Transcript Sudden Cardiac Death in Athletes and

Sudden Cardiac Death
in Athletes and
Preparticipation CV
screening
Antonio Pelliccia, MD
Institute of Sport Medicine and Science. Rome.
[email protected]
Global Debate or
Common Ground ?
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SCD is a catastrophic
event
Athletes are at
increased risk
Prevention is critical
Screening for occult
disease is efficient
Sudden Cardiac Death
in Athletes:
incidence
SCD Incidence

Traditional Estimates in the U.S.
 1 in 200,000 (i.e., 0.5 x 100,000) High
School Athletes/yr 1,2
 1 in 160,000 (i.e., 0.62 x 100,000) in young
of 12-35 age 3
1.
2.
3.
Van Camp; MSSE 1995
Maron; JACC 1998
Maron; Circulation 2009
Sudden Deaths in US Young Competitive Athletes: 1980-2006
Number of sudden deaths
Number of cardiovascular sudden deaths (black bars) in 1,866 young US athletes
Most recent incidence rate = 0.66 x 100,000
Actual incidence underestimated ?
Maron et al. Circulation. 2009;119:1085-1092
Incidence of SCD in young athletes
0.6x100,000
Case identification were through :
Lexis Nexis archival database,
News media reports,
Internet searches,
Reports from the consumer
Product
Safety
3,55
x100,000
wasCommission,
the incidence of sudden
cardiac death
in the
residents
of Olmsted
Pathology
from
NHBLI,
County,
Minnesota,
aged
40 years, in
Reports
submitted
to 20
thetoauthor
the period 1960 to 1989.
3.6x100,000
Cases were collected by a
prospective registry of Juvenile SD,
in a relatively limited geographical
area, with causes identified by
experienced pathologist
Prospective population-based study on OHCA
 11 US/Canadian sites; >260 EMS agencies
 All OHCA with EMS response; Dec 2005 –
March 2007
 Incidence of OHCA in adolescents (age 12-19)
 CV cause: 3.75/100,000 (1 in 27,000)
(from Atkins et al. Circulation 2009)
SCD Incidence:
Challenges and Limitations
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Difficult to compare incidence studies with
profoundly different methodology
No mandatory reporting system in U.S.
U.S. estimates reliant on media and other
electronic sources
What [Numerator] / What
[Denominator]grossly estimated
Sudden Cardiac Death
in Athletes:
pathologic findings
Cardiovascular Causes of Sudden Death
Combined
prevalence of
these cardiac
diseases in the
general
athletic
population is
0.3% (or 3 in
1,000)
From the Minneapolis Heart Foundation Registry
HCM
ARVC
Congenital coronary artery anomalies
Marfan syndrome
Myocarditis
Brugada
WPW
WPW syndrome
Long QT interval
CPVT
Short QT interval
Sudden Cardiac Death
in Athletes:
The role of sport
HCM athlete, judged to be at “low risk” for SCD
Assessment of risk in HCM patients
Risk Factors:
High risk
(1-3%)
100%
90%
≥ 1 risk
factor
70%
(45%)
80%
60%
>3
2
Previous cardiac arrest
Recurrent syncope
Familial premature SD
Sustained VT, or repetitive
NSVT at Holter
 BP at exercise
Massive LVH
50%
40%
1
30%
20%
10%
0
Atrial fibrillation,
LVOT obstruction,
myocardial ischemia
Low risk
(50-55%)
0%
Competitive Sport
RR = 2.5
CI = 1.8-3.4
p < 0.001
Sport activity may increase the risk for SCD in
athletes with underlying structural CV disease
from Corrado et al. JACC 2003
EXERCISE triggers SCD in athletes with cardiomyopathies
TRIGGERS:
Deydratation
Electrolyte
imbalance
Adrenergic
output
Ischemia
VF/Asystole
Unstable Electrophysiological Substrate
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10,611
6
In athletes with structural CV disease
intensive training and48competition
increases the incidence and severity of
2,165
9
arrhythmias at risk for SD.
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0,5
On the contrary, detraning reduces the
risk and may have life-saving impact.
PEAK TRAINING
AFTER DETRAINING
Strategies to prevent
SCDs in Athletes:
The preparticipation
screening
ARVD 23%
Anomalous
c.a. origin 12%
(Corrado et al. NEJM 1998)
Atherosclerotic
c.a. disease 18%
Anomalous
c.a. origin 17%
More than 2/3 of all SCD
causes of are CMPs detectable
HCM 2%
ARVD 4%
Possible
HCM 8%
during life !
HCM 36%
(Maron et al. Circulation 2009)
Atherosclerotic
c.a. disease 2%
Prodromal Symptoms
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HCM: up to 21%1
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CCAA: up to 37%2
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ARVC: up to 68%3
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AN-SUD: up to 14%4
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CPVT: up to 95%5
1.
2.
3.
4.
5.
Maron; JAMA 1996
Basso; JACC 2000
Corrado; PACE 2002
Tester; Mayo Clin Proc 2004
Leenhardt; Circulation 1995
Warning Symptoms & Family
History in Children with SCA
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Retrospective survey of Parent Heart Watch
families whose children had SCA
79 cases from 78 families analyzed
19 of 79 cases (24%) reported syncope and/or
unexplained seizure activity that went undetected
as CV disease
Syncope 14 of 79 cases (18%) (average 2.3 events)
Seizure 10 of 79 cases (13%) (average 2.9 events)
The purpose of screening:
Timely identification and selective withdrawal
from competitive sport of athletes with underlying
cardiac disease, to reduce the risk of sudden death
(or disease progression) and start appropriate
treatment, if necessary
The efficacy of screening by history and P.E.
Only history and
PE
134
Total dead athletes
130
Additional CV
testing
15
115
1
Correct
Diagnosis
7
(Maron BJ, JAMA 1996)
Protocol of preparticipation CV screening
History, P.E., 12-lead ECG
Normal
Findings
Start
training
Abnormal
Findings
No CV
disease
Additonal
testing
CV disease
Management according to Guidelines
The rationale for including the
12-lead ECG in the PPE
1. Symptoms are usually absent (or denied) in
athletes with CMPs!
21%
Syncope, dizziness,
and/or chest pain in
HCM (Maron BJ , JAMA
1996)
2. Physical examination is
unremarkable in most CMPs
patients
3. ECG abnormalites are present in
up to 95% of HCM and 80% of
ARVC patients !
The rationale for including the 12-lead ECG:
ARVC
HCM
RV
LV
LA
RA
ECG abnormalities in up to 95%:
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ST-T wave changes
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Deep Q waves
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Left axis deviation
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Exceedingly high QRS voltages

P wave changes
(LAenlargement)
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ECG abnormalities in > 80%:
Inverted T waves in anterior
precordial leads (beyond V1)
RV conduction delay
Epsilon wave
PVBs with LBBB morphology
Diagnosis of HCM in
young athletes
Abnormal findings
LV Hypertrophy
Genotype
anomalies
Abnormal ECG
Sudden death can occur at any time !
Adolescence
Adulthood
Long-term follow-up of athletes with abnormal ECG
(Pelliccia et al. New Engl J Med 2008; 358: 152-63)
Study group
81
9-year follow-up
No symptoms,
no CV disease
6
70
5
1, sudden death
1, cardiac arrest
Other CV disease (hpt 3, Cardiomyopathies
CAD 1, myoc 1, SVT 1) (HCM3; ARVC1; DCM1)
MM, soccer player at
age of 28 years
PFW
IVS
LV
RV
RA
LA
MM, at the age of
33 years
Probability to identify cardiac diseases at risk of SCD
by ECG or by MH+PE:
ECG
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Hypertrophic cardiomyopathy
Arrhythmogenic right ventricular
cardiomyopathy
Dilated cardiomyopathy
Myocarditis
Marfan’s syndrome
Valvular Disease
Long QT and Short QT syndrome
Brugada syndrome
Preexcitation syndrome (WPW)
Congenital Coronary Artery Anomalies
MH+PE
up to 90%
< 10%
60-80%
30-60%
30-60%
< 10%
< 10%
> 80%
> 90%
> 90%
< 10%
< 10%
< 10%
< 10%
> 90%
> 90%
zero
zero
zero
< 10%
“...The studies quoted indicate that the ECG is about
50–95% sensitive to detect underlying heart disease
...this represents a marked improvement over physical
examination alone, where the sensitivity is only 3–6%
...”
(Lawless et al. Med. Sci. Sports Exerc.; 2008; 5: 787–798)
The 2009 IOC Consensus Statement
Challenges for implementing the ESC proposal
for preparticipation CV screening …
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…the central challenge is practicability,
feasibility and implementation, with the
primary obstacle being adequate resources
and economic support …
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…the substantial burden placed on the
system by false positive test results.
(Maron BJ, Eur Heart J 2005)
Costs of preparticipation CV screening
• History + PE
150-350 $
• 12-lead ECG
50-150
• Echocardiogram
600-900
• Exercise testing
300
• History, PE, ECG
45 €
• Echocardiogram 100-150
• Exercise testing
100-150
31,864 athletes evaluated in 19 National Medical Centers
3.756
(11.8%)
28.108
(88.2%)
Normal ECGs
Abnormal ECGs
ECG abnormalities found at PPS
LAFB WPW
LVH (4%) (1%) Prolonged QT interval
(0.03%)
(7%)
RBBB
(9%)
Inverted T waves
(20%)
Early repolarization;
incomplete RBBB; mildly
increased R/S wave voltages
(59%)
ECG Interpretation in athletes:
Need for a new approach
“Normal”
Common and
Physiologic
Training-related
ECG alteration
“Abnormal”
Uncommon and
Pathologic
Training-unrelated
Need for further
work-up
4,450 athletes judged free of CV diseases
at CV preparticipation screening
ECHOCARDIOGRAPHY
12
(<1%)
Structural CV
Abnormalities
(i.e., false negatives)
4,438
(>99%)
Witout CV diseases
(i.e., true negatives)
(Pelliccia et al. Eur Heart J. 2006)
Athletes with Structural CV Abnormalities
Marfan’s
(n = 2)
ARVC
(n = 1)
MVP
(n = 3)
Aortic valve
disease
(n = 2)
Myocarditis
(n = 4)
No HCM was found !
(Pelliccia et al. Eur Heart J. 2006)
Can we do better ?
Screening for genetic
anomalies in young athletes ?
Genetic testing are commercially available …
Detectable genetic anomalies
HCM gene testing detection efficacy
Génotype-Phenotype Relationship in HCM:
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There is no closeUnresolved
relationship betwen the extent
ethical,
social andand locus or
and pattern of
LV hypertrophy
economic
mutation abnormality;
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implications of
There is a large variability in the extent and
genetic results!
distribution of(see
LV hypertrophy
GINA act, for a given
mutation between
US and
lawwithin
2010)families.
Myofilament-positive HCM
patients are more frequently
characterized by progressive
impairment of LV diastolic
and systolic function, leading
to clinical deterioration and
occurrence of adverse events
(Olivotto J et al. Mayo Cli
Proc 2008; 83: 630)
The efficacy of the PPS to reduce
mortality in young athletes
Reduction in SCDs in young athletes in the Veneto Region
SD per 100,000 athlete-years
Annual incidence of SCD in screened athletes vs. unscreened non-athletes
3.6
0.7
0.8
0.4
Corrado et al. JAMA 2006;296:1593-601
The Veneto region and the state of Minnesota (US) have
similar population demographics and athlete populations in
person-year suitable for comparison of SD estimates.
(Maron et al. Am J Cardiol 2009)
Comparison of Italian and US Sudden Deaths
in Competitive Athletes
Corrado et al. JAMA 2006; 296: 1593-601
Maron et al. Am J Cardiol 2009; in press
CV Screening in 510 Bostonian College Athletes
Cardiac morphology His. + PE
ECG
1, Bicuspid aortic valve
2, Bicuspid aortic valve
3, MVP
4, MVP
5, MVP
6, Pulm. stenosis
7, LV hypertrophy
8, LV hypertrophy
9, LV dilation
10, LV dilation
11, RV dilation
None
None
None
None
None
None
None
None
None
None
None
Pul. St.
QRS volt/LAE None
QRS volt/T neg HCM
LBBB
None
LBBB
Myocarditis
RBBB
None
Murmur
Murmur/Click
Murmur
Murmur
None
Murmur
None
None
None
None
None
Diagnosis
by A. Baggish et al. Ann Int Med 2010; 152: 269
H+P
Correctly identified 5/11 subjects (45%) with valvular
disease.
Missed all athletes with structural myocardial disease,
including HCM and myocarditis.
Sensitivity: 45%; Specificity: 94%; NPV: 98%
H + P + ECG:
Correctly identified all 11 subjects with both valvular and
myocardial abnormalities, including HCM and
myocarditis, that required sport restriction.
Sensitivity: 91%; Specificity: 83%; NPV: 99.8%
Theoretical model to project the costs and survival rates for US highschool and college athletes undergoing screening.
Screening with H + P resulted in 0.56 life-year saved per 1000
athletes compared to no screening.
Cost-effectiveness ratio for H + P vs. no screening was $ 199,000
per life-year saved.
The screening including also the ECG saved 2.1 life-years per
1000 athletes at an incremental cost of $88 per athletes.
Cost-effectiveness ratio of adding ECG to history and physical
examination was $42,000 per life-year saved
H+PE
+ECG
1,473 intercollegiate athletes from University of Virginia
(50%males; mean age 19 years; 71% Caucasian)
5 palpitations:
•3 AVNRT,
•1 atrial tach.
•1 atrial fib.
8
5
$ 68,745 cost for each CV abnormality
0
disqualified
•1 BAV
•4 WPW pattern
•1 long QT
• 2 arrhyth. CMPs?
$ 68,893
2
(R. Malhotra et al. personal communication)
Should be the electrocardiogram required in young
athletes?
Chaitman B
Lancet 2008;371:1489-90
Electrocardiographic screening in athletes: the time
is now for universal screening.
Papadakis M, Sharma S.
Controversies relating to pre-participation cardiovascular
screening in young athletes: Time for a realistic solution?
Papadakis M, Chandra N, Sharma S.
TAKE HOME MESSAGE:
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Screening elite athletes to prevent SD is compelling
on ethical, medical and legal ground;
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Scientific evidence suggest that screening with 12lead ECG is feasible and efficient to identify (or raise
suspicion for) CV disease at risk of SD;
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Implementation of the CV screening in athletes is
efficient to reduce mortality in young athletes
Sudden Death in Athletes
“The fragility of life”
Dedicated to Marc-Vivien Foe
Antonio Pelliccia, MD
Institute of Sport Medicine and Science. Rome.
[email protected]