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SPORTS AND
EXERCISE ADVICE
IN PATIENTS WITH
HCM AND ARVC
Sharlene M. Day, MD
Associate Professor, Cardiovascular Medicine
Director, Program for Inherited
Cardiomyopathies
University of Michigan
USA
Rio De Janeiro 2016
Sport and Exercise Cardiology Symposium
SBC/SOCERJ – ACC
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Hypertrophic cardiomyopathy Arrhythmogenic RV cardiomyopathy
HCM ARVC
Diagnosis Hypertrophy < 13 mm Task Force criteria (2010)Diagnosis Hypertrophy < 13 mm Task Force criteria (2010)
Age of onset Variable Variable
Predominant
ventricle
Left (asymmetric septal most
common), can affect right
Right, can affect left
Genetics Sarcomere ~50-60% Desmosome ~50%
Complications LVOT obstruction, HF, VT/VF, atrial
fib, stroke
VT/VF (higher rate of exertion-related),
HF with LV involvement
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• Triggering of ventricular tachycardia/fibrillation leading to sudden cardiac arrest/death
• Greater prevalence exercise-provoked VT/VF with ARVC compared to HCM
MAJOR CONCERNS ABOUT ATHLETES WITH
HCM OR ARVC COMPETING IN SPORTS
• Potential for disease progression – physiologic stress triggering and/or accelerating pathological remodeling
• No evidence for patients with HCM. In mouse model of HCM, voluntary exercise attenuates disease
• In ARVC, data in mice and humans suggests that vigorous athletic training may exacerbate disease progression
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Causes of SCD in young US
competitive athletes ages < 39
Hypertrophic
Cardiomyopathy
WHAT PROPORTION OF SCDS IN ATHLETES ARE
DUE TO HCM OR ARVC?
ARVC 4%
Cardiomyopathy
(36%)
Maron BJ et al. Circulation 2009;119:1085-1092
Possible HCM
Channelopathies
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HCM AND ARVC EACH ACCOUNT FOR ONLY 3% OF SUDDEN CARDIAC
DEATHS IN NATIONAL COLLEGIATE ATHLETIC ASSOCIATION
ATHLETES.
Autopsy negative
5 year prospective registry of NCAA athletes
Harmon K G et al. Circ Arrhythm Electrophysiol. 2014;7:198-204
Coronary artery
anomaly
DCMHCM
ARVC
LVH
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CAD
HCM ACCOUNTS FOR 9%, ARVC 15% OF SCD IN
YOUNG ITALIAN ATHLETES, AGES < 35 Y/O
Corrado et al., JACC 2003; 42:1959-63
HCM
Myocarditis
ARVC
Conduction system
Corrado et al., JACC 2003; 42:1959-63
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Overall incidence of SCD in athletes = ~3:100,000 person/years
• Male > female
• Black > White
• Certain sports higher risk (i.e. basketball)
Risk of death from accidents, homicide, suicide in NCAA athletes
= 4X risk of SCD overall
Incidence of fatal motor vehicle accidents in teens
= 4-5X risk of SCD overall
Estimated risk of SCD for an athlete with HCM
competing in sports: <0.1% per year
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GUIDELINES RECOMMEND UNIVERSAL EXCLUSION FROM MOST
COMPETITIVE SPORTS FOR ATHLETES WITH PROBABLE OR
DEFINITE DIAGNOSIS OF HCM OR ARVC
• 35th Bethesda Conference (US)
• European Society of Cardiology
Pelliccia A, et al. European Heart Journal 2005
• Updated AHA/ACC Guidelines, Task Force 3
Maron BJ, et al. Task Force 4: J Am Coll Cardiol 2005
• Updated AHA/ACC Guidelines, Task Force 3
Maron BJ et al., JACC 2015
Addition
• “These recommendations do not strictly exclude in absolute terms fully informed athletes
from participating in competitive athletic programs as long as such decision is ultimately
made in concert with their physician and third-party interests….there will always be tolerance
in the system for some degree of flexibility, individual responsibility, and choice in making
decisions for individual student athletes”
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WHY HAVE THE GUIDELINES FOR SPORTS
PARTICIPATION IN HCM NOT CHANGED IN 10 YEARS?
• Not much new data
Sports ICD registry (Lampert et al, Circ 2013;127:2012-2030))
• Contrasts with drastic changes in guidelines for competitive sports participation in channelopathies (Task Force 10)participation in channelopathies (Task Force 10)
• Competitive sports participation may be considered after comprehensive evaluation by heart rhythm specialist, institution of treatment, and no symptoms for 3 months
• Observational studies in LQTS - events with competitive sports participation are very low (only 1/130 had an event in 10 years, event rate = 0.003 events/athlete-years)
Johnson and Ackerman, JAMA 2012;308:764-5
Aziz et al, JACC Electrophysiology 2015
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ENDURANCE EXERCISE INCREASES
PENETRANCE AND DISEASE SEVERITY IN ARVC
DESMOSOME MUTATION CARRIERS
Survival free from VT/VF
Likelihood of meeting TFC for ARVC
James et al., JACC 2013;62:1290-7
Hours of exercise
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ENDURANCE ATHLETICS MAY BE MORE
INFLUENTIAL IN GENOTYPE NEGATIVE ARVC
Sawant et al., JAHA 2014;3:e001471
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COMPETITIVE BUT NOT RECREATIONAL HIGH
DYNAMIC SPORT ASSOCIATED WITH
INCREASED RISK OF VT/VF IN ARVC
All probands
Ruwald et al., Eur Heart J 2015;36:1735-43
Mixed desmosome and
genotype negative
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WHAT’S THE MECHANISM?
• Simple model: Mechanical stress induced by increased RV
volumes with aerobic exercise causes loss of myocyte cell
adhesion which leads to RV dysfunction
• Metabolism?
• Signaling?
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LIMITATIONS OF EXERCISE STUDIES IN ARVC
• Small numbers of patients, <100 most studies
• Many single center, predominantly caucasian
• Referral bias to tertiary care
• Associative, retrospective• Associative, retrospective
• Self reported exercise, no quantitative measures
• Many probands only (selecting for greater disease severity and risk)
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DON’T FORGET THE BENEFIT SIDE OF THE EQUATION
FOR PARTICIPATION IN ORGANIZED SPORTS
Organized sports build:
Aspenprojectplay.org
Organized sports build:
Leadership
Teamwork
Coping
Goal setting
Focus
Cooperation
Social skills
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ADVICE FOR AN ATHLETE WITH HCM OR ARVC
• Balanced discussion of existing data and perceived risk
• Support their autonomy if they wish to continue playing
• Alert teammates or training partners of their condition
• Investigate availability of AED on site, consider having a personal one
• Build a good aerobic base of conditioning (i.e. no weekend warriors!)
• Habitual exercisers in general population have much lower rate of SCD with
a bout of exercise than those that don’t exercise regularly
• Heed any warning signs – do not “push through”
• Know ICD settings to avoid inappropriate shocks, consider a HR monitor
• Hydrate, hydrate, hydrate
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ADVICE FOR A NON-ATHLETE WITH HCM OR ARVC
• Encourage low to moderate intensity exercise, at a minimum of
30 min, 5 days per week
• HCM patients are less active than general population• HCM patients are less active than general population
• Purposeful reduction in exercise (in 64%) had negative emotional impact
• Higher BMI (mean 31) vs NHANES (mean 28)
Reineck, Rolston et al., AJC 2013
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Business Insider
Brazil
CNN 2014
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INTERVENTIONAL TRIAL OF EXERCISE IN HCM:
RESET-HCM
• Collaborative study between U Michigan and Stanford• Randomized trial of 4 month moderate intensity exercise• Inclusion: Ages 18-80, Diagnosis of HCM• Enrollment completed – 128
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LIVE: LIFESTYLE AND EXERCISE IN HCM AND LONG QT
SYNDROME
PIs:
Rachel Lampert
Sharlene Day
Mike Ackerman
NCT02549664
livehcm.org
• 354 HCM patients and 535 LQT patients enrolled to date
• Ages 8-50, diagnosis of HCM or LQT or gene mutation carrier
• Interviews and questionnaires (phone, internet) and a fitbit
• Patients can SELF ENROLL
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TAKE HOME MESSAGESHCM
• Risk of SCD with competitive or recreational sports is very low and many may be able to safely participate
• Need more objective data for better risk stratification and guidance
ARVC
• Risk of SCD with competitive sport appears to be higher, at least for probands
• Vigorous exercise/training may exacerbate underlying ventricular remodeling
• A higher level of recreational exercise than has been traditionally recommended may be OK
OVERALL
• Need better quantitative measures of exercise beyond “competitive” or “recreational”
• Need more data for genotype+ individuals – discrepancy in US vs European guidelines
• Support informed decision making
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LIVE registry
Rachel Lampert (Yale)
Mike Ackerman (Mayo)
Maryann Concannon (Michigan)
Theresa Donovan (Yale)
RESET
Sara Saberi (Michigan)
Euan Ashley (Stanford)
Matt Wheeler (Stanford)
HCMA
Lisa Salberg