April 25, 2025 • 13 mins
This week we review a recent editorial commentary by Drs. Belinda Gray, Rachel Lampert and Michael Papadakis on the concept of the personalized emergency action plan for the patient with genetic heart disease who is 'returning to play' in vigorous sports. With newer data demonstrating that risk for SCA or SCD is perhaps not as high as was once thought in this setting of sport, can the development of a personalized emergency action plan further enhance safety beyond simple measures such as medical adherence? Who should be involved in drafting this emergency action plan and how does this feature in the concept of 'shared decision-making'?
DOI: 10.1161/CIRCULATIONAHA.124.072830
DOI: 10.1161/CIRCULATIONAHA.124.072830
Mark as Played
Transcript
Episode Transcript
Available transcripts are automatically generated. Complete accuracy is not guaranteed.
Speaker 1 (00:16):
Welcome to Pdiheart Pediatric Cardiology.
Speaker 2 (00:18):
Stay.
Speaker 1 (00:18):
My name is doctor Robert Pass and I'm the host
of this podcast. I am Professor of Pediatrics at the
ICONS School of Medicine at Mount Sinai, where I'm the
chief of Pediatric Cardiology. Thank you for joining me for
this three hundred and thirty eighth episode of Pdheart. I
hope everybody enjoyed last week's episode on the topic of
bunsle branch block in the single ventrical patient and an
impact it may have on exercise tolerance. For those of
(00:41):
you interested in this topic, i'd recommend you to take
a listen to last week's episode three hundred and thirty seven.
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
on to the world of genetic cardiology. The title of
the work is Return to Play with Genetic Heart Disease
(01:02):
The Importance of developing a personalized emergency action Plan. The
first author of this work is Belinda Gray, second author
Rachel Lampert, and the senior author Michael Papadakis. And the
authors come to us from Australia, New Haven and the
United Kingdom. So let's move straight on to our description
of this week's editorial comment. The authors of this editorial
(01:23):
on my Mind article begin by reviewing that sudden cardiac
death is rare, but it is the most common cause
of death for athletes during sports, and explain how traditionally
we have placed blanket restrictions on this sort of activity
for patients who have genetic heart disease due to concerns
of sudden cardiac arrest or death. They then review recent
works on genetic heart diseases that have been traditionally those
(01:47):
that were heavily restricted and explain how the recent data
show that perhaps these restrictions have been far too restrictive.
They explain that studies have demonstrated that when managed by
experts using guideline based risks stratification, most athletes with different
inherited cardiac conditions have low rates of cardiac events during sports,
(02:07):
and they reference the recent live HCM or Lifestyle and
Exercise in HCMS study demonstrating no increase in risk for
events in individuals with hypertropic cardiomyopathy who exercised at vigorous
level compared to those who are non vigorous exercisers, with
no real difference in event rates between the vigorous and
(02:27):
the non vigorous types of exercise. The authors then reference
the so called Live long QT Syndrome perspective study demonstrating
low overall rates in long QT syndrome patients with proper
contemporary management and again no differences in event rate between
vigorous and non vigorous exercisers. They then review how studies
(02:48):
with ICD implant patients, who we could assume are amongst
the highest recipatient group, have not shown serious adverse events
in competitive athletes or those participating in high risk sports
like surfing. The authors contrast these situations with something like
a rhythmogenic cardiomyopathy and particularly the PKP two mediated variety,
(03:10):
where we know that such activities as aggressive exercise can
increase the risk for heart failure, ventricularrhythmias, and sudden death.
And so they explain that basically one needs an expert
to help make decisions and personally risk stratify an individual
patient so as to make proper informed decisions regarding allowing
a patient to participate in competitive athletics or rarely not
(03:32):
doing so. They explain that it's for these reasons that
we've reviewed that all of the major cardiology bodies have
evolved and explained that this is why participation in sports
for many athletes with genetic heart disease may quote be
considered or be a two B recommendation, or even be
quote reasonable, which is a two A recommendation. The authors
(03:53):
then review the concept of shared decision making, which we've
mentioned and discussed many times on this podcast, whereby a
personalized conversation between expert and patient or patient family is
commenced in which the best evidence available is use to
understand the uncertainty and controversies of this topic, as well
as to understand the patient values and things that they
(04:14):
view as important. And the authors explain that it is
their belief that such a conversation will empower a patient
and patient family to make an informed decision and how
in the ideal world, this decision will also include all
of the athlete's stakeholders, including the athlete, the family of
the patient, their school, or the sports governing bodies. With
(04:35):
this as a background, in the setting where a patient
with expert inputs decides to participate, the logistical and practical
aspects of mitigating risk become more important, and this is
where the so called personalized emergency action plan comes to
the forefront. The authors explain that having such a plan
in the event of sudden cardiac arrest for team members
(04:56):
is important, and they explain that it will educate all
involved on early signs of sudden cardiac arrest CPR training,
access to early defibrillation with AEDs on site, as well
as to help develop plans for how to dispatch and
transport a patient to medical facilities. The authors make the
point that though the data they have presented from prior
(05:17):
studies shows that the risks are low for an event,
the risk is not zero and so having a personalized
emergency plan for a particular athlete is prudent. They make
recommendations regarding the nature of this document, and in the
figure accompanying this editorial comment, the authors give a great
example that I would strongly recommend listeners take a look
at and consider integrating into their practice, and as always,
(05:40):
the link to this is in the show.
Speaker 3 (05:41):
Notes this week.
Speaker 1 (05:42):
They explain that a personalized emergency action plan should include
standard things for emergencies, but also tailor the document to
the specific sport and clinical situation of an athlete, and
they make the argument that making these sorts of plans
can avoid problems during the rare event, resulting in a
better outcome, and they feel that implementing such a personalized
(06:03):
plan should be considered an essential part of returning to
play for the athlete who has a genetic heart problem
that's associated with sudden cardiac arrest. They believe that this
document should include precautionary measures like monitoring or training of
the support crew, possible potential risks and adverse events based
on the sport and clinical condition, and they offer the
example of an athlete participating in a water sport and
(06:25):
the particulars of managing that scenario. Finally, they feel it
should include the prescribed roles and action for all personnel
in the case of an emergency. The authors are convinced
that such action plans will further reduce risk for athletes
with genetic conditions and feel it to be a critical
part of the shared decision making model of care, and
they state by developing a personalized emergency action plan together
(06:50):
and ensuring all stakeholders, including the athlete, family and club,
are well informed of potential risks and outcomes, the worst
case scenario is clearly highlighted. The authors also make the
practical point that when an athlete is involved in the
development of such a plan, it may re emphasize for
them the risks with returning to play and may actually
impact their decision making. They conclude by stating the development
(07:14):
of a personalized emergency action plan is a critical part
of the shared decision making model of care and takes time,
with worst case scenario situations clearly described and the patient
and stakeholders well informed of all potential risks and outcomes. Well,
this is a brief statement by three very famous and
prominent authorities in this arena, and for this reason I
(07:35):
thought it important. Having the luck of practicing for nearly
thirty years now as a pediatric electrophysiologist, I've seen the
evolution of thought in this regard and its really breathtaking.
For a long part of my career we were very
restrictive in our recommendations. But to day, based upon work
by these authors and others like Mike Ackerman, to just
name a few, we have learned that though our concerns
(07:57):
were justified, they were almost certainly too restrictive for most athletes.
The process or concept of shared decision making is critical
and one that has really changed the playing field. However,
in my view, these authors, through their emphasis on the
personalized emergency Action plan, have highlighted what is likely a
very important part of that shared decision making, which is
(08:20):
deciding together and in advance of a problem, how to
best protect an athlete while also allowing them to play.
I also felt that the author's comments towards the end
about the entire process of making such a plan helping
to give an athlete a better understanding of their own
risks to be a very important point and may really
enhance the concept of informed consent well. At this point
(08:42):
in the podcast, as you know, we would normally be
speaking with a guest. I had hoped very much that
doctor Gray and doctor Lampert would be able to join
us this week, and both were really quite accommodating. Unfortunately, however,
I made the mistake of choosing to publish this podcast
the same week as the Heart Rhythm Society meet. And
I really have no excuse because I myself am attending
(09:02):
that meeting, but both experts are very busy trying to
catch up with work prior to leaving for this meeting,
and For this reason, we'll be unable to have a
guest this week, but I'm hopeful that my description of
the work, which was really an opinion piece from these
eminine experts, was enough to give you all reason to
think about this topic, which is critically important when we're
(09:24):
thinking about liberalizing the exercise activities of our patients with
genetic heart conditions. To conclude this three hundred and thirty
eighth episode of ped Heart Pediatric Cardiology, today we end
with the lovely tosty song Dale, and today we hear
it sung by the Veronese baritone Simone Piazzola, who in
twenty thirteen was awarded second prize and the audience prize
(09:47):
at the Placido Domingo Operalia competition, and he's gone on
to sing in virtually every important venue in Europe in
the world of opera. Thank you for joining me for
this episode, and thanks once again to our guest. All
have a good weekend.
Speaker 2 (10:03):
Keys save me.
Speaker 3 (10:06):
For me.
Speaker 4 (10:11):
Every ETI save me, cool momia for nay on at
ai save.
Speaker 2 (10:31):
Me not already on even.
Speaker 3 (10:38):
If you are.
Speaker 2 (10:42):
For me a sunsafe cream, cool.
Speaker 4 (11:14):
Inter lytles wander lost for.
Speaker 2 (11:22):
You're going into se.
Speaker 5 (11:26):
Hen your far look ye he inquitor, and.
Speaker 2 (11:36):
Us for it alone he spon yesterday enemy, and call.
Speaker 5 (12:00):
A hey, sladd on, it's lost me on.
Speaker 3 (12:06):
Pad didn't not hard. We touldn't not
Welcome to Pdiheart Pediatric Cardiology.
Speaker 2 (00:18):
Stay.
Speaker 1 (00:18):
My name is doctor Robert Pass and I'm the host
of this podcast. I am Professor of Pediatrics at the
ICONS School of Medicine at Mount Sinai, where I'm the
chief of Pediatric Cardiology. Thank you for joining me for
this three hundred and thirty eighth episode of Pdheart. I
hope everybody enjoyed last week's episode on the topic of
bunsle branch block in the single ventrical patient and an
impact it may have on exercise tolerance. For those of
(00:41):
you interested in this topic, i'd recommend you to take
a listen to last week's episode three hundred and thirty seven.
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
on to the world of genetic cardiology. The title of
the work is Return to Play with Genetic Heart Disease
(01:02):
The Importance of developing a personalized emergency action Plan. The
first author of this work is Belinda Gray, second author
Rachel Lampert, and the senior author Michael Papadakis. And the
authors come to us from Australia, New Haven and the
United Kingdom. So let's move straight on to our description
of this week's editorial comment. The authors of this editorial
(01:23):
on my Mind article begin by reviewing that sudden cardiac
death is rare, but it is the most common cause
of death for athletes during sports, and explain how traditionally
we have placed blanket restrictions on this sort of activity
for patients who have genetic heart disease due to concerns
of sudden cardiac arrest or death. They then review recent
works on genetic heart diseases that have been traditionally those
(01:47):
that were heavily restricted and explain how the recent data
show that perhaps these restrictions have been far too restrictive.
They explain that studies have demonstrated that when managed by
experts using guideline based risks stratification, most athletes with different
inherited cardiac conditions have low rates of cardiac events during sports,
(02:07):
and they reference the recent live HCM or Lifestyle and
Exercise in HCMS study demonstrating no increase in risk for
events in individuals with hypertropic cardiomyopathy who exercised at vigorous
level compared to those who are non vigorous exercisers, with
no real difference in event rates between the vigorous and
(02:27):
the non vigorous types of exercise. The authors then reference
the so called Live long QT Syndrome perspective study demonstrating
low overall rates in long QT syndrome patients with proper
contemporary management and again no differences in event rate between
vigorous and non vigorous exercisers. They then review how studies
(02:48):
with ICD implant patients, who we could assume are amongst
the highest recipatient group, have not shown serious adverse events
in competitive athletes or those participating in high risk sports
like surfing. The authors contrast these situations with something like
a rhythmogenic cardiomyopathy and particularly the PKP two mediated variety,
(03:10):
where we know that such activities as aggressive exercise can
increase the risk for heart failure, ventricularrhythmias, and sudden death.
And so they explain that basically one needs an expert
to help make decisions and personally risk stratify an individual
patient so as to make proper informed decisions regarding allowing
a patient to participate in competitive athletics or rarely not
(03:32):
doing so. They explain that it's for these reasons that
we've reviewed that all of the major cardiology bodies have
evolved and explained that this is why participation in sports
for many athletes with genetic heart disease may quote be
considered or be a two B recommendation, or even be
quote reasonable, which is a two A recommendation. The authors
(03:53):
then review the concept of shared decision making, which we've
mentioned and discussed many times on this podcast, whereby a
personalized conversation between expert and patient or patient family is
commenced in which the best evidence available is use to
understand the uncertainty and controversies of this topic, as well
as to understand the patient values and things that they
(04:14):
view as important. And the authors explain that it is
their belief that such a conversation will empower a patient
and patient family to make an informed decision and how
in the ideal world, this decision will also include all
of the athlete's stakeholders, including the athlete, the family of
the patient, their school, or the sports governing bodies. With
(04:35):
this as a background, in the setting where a patient
with expert inputs decides to participate, the logistical and practical
aspects of mitigating risk become more important, and this is
where the so called personalized emergency action plan comes to
the forefront. The authors explain that having such a plan
in the event of sudden cardiac arrest for team members
(04:56):
is important, and they explain that it will educate all
involved on early signs of sudden cardiac arrest CPR training,
access to early defibrillation with AEDs on site, as well
as to help develop plans for how to dispatch and
transport a patient to medical facilities. The authors make the
point that though the data they have presented from prior
(05:17):
studies shows that the risks are low for an event,
the risk is not zero and so having a personalized
emergency plan for a particular athlete is prudent. They make
recommendations regarding the nature of this document, and in the
figure accompanying this editorial comment, the authors give a great
example that I would strongly recommend listeners take a look
at and consider integrating into their practice, and as always,
(05:40):
the link to this is in the show.
Speaker 3 (05:41):
Notes this week.
Speaker 1 (05:42):
They explain that a personalized emergency action plan should include
standard things for emergencies, but also tailor the document to
the specific sport and clinical situation of an athlete, and
they make the argument that making these sorts of plans
can avoid problems during the rare event, resulting in a
better outcome, and they feel that implementing such a personalized
(06:03):
plan should be considered an essential part of returning to
play for the athlete who has a genetic heart problem
that's associated with sudden cardiac arrest. They believe that this
document should include precautionary measures like monitoring or training of
the support crew, possible potential risks and adverse events based
on the sport and clinical condition, and they offer the
example of an athlete participating in a water sport and
(06:25):
the particulars of managing that scenario. Finally, they feel it
should include the prescribed roles and action for all personnel
in the case of an emergency. The authors are convinced
that such action plans will further reduce risk for athletes
with genetic conditions and feel it to be a critical
part of the shared decision making model of care, and
they state by developing a personalized emergency action plan together
(06:50):
and ensuring all stakeholders, including the athlete, family and club,
are well informed of potential risks and outcomes, the worst
case scenario is clearly highlighted. The authors also make the
practical point that when an athlete is involved in the
development of such a plan, it may re emphasize for
them the risks with returning to play and may actually
impact their decision making. They conclude by stating the development
(07:14):
of a personalized emergency action plan is a critical part
of the shared decision making model of care and takes time,
with worst case scenario situations clearly described and the patient
and stakeholders well informed of all potential risks and outcomes. Well,
this is a brief statement by three very famous and
prominent authorities in this arena, and for this reason I
(07:35):
thought it important. Having the luck of practicing for nearly
thirty years now as a pediatric electrophysiologist, I've seen the
evolution of thought in this regard and its really breathtaking.
For a long part of my career we were very
restrictive in our recommendations. But to day, based upon work
by these authors and others like Mike Ackerman, to just
name a few, we have learned that though our concerns
(07:57):
were justified, they were almost certainly too restrictive for most athletes.
The process or concept of shared decision making is critical
and one that has really changed the playing field. However,
in my view, these authors, through their emphasis on the
personalized emergency Action plan, have highlighted what is likely a
very important part of that shared decision making, which is
(08:20):
deciding together and in advance of a problem, how to
best protect an athlete while also allowing them to play.
I also felt that the author's comments towards the end
about the entire process of making such a plan helping
to give an athlete a better understanding of their own
risks to be a very important point and may really
enhance the concept of informed consent well. At this point
(08:42):
in the podcast, as you know, we would normally be
speaking with a guest. I had hoped very much that
doctor Gray and doctor Lampert would be able to join
us this week, and both were really quite accommodating. Unfortunately, however,
I made the mistake of choosing to publish this podcast
the same week as the Heart Rhythm Society meet. And
I really have no excuse because I myself am attending
(09:02):
that meeting, but both experts are very busy trying to
catch up with work prior to leaving for this meeting,
and For this reason, we'll be unable to have a
guest this week, but I'm hopeful that my description of
the work, which was really an opinion piece from these
eminine experts, was enough to give you all reason to
think about this topic, which is critically important when we're
(09:24):
thinking about liberalizing the exercise activities of our patients with
genetic heart conditions. To conclude this three hundred and thirty
eighth episode of ped Heart Pediatric Cardiology, today we end
with the lovely tosty song Dale, and today we hear
it sung by the Veronese baritone Simone Piazzola, who in
twenty thirteen was awarded second prize and the audience prize
(09:47):
at the Placido Domingo Operalia competition, and he's gone on
to sing in virtually every important venue in Europe in
the world of opera. Thank you for joining me for
this episode, and thanks once again to our guest. All
have a good weekend.
Speaker 2 (10:03):
Keys save me.
Speaker 3 (10:06):
For me.
Speaker 4 (10:11):
Every ETI save me, cool momia for nay on at
ai save.
Speaker 2 (10:31):
Me not already on even.
Speaker 3 (10:38):
If you are.
Speaker 2 (10:42):
For me a sunsafe cream, cool.
Speaker 4 (11:14):
Inter lytles wander lost for.
Speaker 2 (11:22):
You're going into se.
Speaker 5 (11:26):
Hen your far look ye he inquitor, and.
Speaker 2 (11:36):
Us for it alone he spon yesterday enemy, and call.
Speaker 5 (12:00):
A hey, sladd on, it's lost me on.
Speaker 3 (12:06):
Pad didn't not hard. We touldn't not
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