THE SCOPE OF THE PROBLEM Athlete Deaths Phidippides 530 BC 490 BC - - PowerPoint PPT Presentation

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6/18/2018

Theodore P. Abraham, MD, FACC

Meyer Friedman Distinguished Professor of Medicine UCSF HCM Center of Excellence University of California at San Francisco

Headlines you DON’T want to see

Disclosures: none

Athlete Deaths – Phidippides 530 BC – 490 BC

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THE SCOPE OF THE PROBLEM

 3000 sudden deaths per year in ages 15-34 years (CDC)  125-300 high school athletes a year (informal estimates)  The population  4 million competitive high school–age athletes (grades 9 - 12)  ~500 000 collegiate  ~ 5000 professional  unknown # of youth, middle school, and masters level competitors  Prevalence of field deaths uncertain  estimated 1:100 000 to 1:300 000 high school–age athletes  disproportionately higher in males  Older athletes estimated at  1:15, 000 in joggers  1:50, 000 in marathon runners Maron 1980, 1996 Thompson 1982 Van Camp 1995

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THE DISEASE MIX

 < 35 years

 Hypertrophic cardiomyopathy ~ ⅓  Coronary artery anomalies

 > 35 years

 Atherosclerotic heart disease

 Deaths most common in basketball and football

Maron 1996

WHY EVALUATE?

 Purpose of screening

 provide medical clearance in competitive sports

• identify clinically relevant and preexisting cardiovascular abnormalities
• reduce the risks associated with organized sports.

 Rationale

 intense athletic training >> increases risk for sudden cardiac death/disease progression

 not possible to quantify that risk

 majority of young athletes die during athletic training or competition  early detection permits timely interventions that may prolong life.

Maron 1980 Burke 1991 Van Camp 1995

AHA VIEWS ON SCREENING

Some form of pre-participation cardiovascular screening for high school and collegiate athletes is justifiable and compelling, based on ethical, legal, and medical grounds.

a) Noninvasive testing can enhance the diagnostic power

• f the standard history and physical examination

b) Not prudent to recommend routine use of such tests as ECG, Echo or graded exercise test

• Large number of competitive athletes in U.S.
• Relatively low frequency of life-threatening cardiovascular lesions
• Low rate of sudden cardiac death

12-element AHA screening guide

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Medical history (with parental confirmation) Personal history

• 1. Exertional chest pain/discomfort
• 2. Unexplained syncope/near-syncope (not neurocardiogenic/vasovagal)
• 3. Excessive exertional and unexplained dyspnea/fatigue, associated with exercise
• 4. Prior recognition of a heart murmur
• 5. Elevated systemic blood pressure

Family history

• 6. Premature death (sudden and unexpected, or otherwise) before age 50 years due to

heart disease, in ≥1 relative

• 7. Disability from heart disease in a close relative <50 years of age
• 8. Specific knowledge of certain cardiac conditions in family members: hypertrophic or

dilated cardiomyopathy, long-QT syndrome or other ion channelopathies, Marfan syndrome, or clinically important arrhythmias Physical examination

• 9. Heart murmur - supine and standing
• 10. Femoral pulses to exclude aortic coarctation
• 11. Physical stigmata of Marfan syndrome
• 12. Brachial artery blood pressure (sitting position) – both arms

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AHA RECOMMENDATIONS ON SCREENING

 Not intended to discourage population screening  Concern re: false-positive test results

 unnecessary anxiety among athletes and families  unjustified exclusion from life insurance coverage and athletic competition  Estimate that false-positives exceed true-positive results.

AHA CONCLUSIONS ON SCREENING

 Personal and family history and physical examination for known cardiovascular risk lesions is the best available and most practical approach to screening, regardless of age.  Such screening is an obtainable objective and should be mandatory for all athletes.  We recommend... history and a physical examination in

• rganized high school (grades 9 through 12) and collegiate
• sports. Screening should then be repeated every 2 years.

EVALUATION INADEQUATE ALTERNATIVE OPINIONS

 European Society of Cardiology (ESC) and the International Olympic Committee (IOC) recommend ECG screening in all athletes.  So why did the AHA decide otherwise

 Cost

 2.5 million amateur competitive athletes (high school + college) in U.S.  Yearly ECGs will cost an estimated $2 billion

 includes additional tests to evaluate significance of ECG abnormalities ECG screening

 False positives would deny opportunity to those at very low risk for cardiovascular events.

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COMMENTS RE AHA APPROACH

 Argues AGAINST screening – but recommend screening  Difference in opinion on what constitutes screening

 AHA recommends the 12-point screen – NO ECG or other investigation

 Argue there is physician shortage for ECG and echo screen

 but recommend physician examine every athlete  compares physician resources to Italy – but primary care coverage is similar

 PCPs per capita 1/1000 in the U.S. vs. 0.9/1,000 in Italy (OECD data 2009)  Italy – 90% ↓ in athlete deaths aer ECG screen started in 1983 [Corrado 2006]

 Athlete death estimates unreliable

 based on media reports

 Cost analysis outdated/flawed

 Overestimates false positive ECGs; abnormals are manageable  Overestimates echo costs - $500 per echo = $250,000 per detection

 - Shorter protocols could reduce cost to $25,000 per detection

 Ignores recent advances in echo techniques Marek 2010 Corrado 2006

Screening athletes is challenging

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Wide range + overlap in athlete vs pathology

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Obvious Anatomic/Functional abnormalities are detectable

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>90% detectable by Echo

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KEY FEATURES OF HCM

Hypertrophy Systolic Anterior Motion Outflow Gradients

HCM WITH SAM

CONTRAST IN POOR QUALITY ECHO

SCD Risk assessment

Athletic hypertrophy

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2% of athletes 2 years of training >5 hrs/week REVERSIBLE on detraining

Maron 1995 Maingourd 1990 Mesko 1989 Obert 1998 Pellicia 1991

HCM >14 F >16 M Normal <12

12-16 mm

10% of US Football players > 13 mm

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Normal range of physiologic LVH

• 947 athletes
• None > 16 mm
• <2% (n=16) in HCM

range (>13 mm)

• 15 rowers
• 1 cyclist
• ECG normal in 9/16

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Pellicia 1991

Racial Differences

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• 300 black athletes
• >15 mm
• 3% (n=9)

black

– No white

• All LV>55mm

Sharma 2002

Separating HCM from Athlete’s Heart

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Challenging Challenging

Often uncertain

Over-Dx Over-Dx

Interrupt training Elimination from competition

Mis-Dx Mis-Dx

Potential SCD risk Family not screened

HCM vs Athlete heart

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LVH

• Age
• Sex
• Ethnicity
• Size
• Type of training

History

• Fam Hx of SCD
• Hx of Heart Dz
• Murmur
• Isotonic +

Isometric

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EKG; Cardiopulmonary stress

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EKG

• T inversion
• Pathologic Qs
• ST depression

CPET

• Peak VO2<50

ml/kg/min - HCM

Sharma 2000

Racial differences

Healthy HCM

COST EFFECTIVENESS OF ECG

Adding ECG vs. history + physical exam

 Costs $88 per athlete  Saves 2.1 life-years per 1,000 athletes  Cost-effectiveness $42,900 per life-year saved

 Adding ECG vs. no screening

 Costs $199 per athlete  2.6 life years per 1,000 athletes  Cost-effectiveness $74,100 per life-year saved

 Wheeler; Ann Int Medicine 2010

 Study of 510 student athletes

 Addition of ECG to history + physical  Improved detection of echocardiographically documented cardiac abnormalities from 5 to 10 out of 11 but increased the false-positive result rate from 5.5% to 16.9%

 Baggish; Ann Int Medicine 2010

ECG CONCERNS

ECG good

Validity of adding ECG to Pre- participation Screen

• Meta analysis of 16 papers
• Represents best clinical practice to

prevent or reduce the risk of sudden cardiac death in athletes.

• Significantly improves sensitivity of

history and physical examination alone;

• Has reasonable specificity and

excellent negative predictive value; and it is cost-effective.

ECG questionable

Variability in interpretation – an

• bstacle to screening
• 138 athletes  7 cardio & 7 sports

MD

• Corrado, Uberoi, Marek and Seattle

criteria

• 7% abnormal by Seattle
• 11-14% by other criteria
• Seattle reduced abn ECGS; ↑

agreement

• Variability remains high

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Alattar 2015 Berte 2015

Imaging

HCM

• LV cavity NL/small
• <45 mm
• Diastology Abnormal
• LA dilated
• RWT ↑

Athlete Heart

• LV cavity dilated
• 55-65 mm
• Diastology Normal
• LA Dilated
• RWT ↓

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REST 6 mmHg

EXERCISE 12 mmHg

SAM & HEMODYNAMICS

REST 90 mmHg

Exercise 150 mmHg

LVOT gradients

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Or BOTH

THE HOPKINS HEART HYPE EXPERIENCE – HS ATHLETES

 Heart Hype

 Brief multimodality screen  Questionnaire + cardiac ascultation+ BP + ECG + focused echo  High school age athletes (~500 screened)

 How accurate is the questionnaire

 14 pt modified AHA questionnaire

554 student athletes

 60% male  Mean age 17±1.5 for boys and 16±1.3 for girls  All asymptomatic

Sensitivity Specificity p-Value¹

AHA Quest Vs Echo

47.5% 39.5% >0.05

AHA Quest Vs EKG

56.5% 40.6% >0.05

AHA Quest Vs Echo& EKG Combined

57.3% 43.2% >0.05

¹Fisher’s, ‘N-1’ Chi-square and Z- tests for association

Low sensitivity and specificity for prediction of EKG or Echo findings.

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ECG vs. Echo (n=134)

• Abnormal findings in 36 (27%) athletes
• EKG only in 22 (61%)
• non-specific ST-T waves
• voltage criteria for LV hypertrophy
• sinus bradycardia
• Echo only in 9(25%)
• septal thickness≥10mm
• EKG and Echo abnormalities in 5 (13%)
• Concordance – EKG and echo low (kappa=0.12;p=NS).
• No significant valvular abnormalities or cardiac pathologies found
• Conclusions:
• Use both ECG and echo for screening

PATHOLOGIC VS PHYSIOLOGIC LVH

The Role of Mechanics (Deformation)

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WHY MECHANICS? THE HEART IS A

MECHANICAL ORGAN

Cyclic changes

muscle length wall thickness

Changes are quantifiable

Rate of change Extent of change

17-year old; 2-sport athlete Borderline hypertrophy; No SAM; normal EF/WM

• Diagnosis when morphologic features are ABSENT

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REGIONAL MECHANICS ABNORMAL

DIASTOLIC STRAIN RATE IS LOW IN HCM VS. ATHLETES

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2 4 6 8 10 12 14 16 HCM Athletes Normal HCM Athletes Normal Late diastole Early diastole Late diastole Early diastole

Palka: JACC 30:760, 1997 Palka: JACC 30:760, 1997

STRESS MECHANICS BLUNTED RESPONSE IN PATHOLOGY

CON Labile Non

Pozios: J Am Soc Echo 2018 Pozios: J Am Soc Echo 2018

CMR

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LVH Atypical LGE HCM Assoc Abn. RV; coronaries

Pre and post de-training

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HCM – LVH can occur anywhere

Chun 2010

CMR DISTRIBUTION/SEVERITY OF LVH DELAYED ENHANCEMENT

De-training

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Detrain HCM PHYSIOL

3-6 months  2-5 mm

NO YES

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Other

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Endomyocardial Biopsy

Not Useful Often non conclusive

Genetics

Yield ~50-60% Useful if (+)

Genetic Testing

Larger panels – more coverage

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High rate of non informative results

Thought Process

• Differentiation

challenging

• History to advanced

imaging

• Long-term monitoring
• Fam Hx
• Type of training
• EKG
• Echo – conventional
• Echo – mechanics
• CMR
• Stress/CPET
• Genetics
• Detraining
• Screen Relatives

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DETECTION = PREVENTION

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Our Approach

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Customized Mechanics Stress CT CMR

UCSF HCM Center of Excellence Athletic Heart Center 415-353-2873

Take home points

Athlete deaths are a distressing problem for society; # uncertain Current AHA recommendations popular - ?efficacy Balance costs vs. efficacy

• History and physical ALONE unlikely to be high yield

Most affected athletes are asymptomatic

• Symptomatic athletes will seek care via conventional routes

ECG or echo alone will miss abnormalities

• Long QT by echo; Marfan’s/HCM/coronary abn by EKG

Standardized protocols Novel Imaging techniques

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Strain is low in HCM not HTN

• HCM (n=20) and HTN

(n=14)

• Biopsy in all
• Systolic strain of 10%
• Sensitivity 85%
• Specificity 100%

Kato: Circulation, 2004 Kato: Circulation, 2004

Wall thickness ratio Strain