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November 14, 2025 17 mins
This week we review a recent retrospective review from Phoenix Children’s Hospital about coronary artery fistulae. How common are they and who needed intervention? How should the cardiologist think about the small fistula? Should they all be ‘followed’ or can they be discharged from cardiology follow-up? How small is ‘small enough’? We speak with Dr. Marie Chevenon who is a fetal cardiologist at Phoenix Children’s Hospital about her recent study on the Phoenix experience with coronary artery fistulae.  

https://doi.org/10.1007/s00246-024-03600-y
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Episode Transcript

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Speaker 1 (00:16):
Welcome to Pdiheart 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'm also the Chief of Pediatric Cardiology.
Thank you for joining me for this three hundred and
sixty second episode of Pdheart. I hope everybody enjoyed last
week's episode on the topic of adult congenital heart disease

(00:37):
and what a medication list might tell us about our
adult congenital patients. For those of you interested in adult
congenital heart disease, I would certainly recommend you tech to
listen to last week's episode three hundred and sixty one.
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

(00:57):
into the world of general cardiology. The title the work
will be reviewing is Single Center Retrospective Evaluation of Carnary
Artery Fistula Outcomes.

Speaker 2 (01:06):
The first author is M. Chevanon and the senior author T. Nolan,
and this work comes to us from Phoenix Children's Hospital
in Phoenix, Arizona. When we're done reviewing this paper, I'm
hopeful that doctor Chevanon will be available to speak with
us about it. Therefore, let's move straight down to this
article and then a brief conversation with its author. This
week's work starts by reviewing that carnary artery fistulae are

(01:28):
rare and they represent only about zero point two percent
of all congenital heart defects, and the authors review what
we all know, which is that most pediatric patients are
asymptomatic and these usually do not require intervention long term,
particularly larger ones, these can be associated with mycarialischemia from
carnary steel, as well as endocarditis, congestive heart failure, or arrhythmias.

(01:52):
They point to prior studies that suggest that these can
be associated with other congenital heart lesions in between twenty
and forty percent of cases. The authors are viewed that
when they need to be closed, the options are either
surgery or transcatheter solutions, and both are associated with good outcomes.
The American Heart Association and American College of Cardiology Adult
Guidelines specified that percutaneous or surgical closure would be a

(02:16):
Class one recommendation for large fistulae with evidence of a schemia, arrhythmia,
ventricular dysfunction, enlargement, or endarteritis. In children, we typically would
refer for closure if there were clinical symptoms like poor
weight gain to kipnia, arrhythmias, or ventricular dysfunction. The authors
then reference a recent study showing that pediatric fistula repair

(02:38):
was associated with lower major complication rates when it was
performed via surgery versus transcatheter fourteen point five percent for
surgery versus thirty four point five percent, with major complications
in that study being myocardialischemia, residual shunts, or nuancet valular regurgitation.
In another study, transcatheter closure of sixty one patients will

(03:00):
reviewed and in that work there were three myocardial infarctions,
three device migrations, and one case of legoschemia. There was
even one death after surgical closure in a distal carnary
fistula that could not be closed in the cathlab. The
authors mention how there is little data on what happens
to smaller fistulae that are not addressed with surgery or

(03:20):
cath and they remind that there are no published recommendations
on how to follow these patients. With this as a background,
the authors explained that their hypothesis was that tiny or
small carnary outery fistulae would not increase over time, and
the aim was to characterize the natural history and outcomes
of patients with the diagnosis of a carnary utery fistula
at a single center over ten years, including both those

(03:43):
who underwent intervention and those managed conservatively. The authors performed
a retrospective review of their experience with carnary utery fistulae
over ten years at Phoenix Children's Hospital, and all of
the expected clinical data were obtained, including echo information, demographics,
any interventions performed, heart function, and dimensions, and the authors
defined carnaryoory fistulae as tiny if there was tiny flow

(04:06):
scene on color Doppler, small if it was a small
degree of flow or one to one point nine millimeters,
Moderate would be two to three point nine by their definition,
and large greater than equal to four millimeters, and in
Figure one the authors give us some echo examples of
each and all to the results. Well. There were one
hundred and fifty eight patients in this work and table

(04:27):
one reviews their demographics. The mean age at diagnosis was
five point eight years, and males made up fifty five
percent of the cohort. Interestingly, forty nine percent of this group,
or seventy seven had an associated congenital defect, but of
course this is certainly a result of ascertainment bias. The
two most common indications for an ECHO was congenital heart
disease that was known in thirty two percent and a

(04:49):
murmur in twenty nine percent. A genetic abnormality was seen
in eighteen percent of the patients, with trisomy twenty one
being the most common. Importantly, no patient in this work
had a scheme changes on ECG or on an ECG
stress test when it was performed, as it was in
twenty two patients in this cohort. Though two patients developed
either non specific TEA wave inversions over time and or

(05:12):
st depression in the anthilateral leads, which resolved on other
resting electro cardiograms. And So, what were the most important findings?
I think perhaps the most important finding first of all
was that in general, during the follow up period of
this study, which was five plus or minus three point
eight years, the vast majority of ninety four percent one
hundred and forty nine of one hundred and fifty eight

(05:33):
did not undergo any intervention on their caronary arteries. In
the small subset that required an intervention, of which there
were seven, two at surgical ligation and the remaining five
a catheter based approach, four were entering the right atrium
for the right ventricle and one the main pulmonary artery,
and the authors demonstrate how different sorts of devices were
used in an off label manner to close these fistulae.

(05:56):
Sixty three percent of the patients identified with a fistula
were followed by imaging, and amongst these, forty four percent
or forty one of ninety four had resolution of the
fistula on echo, eight or eight percent had a decrease
in size of the fistula, and nearly one half or
forty eight percent had no change in the size of
the fistula over time. Importantly, and perhaps not surprisingly, but

(06:19):
certainly reassuringly, amongst the small and tiny fistulae managed conservatively,
there were no significant clinical changes over time in carnary
artery dimensions, LV dimensions or LV function. In their discussion,
the authors restate the findings and also speak a bit
about the different congenital heart disease seen in the fistulae
pation in this work, and they review how they are

(06:40):
very similar to prior reports about this topic. They review
again how the vast majority did not require intervention and
how the moderate to large ones were the ones that
were closed or put on the waiting list to have
it done. And they review how there was perhaps not
surprisingly an association between the size and the likelihood of
having it closed, just that based on their study, any

(07:01):
fistula over four millimeters likely should be intervened upon and
small or tiny fistulae left alone. They review how other
measures of heart size and function did not seem to
change in the smaller fistulae patients, and importantly, how no
one who was small or tiny increased to a larger
category over time. They review how a good number of

(07:21):
these fistulae resolved with time and how this was similar
to prior reports on this topic, and they speak about
how these findings or lack of progression would argue for
conservative approaches to these They wonder allowed if less frequent
follow up, or maybe no follow up at all, is needed,
if the initial evaluation shows a very small or tiny
carnary or a fistula. The authors review the limitations, including

(07:44):
its single center retrospective design with substantial selection bias, a
follow up interval of only five years, which is reasonable
but not very long, the fact that only sixty three
percent of the patients had a follow up echo, and
the inability of this work to comment meaningfully about versus
transcathoet approaches to these anomalies given the small sample size,

(08:04):
and so they conclude surgical or interventional closure occurred only
in moderate or large carnary utery fistulas. No artery fistula
less than two millimeters required intervention. Additionally, no eschemia was
noted on ECG or ECG stress testing for carnary ri
fistula that we're not intervened on. Forty four percent underwent

(08:25):
spontaneous closure over time, and there was no significant increase
in carnary artery Z scores or left ventricular dimensions Z
scores over time. Well, to be honest, this is not
a rocket science paper, but I think sometimes it's a
value to review papers that touch on topics that are
important but maybe something we've not previously reviewed. Herein this
work supports the general approach to not intervene in carnary

(08:48):
utery fistulae unless they're large, and I think its value
is in supporting this conservative approach. As someone who closes
these in the cardiac cathlab on rare occasion, I think
it would be fair to say that the pure is
a potentially dangerous one. Yes, and the majority things go
very well. But anytime you're passing wires and catheters in
the carnary arteries, you are clearly incurring risk for the patient.

(09:12):
And so this work is of importance in making it
clear that that risk is not warranted in the small
fistula patient who is otherwise well, one in which it
can be said that the enemy of good is likely better.
In the interest of time, I think it makes sense
for us to move forward to a conversation with one
of the works authors joining us now to discuss this
week's work is Marie Chevanon. Doctor Chevanon completed her medical

(09:36):
school at Saint George's University in Grenada, followed by residency
at the University of Illinois in Peoria, and that was
followed by fellowship at Phoenix Children's Hospital, where she stayed
on to perform a fourth year fellowship in imaging. It
is a delight to have doctor Chevanon join us all
the way from Phoenix. Welcome doctor Chevanon to p de Heart.
I'm here now with doctor Marie Chevanon of Phoenix Children's Hospital.

(09:57):
Doctor Chevanon, thank you so much for joining us this
week on PETI Heart.

Speaker 3 (10:00):
Thanks so much for having me.

Speaker 2 (10:02):
Real pleasure to have you enjoyed reading your work. Congratulations
to you and your co investigators. I'm wondering sometimes when
I start out these brief interviews, I ask the author
if they might be able to summarize what they themselves
at being most conversant with the data. Believe are the
three or four take home points of your work?

Speaker 4 (10:22):
Yeah?

Speaker 3 (10:22):
Sure. I think one of the take on points is
that only moderate and large fistulas required intervention in our
study and all the small and tiny fistulas didn't. Another
point is that small and tiny coronary fistula didn't enlarge
over time. They either spontaneously closed, decreased in size, or

(10:45):
didn't change in size. And then I think lastly, the
last point was that there was no changes in the
LVIIDD scores coronary artitery dimensions or function over time for
small or tiny coronary fistulas.

Speaker 2 (11:03):
Yeah, very important points. Thank you so much. That's great.
I'm wondering doctor Chevanon is now sort of an expert
in this area. Do you believe that patients who have
small or tiny fistulae ever warnt follow up or do
you think that they can just be released from cardiology
follow up? And I'm wondering if you might be able
to offer for the audience a practical recommendation regarding the

(11:28):
evaluation or management of the small carinary fistula patient.

Speaker 3 (11:32):
I think our findings suggests that there's no clear benefit
to repeat echo over time. I'm not sure that it
can be completely definitive to release patients from cardiology. One
approach maybe to discharge patient whether it's return percussion. The
other maybe to interminently see patients every three to five

(11:52):
years without an echo.

Speaker 2 (11:54):
I see, Yeah, I guess in the end it's a
retrospective review. So there is a little bit of an
ascertainment bias because of you're basically reporting what people chose
to do and seem to.

Speaker 3 (12:06):
Work out exactly. It's hard to make a statement guideline
from the study.

Speaker 2 (12:13):
Well, for those in the audience, it's on the late
side in Arizona and even later in New York. So
I'm going to wrap up this brief interview. You know,
it wasn't the purpose of your work, but I'm wondering
if you have any thoughts on surgery versus cath that
are based approaches for the treatment of carnai fistula. And
I wondered if you knew why the two patients who

(12:34):
had surgeries in this series had a surgery versus a
cath solution.

Speaker 3 (12:39):
I think cash should be the preferred initial approach for closure,
with surgery reserved for the patients that are not amenable
for device closure. Do the patient size or fistula size
or location of the fistula. In our studies, there was
five patients who underwent device closure without major complication. One

(13:00):
of the patients who underwent device closure was noted to
have stasis during the cast in the LMCA, and then
the device was removed at the time of the calf
and then subsequently underwent surgical closure. The other patient who
underwent surgical closure in her study was a patient with

(13:21):
a large coronary fistula, but there was no documentation in
the charts on why the surgery was chosen over the
calf I sick.

Speaker 2 (13:29):
Got it interesting. Yeah, I think it would be fair
to say most of us would lean towards CAF based approaches,
even though a fair high number of complications associated with it.
But when it goes well, it's certainly a lot easier
for the patient than a big open heart surgery, for sure.

Speaker 3 (13:50):
For sure.

Speaker 2 (13:51):
Yeah. Well, doctor chevan and I really appreciate your coming
on this week and sharing with us your insights into
your work. And again I want to thank you and
can congratulate you and your co investigators on a very
interesting work.

Speaker 3 (14:03):
Thank you so much. Really appreciate you interviewing tonight.

Speaker 2 (14:07):
Thank you so much. Well, I don't have a whole
lot more to add. I think doctor Chevanon provided us
with a nice summary of her work, as well as
some good practical advice regarding how to think about particularly
our small or tiny carnary fistuly. Once again, i'd like
to thank you for taking time out of her busy
schedule late on Thursday night before the release to speak

(14:28):
with us this week on PDHART to conclude this three
hundred and sixty second episode of Pedheart Pediatric Cardiology. Today
we hear the famous third act aria of Mimi from
Labo m entitled Donde lieta Ushi, and we hear it
sung by the wonderful Armenian soprano Juliana Grigorian, who recently
is singing this very role at the Metropolitan Opera to

(14:50):
wonderful reviews. And I'm sure you will well understand why
in this live recital performance of this aria from a
competition performance a few years back. Thank you very much
for joining me for this episode, and thanks once again
to our guest. I hope everyone has a good week ahead.

Speaker 5 (15:06):
Holy shame, a little.

Speaker 6 (15:19):
Side ho.

Speaker 4 (15:49):
Sen school, little.

Speaker 5 (16:01):
Talking, not the cay she's far.

Speaker 7 (16:12):
Let me see to start cure when you're trying to
broody bread.

Speaker 4 (16:31):
In abolity.

Speaker 6 (16:38):
By the.

Speaker 5 (16:42):
Abority sorting, go to try not cit.

Speaker 6 (17:02):
Say say.

Speaker 4 (17:13):
Sadly said, b.

Speaker 3 (17:36):
What s to

Speaker 6 (17:49):
Coo
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