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Sports Participation in Patients withKnown Heart DiseaseRavi Mandapati, M.D., FACC.; FHRSDirector, Specialized Program for Arrhythmias in Congenital Heart DiseaseUCLA Cardiac Arrhythmia CenterDavid Geffen School of Medicine at UCLADirector, Pediatric Cardiac ElectrophysiologyLoma Linda University Medical CenterJanuary 14‐15, 2011 SCA Conference1

Outline Sports and SCD: Scope of problemSports and Inherited disorders ( GCVD)Sports and congenital heart diseaseGuidelinesRestrictions What is the basis for restrictions– DataD iis scarce– Guidelines 2004, 2005, 2008 Definition of Competitive vs recreational sports Liability concernsyp ppositive pphenotypeyp negativegppatients Genotype AICD’sJanuary 14‐15, 2011 SCA Conference2

Causes of SCD under 40 years# Deaths per 30Channelopathy40OtherAge (yrs)Asthma (8)Causes of Sudden Death at autopsy inMaron, B. J. N Engl J Med387 High School AthletesHeat2003;349:1064-1075Stroke (6)Myx MV(9)Drug abuse (4)Other CV (4)LQTS (3)Sarcoid (3)DCM (9)Cor ArtDisease (10)Cardiac trauma ((3))AorticStenosis (10)Rupt cerbral artery (3)MyocardialBridge (11)HCM (102)ARVD (11)Ruptured AoAneurysm (12)Myocarditis (20)LVH (29)January 14‐15, 2011 SCA ConferenceCoronaryanomalies (53)CommotioCordis (77)3

Causes of SCD in young athletes in theVeneto region of ItalyConduction systemdisease 8.2%PulmonaryEmbolus 2%Corrado et al, Ann Int Med1998;339:364HypertrophicCM 2%Anomalous CoronaryArtery 1212.2%2%Mitral ValveProlapse 10.2%Myocarditis 6.1%DilatedCardiomyopoathy 2%Dissectingaortic aneurysm 2%Coronaryatherosclerosis 18.4%RV Cardiomyopathy 22.4%Myocardialbridging 4.1%Impact of ECG based pre-participation screeningprogramme in Veneto region of ItalyCorrado, D. et al. JAMA 2006;296:1593-1601.2,009,600 persons in Veneto region of Italy 35 years old269 sudden unexpected cardiac deaths (49athletes and 220 nonathletes) 33,735 athletes underwent 73,718screenings 3,016 (8.9%) required echocardiogramsJanuary 14‐15, 2011 SCA Conference1,058 disqualified, 621 (1.8%) for cardiac reasons;HTN 27.1%SVTs 7.6%HCM 3.5%AVB 1.6%PVCs / VT 9.5%WPW 7.1%LBBB or RBBB / LAD 1.9%Long QT 0.6%4

Absolute Risk of Sudden Cardiac DeathDuring Exertion in Men versus WomenRiskk off SCD dduringRiimoderate/vigorousexertion:Risk of SCD duringlesser or no exertion:Risk Difference:MenWomen1 SCD per 22.88 millionperson-hours at risk1 SCD per 1717.99 millionilliperson-hours at risk1 SCD per 23 millionperson-hours.1 SCD per 66 millionperson-hours.1 excess SCD per 3.2million person hours1 excess SCD per 24.5million person hours88%73%Attributable RiskPercentAlbert et al, Physicians Health Study, N Eng J Med 2000Classification of Sports Type of exercise- Dynamic- Static Level on intensity– Competitive vs recreational Danger of bodily collision *January 14‐15, 2011 SCA Conference5

Definitions Dynamic exercise –Changes in muscle lengthandd jointsj i t withith rhythmich th i contractionst tiandd littlelittlintramuscular force Static exercise – large intramuscular force withlittle or no change in muscle lengthCLASSIFICATION OF SPORTSJanuary 14‐15, 2011 SCA Conference6

GCVDLong QT SyndromeEarly Repolarisation /J point elevationBrugada gicPolymorhic VTShort QT Syndrome2004 GuidelinesJanuary 14‐15, 2011 SCA Conference7

GCVD and Exercise/Considerations Arrhythmogenicity of sportsBurst exertionAuditory stimuliSwimmingDiving– Restricted in all GCVD Greater risk of trauma in sports such as rockclimbing, downhill skiing, ice hockey Increased stress/surges of emotion : roller coasters Paired athletic activitiesLong QT Long QT patients with QTc greater than 0.48 sin males and 00.4848 s in females are restrictedfrom high intensity competitive sports– ESC 0.44 males and 0.46 females Genotype negative and phenotype positive arediscouraged from participation in sports– ESC allll gene positivesiiexcludedl d d Genotype negative and borderline QTc areallowed to participate with close surveillanceJanuary 14‐15, 2011 SCA Conference8

Brugada syndrome Restricted from sports– No clear association between exercise and SCD inBrugada ( potential impact of hyperthermia) Genotype positive and phenotype negative, norestriction– ESC : restrictedARVD and CPVT CPVT– Restricted from sports ARVD– Restricted from sportsJanuary 14‐15, 2011 SCA Conference9

WPW EP study/ablation– Required in pts with impaired consciousness orpersistent palpitations– Advisable in asymptomatic pts engaging inmoderate or high level competitive sports– Asymptomatic athletes with WPW , EPS is notmandatory ESC: mandatory– Return to sports: 4 wks/3 months post EPSPVC’s No limitations in athletes without cardiacdiseasedi Low intensity sports in athletes where PVC’sincrease with exerciseJanuary 14‐15, 2011 SCA Conference10

NSVT No restrictions , in Asymptomatic athleteswithoutith t CHD , no NSVT 10 beats,b t 150/min and demonstrate suppression duringexerciseHCM: Recommendations Excluded from all sports except low intensitysportst suchh as golflf HCM with low risk features– SCD of Cameroon soccer player Marc Vivien Foe Gene positive phenotype negative individuals– US/BC : not pprecluded from sportsp– ESC: restricted, based on regular exercise may play arole in triggering cellular mechanisms leading to HCMphenotype/ cell death and myocardial fibrosisJanuary 14‐15, 2011 SCA Conference11

TASK FORCE 2: Congenital Heart Disease Left-to-right shunting lesions: ASD, VSD, PDA Obstructive lesions: Pulmonary valve stenosis,stenosisAortic valve stenosis, Coarctation of the aorta Cyanotic congenital cardiac disease –unoperated/operated Congenital coronary artery anomaliesAnomalous Coronary ArteriesIntra-arterial courseof Left Main Cor ArtExclusion from all competitive sportsParticipation 3 months after surgery after echo, max excercise testingJanuary 14‐15, 2011 SCA Conference12

Atrial septal defect (ASD)- small or large defect without pulmonary HTN– can participate in all competitive sports- with pulmonary HTN – low intensity sportsonly (Class IA).- with PVOD (pulmonary vascular obstructivedisease). Cannot participate in competitivesports.ASD: s/p closure 3-6 months after closure can fully participatei competitiveini i sports if- No pulmonary hypertension- No arrhythmias- No evidence of myocardial dysfunctionJanuary 14‐15, 2011 SCA Conference13

VSD (ventricular septal defect) Small, restrictive defects – can participate in allcompetitive sports Large VSD – VSD closure recommended.If no pulm HTN – low intensity sports only (IA) 6 months post closure, full participation if:No residual or small residual defect, nopulmonarylHTN, no evidencedoff myocardiald ldysfunction.VSD (ventricular septal defect) Small, restrictive defects – can participate in allcompetitive sports Large VSD – VSD closure recommended.If no pulm HTN – low intensity sports only (IA) 6 months post closure, full participation if:No residual or small residual defect, nopulmonarylHTN, no evidencedoff myocardiald ldysfunction.January 14‐15, 2011 SCA Conference14

Pulmonary stenosis s/p interventionIf adequate relief and normal ventricularf tifunction. Balloon valvulolastyresume full activity after 1 month Surgical valvotomyresume full activity after 3 monthsMild aortic stenosisGradient 20 mmHgCan fully participate if normal ECG,normal exercise tolerance,asymptomatic (no history of exerciserelated chest pain, syncope, orarrhythmia)January 14‐15, 2011 SCA Conference15

Moderate aortic stenosis Gradient 21-40 mmHGMild LVH by echocardiographyNo LV strain on ECGNormal exercise test without ischemia orarrhythmiaLow static/low to moderate dynamic (ClassIA & IB)Moderate static/low dynamic (Class IIA)Severe aortic stenosis Gradient 50 mmHg NO COMPETITIVE SPORTSJanuary 14‐15, 2011 SCA Conference16

Sub AS and Supra Valvar AS Aortic stenosis Criteria also appliesto discrete (membraneous) subaorticstenosis and supravalvar stenosisCoarctation - untreated Mild coarctationNo severe collaterals vessels, no severe aortic rootdilation, normal exercise test, small pressuregradients at rest, peak systolic BP 230 mmHgwith exercise– Can fully participate in competitive sports MMore ththan mildild (systolic( t li arm tot legl gradientdi t 20mmHg or peak systolic blood pressure 230mmHg with exercise) – Low intensity sports(Class IA) only until treated.January 14‐15, 2011 SCA Conference17

Coarctation – post operative Participation in sports 6 months after treatment ifsystolic arm to leg gradient 20 mmHg. Normalpeakk systolict li BP att restt andd withith exerciseiNO high intensity static exercise (Class IIIA, IIIB,IIIC). No contact sports during first postoperative year. After first yearAll sports except power lifting if asymptomatic,asymptomaticnormal BP at rest and exercise.Coarctation - untreated Mild coarctation – Can fully participate incompetitive sportsNo severe collaterals vessels, no severe aortic rootdilation, normal exercise test, small pressuregradients at rest, peak systolic BP 230 mmHgwith exercise More than mild (systolic arm to leg gradient 20mmHg or peak systolic blood pressure 230mmHg with exercise) – Low intensity sports(Class IA) only until treated.January 14‐15, 2011 SCA Conference18

Cyanotic Heart disease - untreated Exercise intolerance and progressivehhypoxemiai withith increasingii effortff t Low-intensity competitive sports (class IA)Cyanotic heart disease - palliated Arterial saturation above 80%No symptomatic arrhythmiasNo ventricular dysfunctionNear-normal capacity by exercise testingLow intensity sports (Class IA)January 14‐15, 2011 SCA Conference19

Tetralogy of Fallot- s/p repair Normal or near normal right heart pressureOnly mild RV volume overloadNo residual left-to-right shuntNo rhythm abnormality by Holter orexercise studyCan participate in all competitive sportsTetralogy of Fallot –s/p repair Residual RVH 50% systemic Severe pulmonary regurgitation Rhythm abnormality by Holter or exerciseLow intensity sports (Class I A)January 14‐15, 2011 SCA Conference20

TGA (Transposition of the great arteries) –s/p arterial switch Normal heart sizeNo residual defectsNormal ventricular functionNormal exercise studyNo arrhythmiasCan participate in all sports, however, highstatic sports with severe isometric effort(Class III A, IIIB, IIIC) should bediscouraged.Marfan’s syndrome Low or moderate competitive sports in absenceoff– Aortic root 4 cm/ 2SD from mean for BSA– Moderate MR– F/H of aortic dissection or SCD ESC– All phenotype or genotype positive are restrictedJanuary 14‐15, 2011 SCA Conference21

Conclusions Current guidelines possibly too restrictive Legal issues Individual exemptions may be made afterdetailed discussions and documentation Genotype positive phenotype negative caseswillill increasingii needd tot beb addressedddd AICD and sportsJanuary 14‐15, 2011 SCA Conference22

VSD (ventricular septal defect) Small, restrictive defects – can participate in all competitive sportscompetitive sports Large VSD – VSD closure recommended. If no pulm HTN – low intensity sports only (IA) 6 months post closure, full participation if: No residual or small residual defect, no puldfdllmonary HTN, no evidence of .