important one that is clearly
lower in the ADHD patient group, perhaps due to the
(12:00):
neurocognitive dysfunction described in this work. However, is this an
example of a situation of the chicken in the egg,
which problem causes which effect. They do posit that if
more focus on educational planning were done earlier in children's
lives with congel heart disease, perhaps this might be an
opportunity to improve later achievement and overall quality of life
(12:20):
in the ADHD patient group. The author's comment on the
relation of subjective measures of neurocognitive dysfunction and anxiety and
depression and feel that this should highlight the vital role
of mental health in the overall health of the ADHD
patient and the potential critical role for mental health professionals
and caring for this group of patients. They bemoan the
insufficient allocation of resources to address neurocognitive problems in this
(12:45):
patient group, which coupled with societal stigma in addressing neurocognitive
dysfunction associated disabilities, means that this is indeed a very
hard problem to address because of the complex and frankly
expensive solutions that that are required. Feel that this work
sounds the alarm for the need for dedicated resources to
screen ACHD patients for neurocognitive dysfunction because of the profound
(13:09):
impact neurocognitive dysfunction can have an overall health and quality
of life, and they again speak about how important they
believe early identification and referral would be to try and
meaningfully improve outcomes in this regard. The authors speak of
the limitations of this work, including what could be a
substantial sampling bias, given that less than one quarter of
patients queried actually participated in the study and the fact
(13:31):
that more than eighty percent were white, with the predominance
of females and more educated New York Heart Class Association
IE patience. All of these may significantly affect generalizability of
these data to all adult congenital patients. The authors also
point to some limitations of the NIH toolbox test that
was used, as well as the neuro Quality of Life test,
(13:54):
and so they conclude our findings reveal that objective neurocognitive
dysfunction we're identified in one third or thirty four percent
of the cohort, showing a correlation with education and New
York Heart Association functional classification. Similarly, subjective neurocognitive dysfunction were
present in one fifth or twenty two percent of the participants,
(14:16):
with links to educational level and negative associations with atril
arrhythmias BMI and underlying psychiatric conditions such as depression and anxiety.
These results underscore the critical need for integrating neurocognitive assessments
into the routine care of adults with congenital heart disease.
By identifying these deficits early, we can inform clinical practice
(14:40):
guidelines aimed at proactive detection and management of neurocognitive dysfunction.
Addressing these challenges paves the way for targeted interventions that
can not only improve cognitive outcomes, but also improve the
overall quality of life for ADHD patients. Accompanying this article
as an editorial comment by doctors shaf Pavandi and ROBERTA.
(15:01):
Williams from UCSF and USC, respectively. The authors of this
commentary review the major findings of the work that we
have just reviewed. They emphasize that this work has demonstrated
a very high burden of neurocognitive defects or dysfunction, more
than one third, and how this means that in the
US alone, we're speaking of over one hundred thousand patients
(15:22):
with these problems, and they suggest to use their terminology
that these findings are jarring. The author's comment on the
finding of educational level being a major factor in the
multi variable model for both objective and subjective cognitive measures,
and they state that this quote begs the question of
which came first poor neurocognitive function making it harder to
(15:43):
succeed in school? Or poor school participation and persistence driving
worse neurocognitive outcomes, and they suggest that the work cannot,
unfortunately ferret out this relationship. They re emphasize the paper's
demonstration that mental health problems were highly associated with neurocos
cognitive dysfunction, and again re emphasize the important role that
(16:03):
mental health support should play in the care of the
adult congenital patient. The authors essentially feel that this work
is a call to action and stress the need for
embedding neurodevelopmental and mental health care early in childhood and
extending this throughout adulthood for patients with significant congenital heart
disease so that, in the author's words, our patients can
(16:24):
achieve their full potential in life.
Speaker 2 (16:27):
Well.
Speaker 1 (16:27):
This is clearly an interesting and important work demonstrating the
very high prevalence of neurocognitive dysfunction amongst ADHD patients with
moderate to severe congenital heart disease. There are a number
of methodological limitations of this work and biases that might
be skewing these data, and most of these patients were
born in the twentieth century, and so it might be
hard to say if these data reflect what a baby
(16:49):
today will be like in twenty more years. But certainly,
as doctors Paivandi and William's right, this is indeed a
call to action not only for the mental health and
neurocognit and of support of ADHD patients, but also pediatric patients.
How this would practically work in pediatric cardiology practice and
how it would be paid for are questions that are
(17:10):
perhaps above this podcast or pediatric cardiologists pay grade. But
certainly this work puts into stark relief the critical nature
of these problems and really should leave us all wondering
how we can intercede to improve our patients' lives. To me,
this work is saying that we should no longer view
ourselves only as people who save lives, but also as
(17:31):
doctors and care providers who wish to improve lives. How
do we do that is likely something that will become
more clear in the coming decades based on data from
works like this week's. In the interest of time, I
think we'll move forward to our conversation with the work's
authors Jennings now to discuss this week's work is its
first and senior author, Doctor Scott Cohene is an ACHD
(17:53):
specialist at the University of Wisconsin. He received his medical
degree from Saint Louis University School of Medicine and completed
rests and fellowship in cardiology at Brown University. Following these,
he went on to perform an ADHD fellowship at the
Children's Hospital of Wisconsin and he is an ADHD specialist
both at Children's Wisconsin and also Freudtert Hospital. Doctor Ali
(18:15):
Zadi is a graduate of the Aga Khan University in Karachi, Pakistan.
He completed his combined internship and residency in internal medicine
and pediatrics at the Penn State University Hershey Medical Center.
He did chief residency in internal medicine at Penn State
before pursuing cardiology training at Columbus, Ohio as well as
adult congenital training. He is a professor of pediatrics and
(18:37):
Medicine at Mount Sinai where he is the director of
the adultkin General Heart Disease Center at Mount Sinai as
well as a co director of our Children's Hearts Center.
It is a delight and pleasure to welcome my friend
doctor Zaidi and doctor Cohne to Pediheart. Welcome doctor Cohne
and doctor Zaidi. I'm here now with doctor Scott Cone
and doctor Ali Zaidi. Guys, thank you very much for
(18:57):
joining me this week on Pdheart.
Speaker 3 (18:59):
Yeah, thanks for the really, thanks Rob, thanks for having us.
Speaker 1 (19:02):
Real pleasure. Really enjoyed it. You know, I'll leave before
we get going. I'm wondering if you could share with us, like,
how did you come to this idea of doing this
project and what exactly did it involve? It seemed like
it was extremely involved, So how did it get going?
Speaker 3 (19:19):
Yeah?
Speaker 2 (19:19):
Thanks, thanks Rob and Scott, thanks for being here. So
so Rob, this started back if you believe it, in
twenty and seventeen or somewhere right around there. It was
I'll take just thirty seconds to explain how this came along.
It was quite honestly all of us in ADHD, and
it was Scott. It was Scott's idea to sort of
look at neurocognitive functioning and just just sort of see,
(19:41):
you know, what's happening in our clinics, you know, sort
of do some sort of some sort of testing. And
it turned out that there was no valid sort of
questionnaire that we could just pull out of our pockets
in our clinics and say, hey, listen, let's let's look
at neurocognitive functioning and AHD. That's how started in twenty
seventeen and Quantoti, we got ARC involved, which is this
(20:04):
large consortium of ADHD clinical investigators, and then we went
to the PHN because we needed funding, and thankfully the
PHN funded it through the NHLBI and we got enough
funding to then use fourteen.
Speaker 3 (20:19):
Centers across North America.
Speaker 2 (20:20):
That was back in twenty and seventeen and eighteen, and
we started looking at this. The way we did this
rob was actually it was a learning experience for all
of us. What we'd learned was that we can't just
really test neurocognitive function in our piic.
Speaker 3 (20:34):
We didn't know how.
Speaker 2 (20:36):
And what we found out was that we had to
involve neuropsychologists that were two part of Minds that were
housed as a core at MCW and they helped us
sort of define which questionnaires needed to be done.
Speaker 3 (20:50):
They gave us those questionnaires and then.
Speaker 2 (20:52):
We did this over three years and we recruited five
hundred patients over when we started, I think was twenty twenty.
Speaker 3 (21:01):
I think that's when we started re suiting patients right YEP.
Speaker 2 (21:04):
And then COVID happened, and we pivoted through that, and
then we went through that and we ended up getting
five hundred patients. It took quite a bit, quite a
bit of effort.
Speaker 3 (21:13):
So that's how we that's how we got here.
Speaker 1 (21:16):
And so the folks, the neuropsychologists in Wisconsin had identified
the appropriate questions or a questionnaire to be used. Is
that how that works? Gone?
Speaker 4 (21:27):
Yeah?
Speaker 5 (21:27):
Correct, So we wanted to assess both objective and subjective
neurocontent functioning, and they helped us identify which questionnaires we
could use. For objective functioning, we use the NH Toolbox
and for the subjective neurocontent functioning, we used the NEUROQOL survey.
Speaker 1 (21:49):
I see right, Well, thank you for that. That's very interesting.
So we're really looking at the efforts of a very
long effort, and congratulations again.
Speaker 3 (21:57):
To both of you on that.
Speaker 1 (21:59):
Just the stick to it miss alone is worthy of congratulations. Scott,
since you're the first author, I wondered, you know, sometimes
I'll start these interviews off and ask the authors of
the work if maybe they could summarize what they believe,
maybe are the four or five most important takeaways from
this work this week? Maybe you could share your insights
into it.
Speaker 3 (22:20):
Yeah. Sure.
Speaker 5 (22:20):
So, as Ali mentioned, we ended up recruiting nearly five
hundred patients for this study. In nationality, it came out
to be four hundred and sixty seven patients with moderate
or severely complex congeneral heart disease age between eighteen and
thirty years across the fourteen sites.
Speaker 3 (22:40):
In the US and Canada.
Speaker 5 (22:42):
We chose this age range because we really were to
focus in on the nervokognitive function in young adults at
the stage of transition where they're going from pediatric to
adult healthcare, where they may be leaving to go to
college or starting a new job, new relationships. And what
we found is and the total cohort, thirty four percent
(23:03):
of these patients scored at least one Stanard deviation below
the normative mean on our objective neurocognctive function. Twenty two
percent scored at least one Seren deviation below the normative
mean for the subjective neuroconnactive function. We found that educational
level and NYHA classification were both independent predictors of objective
(23:27):
neurocognitive function and educational level BMI history of atrilarrhythmias and
history of mental health disorders such as anxiety and depression
were independent predictors of subjective neuroconnactive function. And the last
thing I want to call attention to is just the
characteristics of our cohort.
Speaker 3 (23:47):
During screening.
Speaker 5 (23:48):
We had about more than half of the patients we
approached for consent actually declined consenting for our study, and
so we came out with a population that were very
high functioning, well educated individuals. Nearly eighty percent of our
cohort where NYHA Class one nearly eighty percent had at
(24:08):
least some college experience or an associate degree, Nearly seventy
percent worth either full or part time employed. And so
we are actually likely under estimating the true prevalence of
neurocogncin functioning in our adult in general heart disease population.
Speaker 1 (24:26):
Wow, that's a very important point. So this is really
the best case scenario basically, So what you're.
Speaker 3 (24:32):
Saying, yeah, yeah, I think So that's a.
Speaker 1 (24:34):
Little disappointing, I guess, But as a doctor Pavante suggested,
this is a call to action. So I'm wondering, Scott.
You know, educational achievement, as you mentioned, a moment ago
was highly associated with neurocognitive dysfunction, with lower levels of
education being associated with worse levels of function. In this
(24:57):
regard the office of the editorial, I'll ask this question,
and so I thought I would ask you which came first,
the neurocognitive dysfunction or the lower educational achievement. I mean,
is that even the right question to ask, like which
is causing which or what's their relationship there?
Speaker 5 (25:14):
Yeah, I think that's a really tough question that may
not be able to answer. Based on our cross sectional study,
there's clearly a bi directional relationship between neurocogctive function and
educational attainment, such that those with higher neurocogctant function will
likely reach a higher level of educational attainment. But we
(25:35):
could also think that those that have access to better,
higher quality education may also be able to have improved
neurocognctive function. I think that this relationship is very complicated
and probably future longitudinal studies are needed to better answer
that the question of what that relationship means looking at
(25:58):
whether more or better higher educational attainment can actually improve
neuroconcert function. But at this point in time, I think
the best thing we can do is really focus on
improving access to high quality education for our young conngenital
heart disease patients and really optimizing their educational attainment by
(26:19):
developing appropriate IEPs, assessing the need for accommodations in middle
school and high school, and then really encouraging these patients
to continue the awareness of what they needed and what
kind of combinations they needed in middle school and high
school into their next stage of education, whether that be
college or vocational school or beyond.
Speaker 3 (26:40):
Yeah.
Speaker 2 (26:41):
Wrong than just jumping real quick, I think you hit
the nail on the head as the editorial, because it's
really the question is the chicken and the egg, right
which comes first?
Speaker 3 (26:50):
It's and it's tricky.
Speaker 2 (26:53):
It's very tricky because in essence, we are showing a
bi directional relationship, so that you know, if you have
a bad neural, are going to functioning that bad education?
Or is it bad education leading to the others. So
it is a very complex relationship, and as Scott said,
I think we need to delve into this maybe perhaps
even in the earlier years, you know, not in this
(27:13):
school ward per se.
Speaker 1 (27:15):
Well, you know, it's interesting the patient group you guys
studied were relatively young ADHD patients by intention, As you
explained Scott eighteen to thirty. You know, it's interesting. Doctor
Gilbernowski posted on LinkedIn when I wrote that we were
going to be discussing this this week, and he said,
(27:37):
let's remember all of these patients or the majority, were
born twenty or thirty years ago, and he liked he
said they were ball born in the twentieth century, trying
to make it seem like we're very advanced today. I'm
wondering if you think that the findings that we're finding
that you've identified today in the ADHD young population are
(27:58):
going to be applicable to children and who are infants
today twenty years from now.
Speaker 3 (28:03):
Yeah, that's a good question.
Speaker 5 (28:05):
I think the study, the mind study, reflects the longitudinal
experiences that our cohort had over their eighteen to thirty
years of their lifetime and identified risk factors of objective
and subjective neuroconnactive deficits within those experiences. But for today's infants,
I think there's been achievements and advancements and care models
(28:27):
that may lead to better neuroconnactive outcomes for our young
adult cogeneral heartsease patients.
Speaker 3 (28:34):
So I think.
Speaker 5 (28:35):
There's a growing awareness of neurodevelopmental outcomes and children with
congenital heart disease and that continued issue with neuroconnactive deficits
and our ADHD patients. This awareness, I think, was demonstrated
in a updated American Heart Association scientific statement Neurodevelopmental Outcomes
and Individuals with Congenital Heart Disease that was published by
(28:56):
Erica Sud and others just last year.
Speaker 3 (28:59):
This statement gave.
Speaker 5 (28:59):
Up updates on topics such as risk assessment for a
poor neural developmental outcomes, potential genetic basis or perinatal factors
that impact neural developmental outcomes, and neuroprotective strategies at times
of surgery for kids born with congole heart disease. So
I think, overall, with continued collaborative research in the pediatric
(29:21):
cardiology realm and continued awareness of this issue as it
also moves into adults living with congenital heart disease, my
hope is that we'll see better neurocognitive function in our
young ADHD patients.
Speaker 3 (29:32):
In twenty years.
Speaker 1 (29:33):
Yeah. Well, I certainly think that the efforts of all
of those investigators, as well as the editorialist, doctor Pivandi,
is at the forefront of this. Doctor Jane Neuberger and
many others certainly give us cause for optimism. You know,
I'm wondering if you guys might be able to speak
about the relationship of mental illness to neurocardiac disfunction cocognitive
(29:55):
dysfunction and how should we think about this, Ali, I
know this is a particular interest of yours.
Speaker 2 (30:00):
Yes, yeah, thanks, thanks Robin and it Scott. Thanks for
taking the question to mental health.
Speaker 3 (30:06):
Rob It was.
Speaker 2 (30:07):
It was fascinating because we did look at a questionnaire
looking at mental health, both anxiety and depression, and we
looked across the board and what we found was that
approximately one third of this cohort was had sort of
scored in the mental health spectrum which means either anxiety
or depression. And that's one third, that's about thirty three
(30:27):
thirty five percent of patients. And what we showed was
a clear association with one quality of life and two
it was an independent predictor for subjective neurocognitive functioning. So
if you had anxiety or depression, that was an independent
predictor for subjective neurocognitive functioning.
Speaker 3 (30:44):
So again that makes you.
Speaker 2 (30:45):
Go back and take a look and say, well, hang on,
displays a big part in neurocognition mental health play is
a big part in it. And then the next question
that comes into our mind as we all look at it.
These kids are growing up and they're growing up with
in essence anxiety, PTSD and things along those lines. So
what can we do better? This is one where Rob
(31:07):
we're actually going to do a sub study from the
Mind's cohort and we're going to delve into this even
more and try to see what other factors we can find.
That's one and two. Can we then try to identify
resources that can sort of mitigate this early on so
that we can then help from a neurocognitive functioning standpoint,
(31:28):
So I e. Catch them when they're eight, nine, ten,
eleven years old, mitigate the anxiety as much as we can,
and then see what happens down Thereaw, and now can
we prove this from minds it's a cross sexual study.
We cannot, but I think that's sort of the next
step to take.
Speaker 1 (31:44):
I see, well, both of you guys are also proud
pediatric cardiologists, So I'm going to ask you to put
on your pediatric hats here and ask you know, Scott,
you hinted at this earlier, maybe Ali, you want to
take this question. You know, what's stuff could pediatric cardiology
providers take to interfere with this process of neurocognitive dysfunction.
(32:06):
You just hinted at it, Ali in terms of the
role that we could have in managing mental health problems.
You know what's needed in the early years to improve
the sequelite of this problem that you've identified. I've always
been interested that the adult congenital clinics have psychological and
psychiatric support sort of embedded in them, but we don't
(32:29):
do that at all in pediatric cardiology are very minimally so,
at least routinely. So I'm wondering what what sort of
recommendations do you think you would you would make.
Speaker 2 (32:40):
I can take the first part of this, and that's
got I'm going to take you. Let you let you
take the next part of this. But so, Robbie're absolutely correct.
I mean, this is a spectrum that sort of stems
from pediatric crdology and sort of goes into adult in
genital and quite honestly, there is there is a there's
a there's a big body of work that is Jane
Neuberger Gilbernowski. You said, Michelle Gerbertz, who is a co
(33:01):
investigator of Fowers on this study. Michelle has done some
great work from Boston Children's too, and all the folks
that I mentioned that several more in the country have
worked on this, and what they're trying to say is
that can you catch this earlier on? Is there a
way you can actually do the screening in these kids
when they're younger?
Speaker 3 (33:21):
And there are actually some.
Speaker 2 (33:22):
Scientific statements that have come out from the AHA, again
led by doctor Newberger and doctor gil Bernowski, saying that
if you catch it early, can you mitigate it?
Speaker 3 (33:34):
Now?
Speaker 2 (33:34):
What does that mean? Does that mean mitigation from either
or techniques? Is there something that needs to be done
early on from a developmental standpoint?
Speaker 3 (33:42):
So all those things come into play. That's early on.
Speaker 2 (33:46):
As a pediatric cardiologist, we're getting them when they are
in their twenties, So can we do this and can
we mitigate this by the time they get in their twenties?
So I think so there are techniques in their ways.
There's Scott if you want to add to that. The
other part Rob that I'm going to add is that
we did not get into the forty fifty sixty year
(34:07):
old cohort there's a whole plethora there that may or
may not be affected by acquired disease spectrums that we
didn't even touch, right, right.
Speaker 5 (34:17):
Yeah, I agree with everything Ali mentioned. I would also
add that a good transition program would be essential to
allow or help lower the risk of gaps and care
as these young adults transition from pediatric care into adult
co general heart disease care, so that us as adult
(34:38):
conjeneral heart disease providers can continue the work that was
started in the pediatric realm, and then emphasizing continued neurocognitive
evaluations and referrals to specialists as needed.
Speaker 6 (34:52):
And I think going back to the statement about ADHD
clinics having mental health or SOL workers embedded in their clinics,
I think there's a fair degree of a spectrum in
terms of how much ADHD clinics actually have social workers
or mental health providers within their centers are dedicated within
(35:14):
their centers. I think there are a fair number of
centers where the social workers often split between ADHD and
other specialties and may not always have the bandwidth to accommodate.
Speaker 3 (35:26):
The ADHD team.
Speaker 5 (35:28):
And I think the same could be said for mental
health providers especially in centers that maybe in rural areas
or in the so called ADHD deserts where there really
aren't any major ADHD centers of excellence within their region.
So I think from an ADHD standpoint, when they get
to us as ADHD providers, I think starting to enact
(35:50):
widespread mental health and neuroconnective screening in the outpatient setting
would be a good first step.
Speaker 3 (35:57):
That's easier said than done.
Speaker 5 (35:58):
Of course, we don't really know exactly what screening tool
would be most efficient from a time perspective or a
sensitivity specificity aspect, but I think having that screening in
some way would be a good first step. And then
if you're going to be screening, you have to know
what resources you have available for those individuals at screen positives.
(36:20):
So for those centers that don't have embedded mental health
providers or neuropsychologists, knowing in the community what other centers
may have mental health providers that you could refer to,
and establishing a good referral program or relationship with those
providers I think would be really necessary. And then one
(36:42):
last thought I had was having a check or a
follow up program for those that you do refer to
mental health providers or neuropsychologists, because a lot of these
patients that have neuroconc at dysfunction may not be able
to easily call and set up a point means for
intake with these providers and may get lost to follow
(37:03):
up even though you've put the referral in. And I
think same could be said for individuals with mental health concerns.
So having a good follow up system and a good
Claudter referral process by all providers would really benefit these patients.
Speaker 1 (37:17):
Those are all very interesting ideas, Scott. It's really even
as simple as administrative support is very important for this, Ali.
Speaker 3 (37:26):
I'm wondering.
Speaker 1 (37:28):
You know, we're already hitting on my final question, which is,
you know, in a perfect world, what else would you
want in an ADHD clinic to address these issues?
Speaker 3 (37:38):
Scott?
Speaker 1 (37:38):
I think really nicely summarized the notion of, you know,
making having someone to check that there's adequate follow through.
You can make all the recommendations in the world if
no one follows them. Any other things that in a
perfect world, Ali, you would like to see routinely in clinics.
Speaker 3 (37:56):
So thank you. You know, there's so much to do here.
Speaker 2 (37:59):
I mean, there's this has been quite a learning experience
for all of us in ADHD, this whole neurocognitive journey.
The two things on highlight, it's kind of alluded to
transition clinic, this, that and the other. But the one
thing I learned is that we can screen and we
can make a diagnosis, and we can come up with
and you know that patients can have neurocognitive dysfunction, but
we then need to get them to a specialist, which
(38:20):
is a neurocognitive or neuropsychologist, somebody who specializes in that realm.
And I'll be honest with you, when we did this,
we actually struggled, even in the fourteenth centers we were
working with if they had dedicated people that we could
then refer these patients to. So that resource structure in
(38:40):
itself is few and far between. So we've just screened,
made a diagnosis, but then the intervention itself is a
resource structure, and that is quite that I think is
going to be a heavy lift. I think that to
your point, I mean, would we want that as part
of ADHD absolulutely, but I think that that is a
(39:01):
key metric.
Speaker 3 (39:02):
I don't know if I have the right answer to
them Rob.
Speaker 2 (39:04):
The one other thing I was going to add to
what your question initially and is as far as pediatric
cardiology goes, if I recall it, I think it's twenty
ten or twenty twelve. There is a scientific statement Jane Neuburger,
Brad Marino, and they've done some work in screening and children.
So I just want the viewers to know that our
cohort was adults. It was all adults, so we really
(39:26):
didn't look into the sort of the pediatric side of them,
although we did have some variables that we looked at.
Speaker 1 (39:34):
Wow, well, obviously it seems like we just hit the
tip of a massive iceberg, and I think there's a
lot more that we are going to need to learn
about this. But your work, extraordinary work, is really a
wonderful first step in at least identifying the problem or
(39:54):
the magnitude of the problem. I want to congratulate you,
doctor Cone, Doctor's for a really wonderful work. Also congratulate
all your many many co investigators, all who work very
hard on this project. And I want to thank you
both for spending time with us this week to discuss
the work on pd heart Great.
Speaker 2 (40:13):
Yeah, thank you, thank you Ron, Thanks for having us,
and a shout out to all the patients for letting
us do this over seven years.
Speaker 3 (40:19):
I mean it's without them it would be not doable.
But thank you absolutely well.
Speaker 1 (40:24):
As I'm fond of saying, when the guest is good,
there really is not much to discuss. Doctors Cone and
Zeti were uncommonly clear and offered a lot of important
context for this week's work, and I think the way
in which they made us all aware of these data
representing the best case scenario is important. As both authors
suggested to us that the bias and ascertainment of subjects
(40:45):
in this work may have actually caused the group to
overemphasize the better moderate to complex congenle hard patients. I
found their comments about ways to potentially intercede and improve
outcomes to be important and thoughtful, and think that doctor
Cohne's comments about the importance of transition in avoiding gaps
in care and resources was particularly important among the many
(41:07):
important comments that both authors shared with us. I'd once
again like to thank doctor Cohne and doctor Zadi for
taking time from their very busy schedules to speak with
us this week about this very important work on Pdhart
Before we get to the end of the podcast, I
wanted to mention a very exciting up and coming conference
that we'll be holding at Mount Sinai entitled the Kawasaki
(41:28):
Disease Across the Ages from Childhood Onset to Adult Outcomes,
the fourth Mount Sinai Adult Congenital Heart Disease Symposium. This
conference will be occurring at Mount Sinai from Friday September twelfth,
eight a m. To Saturday September thirteen, five pm and
will be held at the Leon and Norma Hes Center
for Science and Medicine Davis Auditorium here at Mount Sinai.
(41:51):
This event will bring together leading experts in pediatric and
adult cardiology to address the evolving needs of Kawasaki disease
patients across the lifespan. In order to better understand this
conference and what exciting things will be coming next week,
I've invited one of the course code directors, doctor Nadine Schwader,
who's Professor of Pediatrics here at Mount Sinai, to speak
briefly about it. Therefore, let's move straight on to our
(42:14):
brief conversation with Nadine Schwater. I'm here now with doctor
Nadine Schwader, who is the course director of the upcoming
Kawasaki Disease Across the Ages from Childhood Onset to Adult
Outcomes course here at Mount SINAI. Interestingly enough, our guest
that we just hung up the phone with doctor Zaidi
is the co director of this course. Nadine, Welcome to Pedheart.
Speaker 4 (42:37):
Thank you, Rob, Thank you for having me.
Speaker 1 (42:39):
Great Nadine, I explained to you that I really am
very excited by this upcoming course. I'm wondering if you
could share with the audience what the goals are of
this Kawasaki conference and then maybe share with us some
of the sessions that you're particularly looking forward to.
Speaker 4 (42:56):
Thanks Bob, Well, we're very excited about this meeting the uh.
The meeting plans to bring together world experts in Kawasaki disease,
both pediatric and adult cardiologists, to address how Kawasaki disease
affects patients through their lifetime and the you know, the
(43:17):
aims of the meeting is to bridge pediatric and adult
discipline in this particular disease, because we know that Kawasaki
disease is the leading cause of acquired heart disease and
children in North America and with advanced in therapy, more
of these children are surviving. As these patients transition into adulthood,
(43:37):
adult carreologists are now seeing complications like thrombosis, ischemia, acute
coronary syndrome, and right now is the time to educate
across specialties so we can ensure continuity of care and
optimal outcomes.
Speaker 3 (43:51):
So sessions.
Speaker 4 (43:52):
I'm particularly you know, excited about our sessions that bring
both pediatric and adult careologists and adult con genital heart
disease specialists to sit down and talk about how we
can partner to care for these patients, how corinaty archery
(44:12):
disease and children differ from corinaty archy disease and adults
and etherosculotic disease. We're also interested in learning about the
outcomes of Kasaki disease and adults from in Japan. It's
a large surveillance study that will be presented for the
first time. The data will be presented for the first time.
(44:34):
And finally, we have a live scanning echo workshop on
the next day of the meeting that teaches the attendees
how to image the cordinary arteries by echo in children
with Kawasaki disease, since this is the modality that we
use to determine the extent of the disease and the
(44:55):
corinaty archery involvement.
Speaker 1 (44:57):
Well, I know that traditionally when we ever you have
hands on echo sessions, that's always some of the most
desired and the fun parts of the conference. I'm sure
people are going to really enjoy that. On the second
day of the conference. For those in the audience, I'm
going to leave a link to the sign up page
if you want to attend this conference. And this is
(45:19):
an only in person conference, is that right, Nadine.
Speaker 4 (45:22):
Actually, we do have a virtual option. There's a virtual
registration option for it will be live zoom streaming with
ability for the participants and the attendees to ask questions online.
Yet the workshop will not be streamed live because it's
a hands on workshop.
Speaker 1 (45:43):
Okay, great, thanks for clarifying that. So again, maybe you
could just share once more with the audience, Nadine, when
the conferences and where it is.
Speaker 4 (45:53):
So the conference will be held next week September twelve
to thirteen, at the i Can's School of Medicine at
Mount Sinai in the Davis auditorium at the Health Science Center.
Speaker 1 (46:09):
Okay, great, and again for those interested in this very
exciting conference which is being co hosted by doctor Zaidi,
our adult congenital expert, and of course doctor Schweider, who's
world renowned for her expertise in Kawasaki disease. There'll be
a link in the show notes this week for those
of you who are interested to learn about it, and
I hope some of you will come to Mount Sinai.
(46:31):
It's a wonderful time in the year to visit New
York City. The weather is great, lots of things to
do outside of the conference, so learning and fun altogether. Nadine,
thank you very much for coming on and thank you
also for being a sometimes co host of the podcast.
Speaker 3 (46:45):
It's always great to have you on. Thanks again, Rob,
thank you.
Speaker 1 (46:49):
To conclude this episode of p D Heart Pediatric Cardiology,
today we end with one of the great tenors of
the past one hundred years, the wonderful Luciano Pavarotti. Pavarotti
was born and then Modina, Italy and went on to
have one of the largest and most successful of all
operatic careers, and he is a rarity in the field
and that he was not only loved by opera lovers
(47:10):
in the world, but also by all fans of music
because of his very popular concerts that he performed in
sold out gigantic arenas. Today we hear him in a
performance from over thirty years ago of Larze Liana's e
La Sulita storia from Act two of Chilea's opera. This
opera is rarely performed today, but this particular tana aria
(47:31):
is a favorite of many, and you will hear why
when sung by the master Pavarotti with his lovely tone,
timber and diction. Thank you very much for joining me
for this episode, and thanks once again to our wonderful guests.
I hope I'll have a good week ahead.
Speaker 7 (47:47):
Ela sasco.
Speaker 8 (48:00):
Ah so baa cont so don't let me last a
(48:32):
lot of the oh ny a, but order me reps.
Speaker 2 (49:04):
As on.
Speaker 9 (49:06):
All auto.
Speaker 8 (49:12):
Loma a.
Speaker 9 (49:15):
T what aca.
Speaker 8 (49:36):
This monest.
Speaker 3 (49:41):
Feel said you.
Speaker 7 (49:49):
You all listen, send me labouchital, Yes, said.
Speaker 9 (50:24):
Walk scor.
Speaker 7 (50:40):
I said nynacord on anything.
Speaker 9 (50:54):
Be t don't
Speaker 2 (51:00):
Me
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