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

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

• 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

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

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

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

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

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”

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

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

ENDURANCE ATHLETICS MAY BE MORE

INFLUENTIAL IN GENOTYPE NEGATIVE ARVC

Sawant et al., JAHA 2014;3:e001471

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

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?

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)

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

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

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

Business Insider

Brazil

CNN 2014

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

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

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

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