Download L. Környei

Should sports participation be restricted in
LQTS?
L. Környei
Hungarian Pediatric Heart Center
5th February 2017 14:00 – 15:15 Inherited Arrhythmia Syndromes VI: Long QT Syndrome: Complicated Cases
Topics
1.  Early data
2.  Recent data
3.  Recent recommendation
4.  The “real” question to be answered
Early data…
•  increased frequency of cardiac events during
exercise in patients with LQT1
–  (swimming 62% vs sleeping 3%)
Schwartz, Circulation 2001
•  In untreated patients, LQTS-related cardiac events
occurred in 55%
Shimizu, JACC 2001
•  Thus, guidelines have historically advocated for an
aggressive restriction from competitive sports, with
the exception of low-intensity sports
Bethesda 36, ESC 2005
“historical decision making”
•  beta-blockade
•  avoidance of medications that prolong the QT
interval
•  “you're not to play sports”
Physiologic benefits of exercise
sports participation brings to children and adolescents
•  enhanced self-confidence,
•  sense of psychological, physical and social wellbeing
•  improved overall quality of life
…but the pendulum is now swinging away…
LETTERS
It started
with ..
ONLINE FIRST
involved in sports (1
sports (27, 12%) fo
patients (37%, 60 fe
RESEARCH LETTER
[46] milliseconds) r
Competitive Sports Participation in Athletes
including 20 with IC
With Congenital Long QT Syndrome
ences between the to
the nonathletes wer
Johnson, Ackerman,
JAMA, August
8
To the Editor: Competitive
sports participation
for 22/29,
ath- 2012—Vol
competed308,
in aNo.
variet
letes with long QT syndrome (LQTS) is guided by the and 49 of 130 (38%)
36th Bethesda Conference, which recommends that There were 32 athlet
patients with either (1) symptoms, (2) a corrected QT and 8 (6%) competin
interval (QTc) greater than 470 milliseconds (males) or fessional level.
480 milliseconds (females), or (3) an implantable
Seventy athletes (
cardioverter-defibrillator (ICD) not participate in most European guideline
sports.1 The European Society of Cardiology guidelines None had a sport-rela
are more restrictive, disqualifying athletes from all sports (46%) continuing in
based solely on a stringent QTc cutoff (!440 millisec- only 1 experienced s
Research letter
•  105 mutations
•  most common mutation was KCNQ1 (58%)
–  KCNH2 (35%),SCN5A (6%),KCNE2 (2%),KCNE1 (1%)
•  All patients were treated with beta-blockade
–  (1 noncompliant, 1 intolerant)
•  26 pts competitive sports (15 girls; mean follow-up 6.9
years; mean QTc, 461 ms
•  77 pts recreational sports (35 girls; mean follow-up 7.3
years; mean QTc, 470 ms
•  During 755 patient-years of follow-up, no patients had long
QT syndrome symptoms during sports
•  5 appropriate ICD shocks in 2 pts, but non were related to
sports
…but the pendulum is now swinging away…
It is accelerated by ..
JACC: CLINICAL ELECTROPHYSIOLOGY
VOL. 1, NO. 1-2, 2015
ª 2015 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION
PUBLISHED BY ELSEVIER INC.
ISSN 2405-500X/$36.00
http://dx.doi.org/10.1016/j.jacep.2015.03.006
Sports Participation in Genotype Positive
Children With Long QT Syndrome
Peter F. Aziz, MD,* Tammy Sweeten, MS,y Ramon L. Vogel, MD,y William J. Bonney, MD,y
Jacqueline Henderson, RN,y Akash R. Patel, MD,z Maully J. Shah, MBBSy
ABSTRACT
OBJECTIVES The study sought to examine the prevalence and outcomes of sports participation (both competitive and
recreational) in our single-center LQTS genotype positive pediatric population.
Pediatric data
• 
• 
• 
• 
• 
• 
• 
• 
Children’s Hospital of Philadelphia
from 1998 to 2013
212 patients with LQTS
103 participated in sports (53 girls)
all were treated with BB—(nadolol for 97 pts)
mean follow-up, 7.1 years
mean QTc, 468 ms
there were no cardiac events and no deaths
Recent data
Sports participation appears safer today
for children with long QT syndrome ?
Explanation ?
Different patient population
•  Ten years ago before genotyping was mainstream, most
patients were diagnosed as a result of an event
•  With the introduction of genetic testing and cascade
familial testing, there are a lot more patients being
identified with LQTS, without any event
•  the chance of a patient without symptoms presenting for
the first time with sudden cardiac death/cardiac arrest is
approximately 5%
“shared decision”
•  if the restriction is devastating, and in
some cases it might be, harming the child's
psychological, physical, or social well-being
•  sports participation might be safe in certain
circumstances
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
VOL. 66, NO. 21, 2015
ª 2015 BY THE AMERICAN HEART ASSOCIATION, INC. AND
ISSN 0735-1097/$36.00
THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION
http://dx.doi.org/10.1016/j.jacc.2015.09.042
PUBLISHED BY ELSEVIER INC.
AHA/ACC SCIENTIFIC STATEMENT
Eligibility and Disqualification
Recommendations for Competitive Athletes
With Cardiovascular Abnormalities:
Task Force 10: The Cardiac Channelopathies
A Scientific Statement From the American Heart Association and American College of Cardiology
Michael J. Ackerman, MD, PHD, FACC, Chair*
Douglas P. Zipes, MD, FAHA, MACC*
Richard J. Kovacs, MD, FAHA, FACC*
Barry J. Maron, MD, FACC*
The cardiac channelopathies are a collection of primary,
fibrillation. Approximately 1 in 1,000 people are
genetically mediated heart rhythm disorders (also
affected by a cardiac channelopathy, with LQTS
referred to as the primary electrical disorders) that are
being most common, involving an estimated 1 in 2,000
generally associated with a structurally normal heart
people (1).
and a propensity for syncope, seizures, or sudden car-
Presently, these channelopathies should be viewed
diac arrest precipitated by a channelopathy-mediated
as potentially lethal but highly treatable conditions.
episode of nonsustained or sustained polymorphic
However, unlike the various bradyarrhythmias and
AHA/ACC SCIENTIFIC STATEMENT
Eligibility and Disqualification
Recommendations for Competitive Athletes
With Cardiovascular Abnormalities:
Task Force 10: The Cardiac Channelopathies
A Scientific Statement From the American Heart Association and American College of Cardiology
Recommendations
1.  For athletes with a suspected/diagnosed
cardiac channelopathy, a comprehensive
evaluation by a heart rhythm specialist or
genetic cardiologist with sufficient
experience and expertise with these
disorders is recommended (Class I; Level of
Evidence C).
Michael J. Ackerman, MD, PHD, FACC, Chair*
Douglas P. Zipes, MD, FAHA, MACC*
Richard J. Kovacs, MD, FAHA, FACC*
Barry J. Maron, MD, FACC*
The cardiac channelopathies are a collection of primary,
fibrillation. Approximately 1 in 1,000 people are
genetically mediated heart rhythm disorders (also
affected by a cardiac channelopathy, with LQTS
referred to as the primary electrical disorders) that are
being most common, involving an estimated 1 in 2,000
generally associated with a structurally normal heart
people (1).
and a propensity for syncope, seizures, or sudden car-
Presently, these channelopathies should be viewed
diac arrest precipitated by a channelopathy-mediated
as potentially lethal but highly treatable conditions.
episode of nonsustained or sustained polymorphic
However, unlike the various bradyarrhythmias and
ventricular tachycardia (torsade de pointes) or ven-
tachyarrhythmias detailed in the Task Force 9 report
tricular fibrillation. These cardiac channelopathies
(2), there remains significant variability and hetero-
include long-QT syndrome (LQTS), catecholaminergic
geneity among pediatric and adult heart rhythm
polymorphic ventricular tachycardia (CPVT), Brugada
specialists in terms of their ability to diagnose, risk
syndrome (BrS), early repolarization syndrome, short-
stratify, and treat patients with these conditions. For
QT syndrome, and potentially idiopathic ventricular
example, in 1 study, 40% of the patients who received
AHA/ACC SCIENTIFIC STATEMENT
Eligibility and Disqualification
Recommendations for Competitive Athletes
With Cardiovascular Abnormalities:
Task Force 10: The Cardiac Channelopathies
A Scientific Statement From the American Heart Association and American College of Cardiology
Recommendations
2.  It is recommended that symptomatic
athletes with any suspected or diagnosed
cardiac channelopathy be restricted from
all competitive sports until a
comprehensive evaluation has been
completed
Michael J. Ackerman, MD, PHD, FACC, Chair*
Douglas P. Zipes, MD, FAHA, MACC*
Richard J. Kovacs, MD, FAHA, FACC*
Barry J. Maron, MD, FACC*
The cardiac channelopathies are a collection of primary,
fibrillation. Approximately 1 in 1,000 people are
genetically mediated heart rhythm disorders (also
affected by a cardiac channelopathy, with LQTS
referred to as the primary electrical disorders) that are
being most common, involving an estimated 1 in 2,000
generally associated with a structurally normal heart
people (1).
and a propensity for syncope, seizures, or sudden car-
Presently, these channelopathies should be viewed
diac arrest precipitated by a channelopathy-mediated
as potentially lethal but highly treatable conditions.
episode of nonsustained or sustained polymorphic
However, unlike the various bradyarrhythmias and
ventricular tachycardia (torsade de pointes) or ven-
tachyarrhythmias detailed in the Task Force 9 report
tricular fibrillation. These cardiac channelopathies
(2), there remains significant variability and hetero-
include long-QT syndrome (LQTS), catecholaminergic
geneity among pediatric and adult heart rhythm
polymorphic ventricular tachycardia (CPVT), Brugada
specialists in terms of their ability to diagnose, risk
syndrome (BrS), early repolarization syndrome, short-
stratify, and treat patients with these conditions. For
QT syndrome, and potentially idiopathic ventricular
example, in 1 study, 40% of the patients who received
AHA/ACC SCIENTIFIC STATEMENT
Eligibility and Disqualification
Recommendations for Competitive Athletes
Recommendations
With Cardiovascular Abnormalities:
It is reasonable
for Force
an asymptomatic
athlete with
genotype-positive/
Task
10: The Cardiac
Channelopathies
phenotype-negative LQTS
A Scientific Statement From the American Heart Association and American College of Cardiology
1) avoidance of QT-prolonging drugs for athletes with LQTS
Michael J. Ackerman, MD, P D, FACC, Chair*
Douglas P. Zipes, MD, FAHA, MACC*
(http://www.crediblemeds.org)
Richard J. Kovacs, MD, FAHA, FACC*
H
Barry J. Maron, MD, FACC*
3) electrolyte/ hydration replenishment and avoidance of dehydration
The cardiac channelopathies are a collection of primary,
fibrillation. Approximately 1 in 1,000 people are
4) avoidance
or
treatment
of
hyperthermia
from
febrile illnesses or traininggenetically mediated heart rhythm disorders (also
affected by a cardiac channelopathy, with LQTS
related heatreferred
exhaustion
orelectrical
heat disorders)
strokethatfor
with involving
eitheranLQTS
BrS
to as the primary
are athletes
being most common,
estimated 1or
in 2,000
generally associated with a structurally normal heart
people (1).
and a propensity for syncope, seizures, or sudden car-
Presently, these channelopathies should be viewed
tricular fibrillation. These cardiac channelopathies
(2), there remains significant variability and hetero-
diacof
arrest
by a automatic
channelopathy-mediated
as potentially
lethal but highly
conditions.
5) acquisition
a precipitated
personal
external
defibrillator
as treatable
part of
the
episode of nonsustained or sustained polymorphic
However, unlike the various bradyarrhythmias and
athlete’s personal
sports
safety
gear,
and
ventricular tachycardia (torsade de pointes) or ventachyarrhythmias detailed in the Task Force 9 report
include long-QT syndrome (LQTS), catecholaminergic
geneity among pediatric and adult heart rhythm
6) establishment
of
an
emergency
action
plan
with the appropriate school or
polymorphic ventricular tachycardia (CPVT), Brugada
specialists in terms of their ability to diagnose, risk
syndrome
(BrS), early
shortstratify,
team officials
(Class
IIa;repolarization
Level ofsyndrome,
Evidence
C). and treat patients with these conditions. For
QT syndrome, and potentially idiopathic ventricular
example, in 1 study, 40% of the patients who received
AHA/ACC SCIENTIFIC STATEMENT
Eligibility and Disqualification
Recommendations for Competitive Athletes
With Cardiovascular Abnormalities:
Task Force 10: The Cardiac Channelopathies
A Scientific Statement From the American Heart Association and American College of Cardiology
Recommendations
Ackerman, MD, P D, FACC, Chair*
Douglas P. Zipes, MD, FAHA, MACC*
5.  ForMichael
an J.athlete
with either symptomatic
LQTS or
Richard J. Kovacs, MD, FAHA, FACC*
Barry J. Maron, MD, FACC*
electrocardiographically manifest
LQTS (i.e.,
corrected QT interval >470 ms in males or >480 ms
The cardiac channelopathies
are a collection of primary,
fibrillation.
Approximately 1 in
1,000 people are
in females),
competitive
sports
participation
(except
genetically mediated heart rhythm disorders (also
affected by a cardiac channelopathy, with LQTS
referred to as the primary
electrical disorders)
being most common,
involving an estimated 1 in 2,000
competitive
swimming
inthataarepreviously
symptomatic
generally associated with a structurally normal heart
people (1).
LQT1
host)formay
considered
aftertheseinstitution
of be viewed
and a propensity
syncope, be
seizures,
or sudden carPresently,
channelopathies should
diac arrest precipitated by a channelopathy-mediated
as potentially lethal but highly treatable conditions.
treatment
and appropriate
precautionary
measures
episode of nonsustained
or sustained polymorphic
However, unlike the various
bradyarrhythmias and
ventricular tachycardia (torsade de pointes) or ventachyarrhythmias detailed in the Task Force 9 report
assuming
the
athlete
has
been
asymptomatic
onand heterotricular fibrillation. These cardiac channelopathies
(2), there
remains significant variability
include long-QT syndrome (LQTS), catecholaminergic
geneity among pediatric and adult heart rhythm
treatment
for
at
least
3
months
(Class
polymorphic ventricular tachycardia (CPVT), Brugada
specialists
in termsIIb)
of their ability to diagnose, risk
H
syndrome (BrS), early repolarization syndrome, short-
stratify, and treat patients with these conditions. For
QT syndrome, and potentially idiopathic ventricular
example, in 1 study, 40% of the patients who received
Practical recommendations
•  BB - 15%-20% reduction in peak HR during exercise
•  avoid routinely exceeding 65% of their target HR
•  emphasize the importance of
– 
– 
– 
– 
compliance with medication
maintaining hydration
resting when experiencing symptoms
rescue systems are in place
•  good relationship with the family
–  surveillance monitoring for efficacy of medical therapy
(Holter,exercise testing)
–  access to advice and feedback regarding exercise
Cheung et al, Canadian Journal of Cardiology 32 (2016) 452e458
doctor relationship (Fig. 3).
and the Canad
Making
recommendations fromDisclosures
Conclusions
Recent evidence suggests a lower risk of exercise-related
A.D.K. rec
arrhythmias
in
treated
patients
than
previously
assumed.
evaluation to participation Foundation of
Stroke Founda
Chair in Heart
Charles Kerr D
netics. S.S. is s
of Canada (G
conflicts of int
References
1. Krahn AD, H
tients with un
Preserved Ejec
278-85.
2. Behr E, Wood
degree relativ
2003;362:145
3. Tan HL, Hof
Sudden unexp
logical and g
2005;112:207
Figure 3. Making recommendations from evaluation to participation.
al, Canadian
Journal
of Cardiology
32
AED,Cheung
automatedetexternal
defibrillator;
ICD, implantable
cardioverter
4. McGorrian C
(2016) 452e458
screening in r
Fact and question
Fact:
aerobic activity is a "potential risk-taking behavior"
for patients with LQTS
Question:
Whether the increased risk can be "circumvented,
neutralized, or minimized" rather than
disqualifying the patient from sports entirely.
M. Ackerman
ugh
of
ins
ing
een
no
ost
ntof
odnes
owver,
cise
ith
arula-
condition.
Cartoon
toofencourage
safe
and
Increased
awareness
the patient, family,
and community
issupervised
essential to managing
the riskactivity
of arrhythmic
and SCD
physical
in
children
events, and we emphasize the patient’s role in creating a safe
LQTS
environment for sportswith
participation
(Fig. 1). Proficiency in
Figure
1. et
Cartoon
to encourage
and supervised
physical
activity
Cheung
al, Canadian
Journalsafe
of Cardiology
32 (2016)
452e458
in children.