September 12, 2025 • 27 mins
This week we review a recent report from the Boston Children's team on their 39 year experience managing and caring for children with myocardial infarction following Kawasaki disease (KD). What were the clinical features of KD patients that were most associated with this rare complication? What signs or symptoms should be concerning for the caregiver of patients with KD? What are the more common approaches to the management of MI in this fragile and young patient population? Dr. Sunil Ghelani of Boston Children's Hospital offers the answers this week.
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Episode Transcript
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Speaker 1 (00:16):
Welcome to Pdheart 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, where I am the chief of Pediatric
Cardiology and the co director of the Children's Heart Center.
Thank you for joining me for this three hundred and
fifty fifth episode of Pdheart. I hope everybody enjoyed last
week's episode, in which we spoke with doctor Cohne and
(00:38):
doctor Zeidi about neurocognitive dysfunction amongst adult congenital heart patients.
For those of you interested in this important topic, i'd
recommend you take to listen to last week's episode three
hundred and fifty four. As I say most weeks, if
you'd like to get in touch with me, my email
is easy to remember. It's Pdheart at gmail dot com.
This week we move into the world of Kawasaki disease,
(01:00):
which seems appropriate since we are hosting a Kawasaki program
symposium at Mount Sinai on Friday and Saturday of this week.
The title of the work we'll be reviewing is maya
cardial infarction in Kawasaki disease. The first author of this
work is Sunil Ghilani and the senior author Jane Neuberger.
And the authors come to us from the Department of
Cardiology at the Children's Hospital Boston. When we're done reviewing
(01:24):
this paper, the work's first author, doctor Glani, has graciously
great to speak with us about it. Therefore, let's move
straight onto this article and then a conversation with doctor Galani.
This week's work begins with a review of Kawasaki disease,
reminding us that it is the leading cause of acquired
heart disease in the developed world, that it is most
common in children less than five years of age, and
(01:44):
they review the common presenting signs including fever, rash, non
exudative conjunctiveitis, inflammation of the oral mukosa, unilateral cervical at anopathy,
and extremity findings. They then review the fact that up
to one quarter of patients with Kawasaki disease will develop
carnary aneurisms if timely administration of IVIIG is not performed.
(02:05):
Because of what we know about this and the timely
interventions that are typically performed, only about one percent of
KD patients develop large or giant carinary aneurisms, and we
are all aware that these can predispose children to thrombosis, stenosis, myacardalinfarction,
and even sudden death. The authors then review the facts
that because my carlin farction is thankfully rare in this setting,
(02:29):
diagnosis of mi I and small children and the appropriate
treatment is challenging given the dearth of information regarding mi
I in this setting. The authors note that there are
a few reports of mi in Kawasaki disease in Japan,
but thus far no such reports in North America. With
this as a background, the authors explained that the goal
of this work was to review patient characteristics, clinical course,
(02:53):
and outcomes following my cardalin farction secondary to Kawasaki disease
in a very large, single center cohort. This was a
retrospective study of all patients with the history of Kawasaki
disease and mycarial infarction who were evaluated and treated at
Boston Children's Hospital from January of nineteen eighty four to
January of twenty twenty three, in other words, over a
(03:14):
thirty nine year period. The authors explained what an acte
MII was defined as for this study and suffice it
to say that the definition is robust and it would
seem unlikely that anyone would be in that category in
this study without an actual mi The authors reviewed the
data that they were collecting, including massive amounts of clinical
data such as age at the time of onset of
(03:34):
Kawasaki disease, time to the first IVIIG treatment, number of
courses of IVIG, use of steroids or other immunomodulators, as
well as antithrombotic or thrombolytic therapy use. They recorded all
sorts of morphological aspects of the caronaries and the aneurysms themselves,
and of course reviewed in detail the clinical courses of
(03:55):
these patients, trying to determine if bypass graphs or interventions
were needed or even ICD implantations and dawn to the results.
Speaker 2 (04:03):
Well.
Speaker 1 (04:04):
Over this long thirty nine year period, there were twenty
two patients who met inclusion criteria, and there was a
significant preponderance of males at eighty six percent with the
median onset of Kawasaki disease at fourteen months, So again
there were twenty two total Kawasaki disease mi I patients
over forty years at Boston Children's Hospital, the median age
(04:24):
at Kawasaki disease onset was fourteen months, with a range
of three months to fifteen years. Twenty of the twenty
two patients had received IVIIG, with initial treatment occurring at
a median day of illness of ten, with a range
of four to forty two days, with six of twenty
two having had their first IVIIG at less center equal
to day seven of illness, and three of twenty two
(04:47):
on days eight to ten of illness. For the two
patients who did not receive ivig one at Kawasaki disease,
presenting in nineteen eighty four and the other presented in
nineteen ninety nine with a remote history of what was
likely missed Kawasaki disease in infancy of note ten of
the twenty two patients received a second dose of IPIG
(05:07):
and three patients a third dose. So in general these
were difficult patients to treat. And when did the mis occur, well,
forty eight percent occurred within three months of acute Kawasaki
disease and ninety one percent within two years, and so
it does seem that getting past two years without this
complication is perhaps somewhat reassuring. All of the patients in
(05:29):
this cohort had at least one large or giant carnary
artery aneurysm, with most common being the proximal left anterior
descending coronary. The majority, or eighty five percent, had bilateral,
larger giant aneurysms. Of note, only thirty two percent or
seven patients actually had symptoms at the time of the
acute MI with four at the time of MI and
(05:51):
three in retrospect, and their symptoms were fatigue into chest
pain in one, syncope in one, ear pain in one,
upper extremity pain in one, and vomiting in one. And
how were these diagnosed while imaging either echo or MRI
contributed to the diagnosis in fifty percent and what happened
to these patients? While seven patients had caronary artery bypass grafting,
(06:16):
one had a percutaneous carnary intervention, and one was listed
for transplant. Two patients in this cohort died from mycardalin
farction related complications, but these were both patients in the
nineteen eighties, and twenty three percent of these mi I
patients had LV dysfunction at follow up importantly, ventricular at
tachycardia was seen in three patients, two of whom received
(06:38):
an ICD. In their discussion, the author's state and I
quote myocardalin friction is the leading cause of death in
patients with Kawasaki disease and occurs almost solely in patients
with large giant carnary aneurysms, defined as those having a
Z score greater than are equal to ten or absolute
diameter of eight millimeters. The incidents of micarl ourlin fraction
(07:00):
peaks in the first few months after Kawasaki disease onset
and declined substantially after two years from disease onset, but
sudden death from myacarlin fraction can occur later in life. Indeed,
six point seven percent and five percent of adults less
than forty years of age with acute carnary syndromes in
Egypt and San Diego, respectively at carnary aneurysms and stenoses
(07:23):
consistent with Kawasaki disease early in life. The authors speak
about the nonspecific nature of the symptoms of the myacarlinfraction
patients in this work and orfer this as a reason
for why these were often missed. They note that even
amongst adults, it's not uncommon to have found that an
am I may have occurred in the past without obvious
symptoms in real time. The authors explain that CMR is
(07:46):
a good test to surveil for a cult MI in
adults and will likely be increasingly used amongst pediatric Kawasaki patients,
and this may result in better identification and then treatment
with things like beta blockers, cholesterol lowering medications, and ACE inhibitors,
meaning that its hope that with improved surveillance techniques, outcomes
will also long term improve The authors re emphasize that
(08:09):
myocarl in fractions occurred only in patients with giant aneurysms
and how seventy five percent of patients had their mycaralinfarction
in the first two years after presentation. They review how
these findings are consistent with prior Japanese reports on this topic.
They speak about how even being on antiquagulation was not
uniformly protective of myocarlin fraction in the setting of giant aneurysms,
(08:33):
and how newer direct oral antiquagulants are being increasingly used
due to compliance and ability to be therapeutic more consistently,
and how data suggests that results have been quite good
using these newer agents, and they suggest that improved methods
of thromboprophylaxis and management of myocarl infraction in the current
era may lead to better outcomes. The authors discuss computational
(08:57):
modeling in Kawasaki disease patients with carnarya aneurysms and how
minor differences in morphology and shape and size of these
aneurysms may be associated with important differences in risk for thrombosis,
and wonder if this sort of modeling might in the
future result in better, more individualized therapies for anticoagulation in
the Kawasaki disease patient with large aneurysms. The authors speak
(09:20):
of the limitations of a retrospective study design in a
single center, and also the fact that the vast majority
of patients in this work were initially treated elsewhere, not
at Boston Children's Hospital, making risk factor analysis challenging. They
also speak about the absence of serial follow up in
some of the patients, making long term efficacy of these
approaches difficult to assess. In this work. They end by
(09:43):
suggesting that pediatric caregivers should be educated about the signs
and symptoms of myocarlin fraction and children so that timely
treatment can be initiated, and they suggest that further research
on who should be surveilled for caronary thrombosis is needed
so as to better identif to who should receive enhance
surveillance and who more aggressive anti thrombotic treatment. Well, this
(10:06):
is an interesting report on a very rare group of
patients who have had a myacardalan farction following Kawasaki disease
with giant aneurysms. As I read this, I wondered why
it seems that patients today have done on average better
than in the past. The authors suggest that we are
better in the ICU and that this may explain the differences,
but I wonder if there are other factors. I also
(10:29):
wondered about the last comments of the authors that I've
just read to you regarding how we should better educate
pediatric providers about the signs or symptoms of mycarland fraction
in Kawasaki disease. It seems to me that most of
these signs and symptoms were quite nonspecific, and I do
wonder what doctor Galani and colleagues mean by this recommendation.
(10:49):
In the interest of time, therefore, let's move straight on
to our conversation with the work's first author, doctor Suneil Galani,
dreating us now to discuss this week's work. Is the
work's first author, Doctor Suneil doctor Glani is a senior
Associate cardiologist at Boston Children's Hospital and Associate Professor of
Pediatrics at the Harvard Medical School. Within the Division of
(11:09):
Cardiac Imaging. He serves as the Director of CARDIACCT specializing
in the use of advanced imaging modalities for diagnosing and
managing complex congel heart disease. Doctor Glaney performed his residency
at Children's National Medical Center in Washington, DC, and following this,
completed fellowship in general pediatric cardiology as well as cardiac imaging.
(11:30):
It is a delight to welcome him to the podcast.
Welcome doctor Galani to PD Heart.
Speaker 3 (11:34):
I'm here now with doctor Sunil Glani of Boston Children's Hospital.
Doctor Glani, thank you very much for joining us this
week on PDHART.
Speaker 2 (11:41):
Thank you doctor Pasman writing me. I'm honored and I
wanted to thank you for doing these podcasts over all
these years.
Speaker 4 (11:47):
Thank you.
Speaker 3 (11:48):
It's very kind of you to say thank you. You know,
doctor Glani really enjoyed this work, very important work on
a very rare topic. You know. I was wondering amongst
this extremely challenging group of patients, I thought it was
noteworthy that only two patients, despite having a terrible situation
of having an MI, actually died, and both were in
(12:10):
the earliest period of this very long series that you report,
And I wondered what you attribute the fact that in
the recent present era it seems like mortality is substantially
less common following an MI in patients who've had Kawasaki disease.
Speaker 4 (12:25):
Thank you.
Speaker 2 (12:25):
That is an important observation, you know. Before going into why,
I would like to have one note of caution. You know,
almost all the patients in our study were referred after
surviving their initial mi I, so this introduces a survival bias,
obviously and should not be generalized. Having said that, the
(12:45):
overall survival after m I and KD is probably much
better these days compared to prior like in the eighties,
you know, and I would attribute that to the progress
we've made in karia critical care as well as early
recognition and treatment, including anti cooogulation strategies that have come by.
(13:08):
You know, not all ams that equal. You know, some
AM is a large proximilarity in fraction, for example, and
it would depend on how sick the patient is at presentation,
whether they can make it or not.
Speaker 3 (13:21):
Yeah, yeah, good points. All. I didn't even think of
that survival bias, which is obviously very important. You know,
of the thirty two percent of the patients, or seven
of twenty two, only that small number had any sort
of symptoms. And this is somewhat lower than we would
expect in older patients or having an MI. In some
(13:43):
it would be understandable obviously, given this the low age
of the patients. However, I'm wondering if you have any
thoughts on why so many are not presenting with any
symptoms that we would commonly attribute to an MI. Are
they not having symptoms? Are we not appreciating the symptoms.
I'm wondering if any of this changes how you think
(14:04):
about surveilling this group of patients with giant aneurysms.
Speaker 4 (14:09):
Yeah, and again a very good point.
Speaker 2 (14:11):
I think you know, I was surprised when I saw
that too, that only seven had you know, symptoms, But
I think it's more likely that the symptoms are not
recognized because they are atypical. As you know, you know,
our population is very young, and in majority of these
ams occur early after KD or a cute KTE. So
symptoms like fatigue, vomiting, and even earache and neck pain
(14:33):
in one of our patients, all of these are common
in childhood and can be missed as signs of m
I that you know, some of the mis at least
appear to be truly asymptomatic, which is interesting because these
were discovered incidentally as a for example, a scar or
an MRI. You know, it can be really difficult to
piece together prior history, especially when am I occurred, you know,
(14:58):
maybe a year or two or three year. Now that
it's a dense car on the on the MRI, it
is possible that there were some settle symptoms that were
kind of not recognized, but at least in our charter view,
you know, some of the patients were actually truly asymptomatic.
Speaker 3 (15:13):
Very interesting, you know, I noticed that carnary artery bypass
graphs were used much more commonly in this patient group
than antioplasty or stenting for these patients, and I'm wondering,
if you might for the audience review with us, what
the rationale for this was. Is it because of the
small size of carnary arteries and availability of stats or
(15:36):
catheters for this to maintain patency? Or is it something
related to the biology of the type of stenosis and
obstructions that we see in Kawasaki disease.
Speaker 4 (15:48):
I think I think both of those are true.
Speaker 2 (15:50):
I think, you know, obviously, patient size and vessel size
are the primary.
Speaker 4 (15:54):
Issues in our young you know, young gohrd.
Speaker 2 (15:58):
But like you said, you know, the nature of coronary
artery disease in Kawasaki disease is fundamentally different.
Speaker 4 (16:04):
Unlike build up of lack and.
Speaker 2 (16:06):
Calcification in a well defined lesion, in an adult am
I lesions are preceded by gina aneurysms. Often they have
multiple areas of stenosis and then hence require different strategy
and a single stent is not often sufficient. Another issue
is long term durability, you know, and for example, an
(16:28):
internal memory graft has the potential to grow with a
child as opposed to a stent. If you implant it
at say three years of age, what's going to happen
to it in two or three years. Again, you can't
keep dilating it successfully indefinitely. So the decision to use
cabbage versus stent is still individualized, considering factors such as
multi vessel disease, location and severity of the stenosis, patient age,
(16:52):
as well as the surgical risk. You know, if somebody
who has a higher surgical risk, one might opt to
do a suboptimal but still do a stent instead.
Speaker 4 (17:01):
Yeah, it makes all makes good sense.
Speaker 3 (17:02):
You know. I was looking at the cabbage patients and
one patient died in the operating room, one had a
cute occlusion of a bypass graft needing a repeat a cabbage,
and one had stenting of the graph seven years post surgery.
I'm assuming that most of these complications are related to
(17:22):
the small size of the blood vessels that are being
worked on by the surgeons. Really almost miraculous that they're
able to do these in these small patients. I'm wondering
if there have been any surgical insights that have developed
over time from the surgeons that may be making this
procedure safer in the future. Have there been tricks that
they've learned to perform these operations in very small patients.
Speaker 4 (17:47):
Yeah, no, that's a good question.
Speaker 2 (17:48):
I think, you know, cabbage and an acutely ill katy
patient is a high risk corporation. And you know, the
patient who died actually had a large infection and was
very sick going in. I don't know that the surgical
techniques have really evolved, you know, to be much better.
I think, you know, microvascular surgery has gotten better, but
(18:11):
I think the need for reintervention is definitely high. You know,
in our in our cohort, you know, we had a
handful of patients with cabbage, but you know, we can
learn much more from the Japanese data. For example, Kitamura
and colleagues have followed over one hundred patients with KDI
who underwent cabbage over the last several decades, and they've
shown ninety five percent survival over the twenty five years of.
Speaker 4 (18:34):
Follow up or so.
Speaker 2 (18:35):
However, if you read through the paper, about twenty three
percent of them required either a second cabbage or a
PCI or something like that. So these these don't last forever,
and requiring reintervention is something to almost expect in one
in four patients. I think, you know, closer monitoring of
these patients with interval cts mrs cathorizations. These should guide
(18:58):
the timing and nature of the intervention treats when necessary,
so close follow up is what is essential after doing
original cabbage.
Speaker 3 (19:05):
Yeah, certainly would seem the case well for those in
the audience. I got doctor Glana to speak with us
smack deev in the middle of a busy afternoon. So
I'm going to finish up with the most provocative of
the questions today. You know, doctor Glani, you and your
colleagues ended the work by suggesting that pediatric providers should
be taught the signs of m I and children so
(19:28):
as to potentially identify earlier patients who are at risk
for this obviously potentially lethal outcome of kawsaka disease. I'm
wondering if you could share with us, what are those
signs that you think should be taught to practitioners that
are being missed.
Speaker 4 (19:44):
It's a good point.
Speaker 2 (19:44):
Maybe we could awarded it a little different. It's so
much teaching the signs. I admit that I think the
key take of it is to maintain.
Speaker 4 (19:53):
A high index of suspicion.
Speaker 2 (19:55):
You know, any symptom that is otherwise unexplained settles seemingly
unrelated could actually be a sign of am I.
Speaker 4 (20:04):
So just have that high index.
Speaker 2 (20:05):
Of suspicion and to that, and I want to point
out that you know, all of our patients where.
Speaker 4 (20:11):
Am I had giant aneurysm, so.
Speaker 2 (20:13):
That suspicion should be much higher for patients who have
giant aneurysms. Put differently, nobody without giant aneurysm had an
m in I. Cohort also wanted to highlight that about
half of the mis occurred within the first three months
of a cute KD, but also remember that about one
(20:33):
in five or twenty percent were late, that is to say,
after two years of a cute KD.
Speaker 4 (20:38):
So this could happen at any time.
Speaker 2 (20:40):
And so having that high index of suspicion if you've
ever had a giant coronary aneurysm is what I would
kind of, you know, want the additions to take away from.
Speaker 3 (20:50):
This, well, I think actually very important point you just
made about having a high insect of suspicion. I think
that really is you know, amazing, amazing that you found
in your group twenty two am I patients after Kawasaki
disease and equally amazing that you were able to get
the data on patients from as early as the early
(21:12):
nineteen eighties. I really think this has added substantially to
the literature of Kawasaki disease. Definitely a scary group of
patients for sure. I want to thank you, doctor Galani,
and I want to congratulate you and all of your
many co investigators at Boston Children's Hospital for really important
and revealing paper. Thank you very much.
Speaker 4 (21:34):
Well, thank you so much.
Speaker 2 (21:35):
I had a lot of fun talking to you as
well as actually doing charterview for the this patient. I
want to say you know, it was fun finding the
paper chart written by fellow doctor tal Gavi and attending
doctor Richard vanfrag in the pathology pathology records, So it
was a lot of deep digging into the paper charts,
(21:58):
but it was fun.
Speaker 3 (21:59):
I guess that's what happens. We need to do a
paper that has thirty nine years of follow up, So
thank you very much. A great pleasure to do it
this week.
Speaker 1 (22:08):
I'm sure that you enjoyed this brief conversation as much
as I did, and doctor Glani had many important points
to make. I thought one of the most important things
he had to say were his comments about having a
high index of suspicion for maa cardalin farction amongst patients
with larger gigantic aneurysms. Though the precise specific signs or
symptoms of m I in our pediatric Kawasaki patients may
(22:31):
be somewhat challenging to parse out, it seems clear that
we all need to have a very high index of
suspicion for many different signs or symptoms as he suggested.
I also thought his emphasis regarding the timing of m
I in the setting of Kawasaki disease, with most being
in the first months of the disease but still roughly
twenty percent later, to be very important for us all.
(22:54):
Once again, I'd like to thank him for taking time
from his very busy schedule to speak with us this
week on Pdhart. To conclude this three hundred and fifty
fifth episode of Pediheart Pediatric Cardiology. Today we end with
the fiendishly difficult aria come scoglio from Act one of
Mozart's Cosi Fantute, and we hear this aria sung by
(23:14):
the wonderful Hungarian German soprano Julia Vardi, who sang throughout
Europe in all of the major European opera houses, and
who today is in her retirement from active singing, but
remains a professor of singing in Berlin. Thank you very
much for joining us for this three hundred and fifty
fifth episode, and thanks once again to doctor Glauni for
his insights. I hope we'll have a good week ahead.
Speaker 5 (23:36):
Oh mar.
Speaker 6 (23:58):
Ah mist cool, the mist waiting for things the party
(24:42):
if your pity j.
Speaker 3 (24:46):
So it.
Speaker 6 (24:58):
Is sad to good cold schooling stall story, Ah stop.
Speaker 7 (26:12):
West, He's me.
Speaker 5 (26:46):
Rist said, he goes un top.
Speaker 6 (26:52):
On top. Don't hold, no hold would know
Welcome to Pdheart 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, where I am the chief of Pediatric
Cardiology and the co director of the Children's Heart Center.
Thank you for joining me for this three hundred and
fifty fifth episode of Pdheart. I hope everybody enjoyed last
week's episode, in which we spoke with doctor Cohne and
(00:38):
doctor Zeidi about neurocognitive dysfunction amongst adult congenital heart patients.
For those of you interested in this important topic, i'd
recommend you take to listen to last week's episode three
hundred and fifty four. As I say most weeks, if
you'd like to get in touch with me, my email
is easy to remember. It's Pdheart at gmail dot com.
This week we move into the world of Kawasaki disease,
(01:00):
which seems appropriate since we are hosting a Kawasaki program
symposium at Mount Sinai on Friday and Saturday of this week.
The title of the work we'll be reviewing is maya
cardial infarction in Kawasaki disease. The first author of this
work is Sunil Ghilani and the senior author Jane Neuberger.
And the authors come to us from the Department of
Cardiology at the Children's Hospital Boston. When we're done reviewing
(01:24):
this paper, the work's first author, doctor Glani, has graciously
great to speak with us about it. Therefore, let's move
straight onto this article and then a conversation with doctor Galani.
This week's work begins with a review of Kawasaki disease,
reminding us that it is the leading cause of acquired
heart disease in the developed world, that it is most
common in children less than five years of age, and
(01:44):
they review the common presenting signs including fever, rash, non
exudative conjunctiveitis, inflammation of the oral mukosa, unilateral cervical at anopathy,
and extremity findings. They then review the fact that up
to one quarter of patients with Kawasaki disease will develop
carnary aneurisms if timely administration of IVIIG is not performed.
(02:05):
Because of what we know about this and the timely
interventions that are typically performed, only about one percent of
KD patients develop large or giant carinary aneurisms, and we
are all aware that these can predispose children to thrombosis, stenosis, myacardalinfarction,
and even sudden death. The authors then review the facts
that because my carlin farction is thankfully rare in this setting,
(02:29):
diagnosis of mi I and small children and the appropriate
treatment is challenging given the dearth of information regarding mi
I in this setting. The authors note that there are
a few reports of mi in Kawasaki disease in Japan,
but thus far no such reports in North America. With
this as a background, the authors explained that the goal
of this work was to review patient characteristics, clinical course,
(02:53):
and outcomes following my cardalin farction secondary to Kawasaki disease
in a very large, single center cohort. This was a
retrospective study of all patients with the history of Kawasaki
disease and mycarial infarction who were evaluated and treated at
Boston Children's Hospital from January of nineteen eighty four to
January of twenty twenty three, in other words, over a
(03:14):
thirty nine year period. The authors explained what an acte
MII was defined as for this study and suffice it
to say that the definition is robust and it would
seem unlikely that anyone would be in that category in
this study without an actual mi The authors reviewed the
data that they were collecting, including massive amounts of clinical
data such as age at the time of onset of
(03:34):
Kawasaki disease, time to the first IVIIG treatment, number of
courses of IVIG, use of steroids or other immunomodulators, as
well as antithrombotic or thrombolytic therapy use. They recorded all
sorts of morphological aspects of the caronaries and the aneurysms themselves,
and of course reviewed in detail the clinical courses of
(03:55):
these patients, trying to determine if bypass graphs or interventions
were needed or even ICD implantations and dawn to the results.
Speaker 2 (04:03):
Well.
Speaker 1 (04:04):
Over this long thirty nine year period, there were twenty
two patients who met inclusion criteria, and there was a
significant preponderance of males at eighty six percent with the
median onset of Kawasaki disease at fourteen months, So again
there were twenty two total Kawasaki disease mi I patients
over forty years at Boston Children's Hospital, the median age
(04:24):
at Kawasaki disease onset was fourteen months, with a range
of three months to fifteen years. Twenty of the twenty
two patients had received IVIIG, with initial treatment occurring at
a median day of illness of ten, with a range
of four to forty two days, with six of twenty
two having had their first IVIIG at less center equal
to day seven of illness, and three of twenty two
(04:47):
on days eight to ten of illness. For the two
patients who did not receive ivig one at Kawasaki disease,
presenting in nineteen eighty four and the other presented in
nineteen ninety nine with a remote history of what was
likely missed Kawasaki disease in infancy of note ten of
the twenty two patients received a second dose of IPIG
(05:07):
and three patients a third dose. So in general these
were difficult patients to treat. And when did the mis occur, well,
forty eight percent occurred within three months of acute Kawasaki
disease and ninety one percent within two years, and so
it does seem that getting past two years without this
complication is perhaps somewhat reassuring. All of the patients in
(05:29):
this cohort had at least one large or giant carnary
artery aneurysm, with most common being the proximal left anterior
descending coronary. The majority, or eighty five percent, had bilateral,
larger giant aneurysms. Of note, only thirty two percent or
seven patients actually had symptoms at the time of the
acute MI with four at the time of MI and
(05:51):
three in retrospect, and their symptoms were fatigue into chest
pain in one, syncope in one, ear pain in one,
upper extremity pain in one, and vomiting in one. And
how were these diagnosed while imaging either echo or MRI
contributed to the diagnosis in fifty percent and what happened
to these patients? While seven patients had caronary artery bypass grafting,
(06:16):
one had a percutaneous carnary intervention, and one was listed
for transplant. Two patients in this cohort died from mycardalin
farction related complications, but these were both patients in the
nineteen eighties, and twenty three percent of these mi I
patients had LV dysfunction at follow up importantly, ventricular at
tachycardia was seen in three patients, two of whom received
(06:38):
an ICD. In their discussion, the author's state and I
quote myocardalin friction is the leading cause of death in
patients with Kawasaki disease and occurs almost solely in patients
with large giant carnary aneurysms, defined as those having a
Z score greater than are equal to ten or absolute
diameter of eight millimeters. The incidents of micarl ourlin fraction
(07:00):
peaks in the first few months after Kawasaki disease onset
and declined substantially after two years from disease onset, but
sudden death from myacarlin fraction can occur later in life. Indeed,
six point seven percent and five percent of adults less
than forty years of age with acute carnary syndromes in
Egypt and San Diego, respectively at carnary aneurysms and stenoses
(07:23):
consistent with Kawasaki disease early in life. The authors speak
about the nonspecific nature of the symptoms of the myacarlinfraction
patients in this work and orfer this as a reason
for why these were often missed. They note that even
amongst adults, it's not uncommon to have found that an
am I may have occurred in the past without obvious
symptoms in real time. The authors explain that CMR is
(07:46):
a good test to surveil for a cult MI in
adults and will likely be increasingly used amongst pediatric Kawasaki patients,
and this may result in better identification and then treatment
with things like beta blockers, cholesterol lowering medications, and ACE inhibitors,
meaning that its hope that with improved surveillance techniques, outcomes
will also long term improve The authors re emphasize that
(08:09):
myocarl in fractions occurred only in patients with giant aneurysms
and how seventy five percent of patients had their mycaralinfarction
in the first two years after presentation. They review how
these findings are consistent with prior Japanese reports on this topic.
They speak about how even being on antiquagulation was not
uniformly protective of myocarlin fraction in the setting of giant aneurysms,
(08:33):
and how newer direct oral antiquagulants are being increasingly used
due to compliance and ability to be therapeutic more consistently,
and how data suggests that results have been quite good
using these newer agents, and they suggest that improved methods
of thromboprophylaxis and management of myocarl infraction in the current
era may lead to better outcomes. The authors discuss computational
(08:57):
modeling in Kawasaki disease patients with carnarya aneurysms and how
minor differences in morphology and shape and size of these
aneurysms may be associated with important differences in risk for thrombosis,
and wonder if this sort of modeling might in the
future result in better, more individualized therapies for anticoagulation in
the Kawasaki disease patient with large aneurysms. The authors speak
(09:20):
of the limitations of a retrospective study design in a
single center, and also the fact that the vast majority
of patients in this work were initially treated elsewhere, not
at Boston Children's Hospital, making risk factor analysis challenging. They
also speak about the absence of serial follow up in
some of the patients, making long term efficacy of these
approaches difficult to assess. In this work. They end by
(09:43):
suggesting that pediatric caregivers should be educated about the signs
and symptoms of myocarlin fraction and children so that timely
treatment can be initiated, and they suggest that further research
on who should be surveilled for caronary thrombosis is needed
so as to better identif to who should receive enhance
surveillance and who more aggressive anti thrombotic treatment. Well, this
(10:06):
is an interesting report on a very rare group of
patients who have had a myacardalan farction following Kawasaki disease
with giant aneurysms. As I read this, I wondered why
it seems that patients today have done on average better
than in the past. The authors suggest that we are
better in the ICU and that this may explain the differences,
but I wonder if there are other factors. I also
(10:29):
wondered about the last comments of the authors that I've
just read to you regarding how we should better educate
pediatric providers about the signs or symptoms of mycarland fraction
in Kawasaki disease. It seems to me that most of
these signs and symptoms were quite nonspecific, and I do
wonder what doctor Galani and colleagues mean by this recommendation.
(10:49):
In the interest of time, therefore, let's move straight on
to our conversation with the work's first author, doctor Suneil Galani,
dreating us now to discuss this week's work. Is the
work's first author, Doctor Suneil doctor Glani is a senior
Associate cardiologist at Boston Children's Hospital and Associate Professor of
Pediatrics at the Harvard Medical School. Within the Division of
(11:09):
Cardiac Imaging. He serves as the Director of CARDIACCT specializing
in the use of advanced imaging modalities for diagnosing and
managing complex congel heart disease. Doctor Glaney performed his residency
at Children's National Medical Center in Washington, DC, and following this,
completed fellowship in general pediatric cardiology as well as cardiac imaging.
(11:30):
It is a delight to welcome him to the podcast.
Welcome doctor Galani to PD Heart.
Speaker 3 (11:34):
I'm here now with doctor Sunil Glani of Boston Children's Hospital.
Doctor Glani, thank you very much for joining us this
week on PDHART.
Speaker 2 (11:41):
Thank you doctor Pasman writing me. I'm honored and I
wanted to thank you for doing these podcasts over all
these years.
Speaker 4 (11:47):
Thank you.
Speaker 3 (11:48):
It's very kind of you to say thank you. You know,
doctor Glani really enjoyed this work, very important work on
a very rare topic. You know. I was wondering amongst
this extremely challenging group of patients, I thought it was
noteworthy that only two patients, despite having a terrible situation
of having an MI, actually died, and both were in
(12:10):
the earliest period of this very long series that you report,
And I wondered what you attribute the fact that in
the recent present era it seems like mortality is substantially
less common following an MI in patients who've had Kawasaki disease.
Speaker 4 (12:25):
Thank you.
Speaker 2 (12:25):
That is an important observation, you know. Before going into why,
I would like to have one note of caution. You know,
almost all the patients in our study were referred after
surviving their initial mi I, so this introduces a survival bias,
obviously and should not be generalized. Having said that, the
(12:45):
overall survival after m I and KD is probably much
better these days compared to prior like in the eighties,
you know, and I would attribute that to the progress
we've made in karia critical care as well as early
recognition and treatment, including anti cooogulation strategies that have come by.
(13:08):
You know, not all ams that equal. You know, some
AM is a large proximilarity in fraction, for example, and
it would depend on how sick the patient is at presentation,
whether they can make it or not.
Speaker 3 (13:21):
Yeah, yeah, good points. All. I didn't even think of
that survival bias, which is obviously very important. You know,
of the thirty two percent of the patients, or seven
of twenty two, only that small number had any sort
of symptoms. And this is somewhat lower than we would
expect in older patients or having an MI. In some
(13:43):
it would be understandable obviously, given this the low age
of the patients. However, I'm wondering if you have any
thoughts on why so many are not presenting with any
symptoms that we would commonly attribute to an MI. Are
they not having symptoms? Are we not appreciating the symptoms.
I'm wondering if any of this changes how you think
(14:04):
about surveilling this group of patients with giant aneurysms.
Speaker 4 (14:09):
Yeah, and again a very good point.
Speaker 2 (14:11):
I think you know, I was surprised when I saw
that too, that only seven had you know, symptoms, But
I think it's more likely that the symptoms are not
recognized because they are atypical. As you know, you know,
our population is very young, and in majority of these
ams occur early after KD or a cute KTE. So
symptoms like fatigue, vomiting, and even earache and neck pain
(14:33):
in one of our patients, all of these are common
in childhood and can be missed as signs of m
I that you know, some of the mis at least
appear to be truly asymptomatic, which is interesting because these
were discovered incidentally as a for example, a scar or
an MRI. You know, it can be really difficult to
piece together prior history, especially when am I occurred, you know,
(14:58):
maybe a year or two or three year. Now that
it's a dense car on the on the MRI, it
is possible that there were some settle symptoms that were
kind of not recognized, but at least in our charter view,
you know, some of the patients were actually truly asymptomatic.
Speaker 3 (15:13):
Very interesting, you know, I noticed that carnary artery bypass
graphs were used much more commonly in this patient group
than antioplasty or stenting for these patients, and I'm wondering,
if you might for the audience review with us, what
the rationale for this was. Is it because of the
small size of carnary arteries and availability of stats or
(15:36):
catheters for this to maintain patency? Or is it something
related to the biology of the type of stenosis and
obstructions that we see in Kawasaki disease.
Speaker 4 (15:48):
I think I think both of those are true.
Speaker 2 (15:50):
I think, you know, obviously, patient size and vessel size
are the primary.
Speaker 4 (15:54):
Issues in our young you know, young gohrd.
Speaker 2 (15:58):
But like you said, you know, the nature of coronary
artery disease in Kawasaki disease is fundamentally different.
Speaker 4 (16:04):
Unlike build up of lack and.
Speaker 2 (16:06):
Calcification in a well defined lesion, in an adult am
I lesions are preceded by gina aneurysms. Often they have
multiple areas of stenosis and then hence require different strategy
and a single stent is not often sufficient. Another issue
is long term durability, you know, and for example, an
(16:28):
internal memory graft has the potential to grow with a
child as opposed to a stent. If you implant it
at say three years of age, what's going to happen
to it in two or three years. Again, you can't
keep dilating it successfully indefinitely. So the decision to use
cabbage versus stent is still individualized, considering factors such as
multi vessel disease, location and severity of the stenosis, patient age,
(16:52):
as well as the surgical risk. You know, if somebody
who has a higher surgical risk, one might opt to
do a suboptimal but still do a stent instead.
Speaker 4 (17:01):
Yeah, it makes all makes good sense.
Speaker 3 (17:02):
You know. I was looking at the cabbage patients and
one patient died in the operating room, one had a
cute occlusion of a bypass graft needing a repeat a cabbage,
and one had stenting of the graph seven years post surgery.
I'm assuming that most of these complications are related to
(17:22):
the small size of the blood vessels that are being
worked on by the surgeons. Really almost miraculous that they're
able to do these in these small patients. I'm wondering
if there have been any surgical insights that have developed
over time from the surgeons that may be making this
procedure safer in the future. Have there been tricks that
they've learned to perform these operations in very small patients.
Speaker 4 (17:47):
Yeah, no, that's a good question.
Speaker 2 (17:48):
I think, you know, cabbage and an acutely ill katy
patient is a high risk corporation. And you know, the
patient who died actually had a large infection and was
very sick going in. I don't know that the surgical
techniques have really evolved, you know, to be much better.
I think, you know, microvascular surgery has gotten better, but
(18:11):
I think the need for reintervention is definitely high. You know,
in our in our cohort, you know, we had a
handful of patients with cabbage, but you know, we can
learn much more from the Japanese data. For example, Kitamura
and colleagues have followed over one hundred patients with KDI
who underwent cabbage over the last several decades, and they've
shown ninety five percent survival over the twenty five years of.
Speaker 4 (18:34):
Follow up or so.
Speaker 2 (18:35):
However, if you read through the paper, about twenty three
percent of them required either a second cabbage or a
PCI or something like that. So these these don't last forever,
and requiring reintervention is something to almost expect in one
in four patients. I think, you know, closer monitoring of
these patients with interval cts mrs cathorizations. These should guide
(18:58):
the timing and nature of the intervention treats when necessary,
so close follow up is what is essential after doing
original cabbage.
Speaker 3 (19:05):
Yeah, certainly would seem the case well for those in
the audience. I got doctor Glana to speak with us
smack deev in the middle of a busy afternoon. So
I'm going to finish up with the most provocative of
the questions today. You know, doctor Glani, you and your
colleagues ended the work by suggesting that pediatric providers should
be taught the signs of m I and children so
(19:28):
as to potentially identify earlier patients who are at risk
for this obviously potentially lethal outcome of kawsaka disease. I'm
wondering if you could share with us, what are those
signs that you think should be taught to practitioners that
are being missed.
Speaker 4 (19:44):
It's a good point.
Speaker 2 (19:44):
Maybe we could awarded it a little different. It's so
much teaching the signs. I admit that I think the
key take of it is to maintain.
Speaker 4 (19:53):
A high index of suspicion.
Speaker 2 (19:55):
You know, any symptom that is otherwise unexplained settles seemingly
unrelated could actually be a sign of am I.
Speaker 4 (20:04):
So just have that high index.
Speaker 2 (20:05):
Of suspicion and to that, and I want to point
out that you know, all of our patients where.
Speaker 4 (20:11):
Am I had giant aneurysm, so.
Speaker 2 (20:13):
That suspicion should be much higher for patients who have
giant aneurysms. Put differently, nobody without giant aneurysm had an
m in I. Cohort also wanted to highlight that about
half of the mis occurred within the first three months
of a cute KD, but also remember that about one
(20:33):
in five or twenty percent were late, that is to say,
after two years of a cute KD.
Speaker 4 (20:38):
So this could happen at any time.
Speaker 2 (20:40):
And so having that high index of suspicion if you've
ever had a giant coronary aneurysm is what I would
kind of, you know, want the additions to take away from.
Speaker 3 (20:50):
This, well, I think actually very important point you just
made about having a high insect of suspicion. I think
that really is you know, amazing, amazing that you found
in your group twenty two am I patients after Kawasaki
disease and equally amazing that you were able to get
the data on patients from as early as the early
(21:12):
nineteen eighties. I really think this has added substantially to
the literature of Kawasaki disease. Definitely a scary group of
patients for sure. I want to thank you, doctor Galani,
and I want to congratulate you and all of your
many co investigators at Boston Children's Hospital for really important
and revealing paper. Thank you very much.
Speaker 4 (21:34):
Well, thank you so much.
Speaker 2 (21:35):
I had a lot of fun talking to you as
well as actually doing charterview for the this patient. I
want to say you know, it was fun finding the
paper chart written by fellow doctor tal Gavi and attending
doctor Richard vanfrag in the pathology pathology records, So it
was a lot of deep digging into the paper charts,
(21:58):
but it was fun.
Speaker 3 (21:59):
I guess that's what happens. We need to do a
paper that has thirty nine years of follow up, So
thank you very much. A great pleasure to do it
this week.
Speaker 1 (22:08):
I'm sure that you enjoyed this brief conversation as much
as I did, and doctor Glani had many important points
to make. I thought one of the most important things
he had to say were his comments about having a
high index of suspicion for maa cardalin farction amongst patients
with larger gigantic aneurysms. Though the precise specific signs or
symptoms of m I in our pediatric Kawasaki patients may
(22:31):
be somewhat challenging to parse out, it seems clear that
we all need to have a very high index of
suspicion for many different signs or symptoms as he suggested.
I also thought his emphasis regarding the timing of m
I in the setting of Kawasaki disease, with most being
in the first months of the disease but still roughly
twenty percent later, to be very important for us all.
(22:54):
Once again, I'd like to thank him for taking time
from his very busy schedule to speak with us this
week on Pdhart. To conclude this three hundred and fifty
fifth episode of Pediheart Pediatric Cardiology. Today we end with
the fiendishly difficult aria come scoglio from Act one of
Mozart's Cosi Fantute, and we hear this aria sung by
(23:14):
the wonderful Hungarian German soprano Julia Vardi, who sang throughout
Europe in all of the major European opera houses, and
who today is in her retirement from active singing, but
remains a professor of singing in Berlin. Thank you very
much for joining us for this three hundred and fifty
fifth episode, and thanks once again to doctor Glauni for
his insights. I hope we'll have a good week ahead.
Speaker 5 (23:36):
Oh mar.
Speaker 6 (23:58):
Ah mist cool, the mist waiting for things the party
(24:42):
if your pity j.
Speaker 3 (24:46):
So it.
Speaker 6 (24:58):
Is sad to good cold schooling stall story, Ah stop.
Speaker 7 (26:12):
West, He's me.
Speaker 5 (26:46):
Rist said, he goes un top.
Speaker 6 (26:52):
On top. Don't hold, no hold would know
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