Assessing and Managing the Health of a Young Athlete Predisposed to Myoneural Dysfunction

Assessing and Managing the Health of a Young Athlete Predisposed to Myoneural Dysfunction Professor Sanjay Sharma St George s University of London sasharma@sgul.ac.uk @SSharmacardio

Sudden Cardiac Death in Athletes

Incidence of Sudden Cardiac Death POPULATION AGE DURATION INCIDENCE Organised high school 1 13-17 12 years 0.5/100,000 and college athletes Competitive athletes 2 14-35 25 years 2/100,000/yr Marathon (London) 3 Mean 42 26 years 2.2/100,000 runs Rhode island jogger 4 30-65 7 years 13/100,000/yr 1. Roberts WO. JACC. 2013; 62: 1298 2. Corrado D. JAMA. 2006; 296: 1953 3. Tunstall-Pedoe D. Sports Med. 2007; 37: 448 4. Thompson P. JAMA. 1982; 12: 247

90% deaths during or immediately after exertion Sudden Cardiac Death in Young Athletes Sudden Death In Young Athletes Incidence is approximately 1/50,000 Harmon. Heart. 2014; 15: 1185-1192 Mean age at death in athletes 23 years-old 40% deaths in athletes aged < 18 years old Males > females (9:1) Black athletes > white athletes

Sudden Cardiac Death in Sport Hypertrophic Cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy

Coronary arteries and aorta

Electrical Disorders Sudden Cardiac Death with a Normal Heart LQTS Brugada WPW

Causes of Sudden Cardiac Death in Sport 357 consecutive athletes. Mean age 29 ±11 years old. 92% Male. 69% competitive. Finnochiaro G, Sharma JACC 2016

Cardinal Symptoms of Cardiac Disease Chest tightness on exertion or emotional stress Breathlessness Palpitation Syncope

Cardinal Symptoms of Cardiac Disease Chest tightness on exertion or emotional stress Breathlessness Palpitation Syncope

Syncope Transient loss of consciousness and loss of postural tone. Rapid onset. Spontaneous recovery. Due to global cerebral hypoperfusion. Usually benign. May be indicative of a life threatening disorder or may precede cardiac arrest.

Syncope after Exercise Neurocardiogenic Neurological Orthostatic Cardiac Syncope Metabolic

London Marathon Experience 3% of contacts presenting to the medical team during the London Marathon. Mostly benign. Usually due to physiological factors

Volume depletion Cerebral Hypoperfusion/Syncope Altered Neuro-cardiogenic reflexes Empty LV Vasodilation Increased Mechanoreceptor activity Reduced Activity from Muscle Pump? Potentiated by sudden withdrawal of sympathetic tone Heat ADP Acidosis

Syncope after Exercise May be Serious Dehydration Adrenergic surges Electrolyte imbalance Acid/base disturbance

Assessment Aim is to exclude a potentially sinister cause History (helpful to have witness account) Examination (vitals, cardiovascular and neurological) (Blood glucose) ECG

Vasovagal Syncope Loss of vasomotor tone cardioinhibition Transient loss of consciousness Always upright More common in a hot environment Transient drowsiness but complete recovery No cardiac history Hypotension with relatively low heart rate (< 100 bpm) 4Ps Posture (patient is usually upright) at time of onset. Provoking factors (fatigue, dehydration, pain) Prodrome; unwell, dizzy, diminution of vision, nausea and vomiting. Pallor

Red Flags Age > 45 years old Syncope whilst (1) lying supine (2) syncope without warning and (3) syncope during exertion Chest pain, palpitation, headache, transient seizure like activity. New or unexplained breathlessness Known history of cardiovascular disease Family history of premature sudden cardiac death Cardiac murmur/focal neurology Abnormal ECG

Red Flags Age > 45 years old Syncope whilst (1) lying supine (2) syncope without warning and (3) syncope during exertion Chest pain, palpitation, headache, transient seizure like activity. New or unexplained breathlessness Known history of cardiovascular disease Family history of premature sudden cardiac death Cardiac murmur/focal neurology Abnormal ECG

Colvocci et al. EHJ 2004; 15: 1749

AF Abnormal repolarisation Long QT VT Complete Heart Block Role of the ECG Pre-excitation Ischaemia Multiple VEs Brugada ECG pattern Brugada syndrome

Management A B C Airway Breathing Circulation May need to proceed to cardiac arrest protocol (D) E F Determine blood glucose, core temp and plasma sodium (if slow to recover/disorientated and confused 5 minutes afterwards) Elevate legs (ECG monitoring) 12-lead ECG in all Fluids/fuel (orally)

Management of Post Exercise Syncope Fluid redistribution/replacement to improve cerebral and core organ circulation Elevation of legs and buttocks Oral fluids in the majority Carbohydrate fuel (Treat temperature abnormalities)

Indicators of Transfer to Hospital from a Field Hospital Environment Cardiac symptoms Previous history of cardiac disease. Focal neurology/disorientation or drowsiness despite normal blood glucose, core temp and plasma sodium. Cardiac arrhythmia. Systolic BP persistently below 90 mm Hg despite fluids ECG suggestive of a serious cardiac disease.

Cardiac Investigations History, Examination and ECG Possible Cardiac Cause Simple Faint Echocardiogram and Exercise Stress Test At least 48 hour ECG Monitoring Ziopatch REVEAL device

Case 1 33 year old male completed his first marathon in 3 h 20 min. Collapsed and was unconscious for approximately 30 secs. His friends reported that he was pale prior to collapse. The paramedics arrived at the scene within 1 minute. By this time the patient was conscious and was complaining of nausea.

Core temp 38 o C. Blood glucose 8 mmol/l Case 1 On examination he appeared pale and was slightly disorientated. No evidence of central cyanosis. HR 90/min and regular. BP 80 mm Hg systolic. RR 15/min Normal heart sounds. Chest clear. No focal neurology.

What would you do next? A Start IV fluids B Elevate legs/oral fluids C High concentration oxygen D Cool patient aggressively E Give glucogel

Case 1 Patient improved after his legs were elevated. He did not have any chest pain prior to collapse but recalls dizziness, nausea and diminution of vision prior to losing consciousness. Similar history 2 years ago after running a half marathon at high temperatures. Which investigation is required next? A Plasma sodium concentration B ECG C Echocardiography D Cardiac troponin E Serum BNP level

Next step ECG was normal. What would you do next? A Arrange out-patient 24 hour ECG monitoring B Echocardiography C Coronary angiography D Discharge; no further investigations necessary E Organise CT scan of the brain.

No obvious FH of note. No DH. Case 2 39 year old male completed his second marathon and collapsed shortly afterwards. Witnesses reported brief tonic-clonic movements whilst he was on the ground. Rousable after 1 minute. No warning before collapse apart from fatigue.

Case 2 On examination he appeared pale and was slightly disorientated for a few minutes. No evidence of central cyanosis. HR 100/min and regular. BP 108 mm Hg systolic. RR 15/min Normal heart sounds. Chest clear. No focal neurology. Core temp 39.2 o C Blood glucose 9.4 mmol/l Oxygen sat 94%

Case 2

What would you do next? A Arrange out-patient 24 hour ECG monitoring. B Transfer to hospital for further assessment and monitoring. C Discharge; no further investigations necessary

Electrocardiogram demonstrating the right precordial leads V1-V3 ST-segment changes prior, at peak and during the recovery phase of the exercise stress test V1 V2 V3

Case 3 53 year old man collapsed suddenly just before the finish line after a half marathon. Transient seizure activity. Head and facial lacerations. No pulse felt by paramedics with immediate bystander CPR commenced for a minute and prompt return of spontaneous circulation. Confused for over 20 minutes afterwards

Case 3

Case 3 Blood tests normal with exception of a mild rise in cardiac troponin. Echocardiogram- normal biventricular function with no valve pathology

ETT performed few days later- 15 minutes 34 seconds (18 METS). No ECG features of ischaemia.

Screening Strategies for Detecting Athletes with Potentially Serious Cardiac Disease

Screening Athletes Condition History Examn ECG Echo HCM Pos/Neg Pos in 25% Positive Pos ARVC Pos/Neg Negative Positive Neg/Pos WPW Pos/Neg Negative Positive Neg LQTS Pos/Neg Negative Positive Neg Marfan Pos/Neg Positive Negative Pos CAA Pos/Neg Negative Negative Neg Myocarditis Pos/Neg Pos/Neg Pos/Neg Pos INCREASING COST

ECG in Patients with Cardiomyopathy Inferior and/or lateral TWI ST segment depression Pathological q waves HCM ARVC 95% 40-50% Anterior TWI (V2-V3/V4) with isoelectric J point Epsilon wave Ventricular extra-systoles

NEJM, 1998; 339(6):364-369 33,735 competitive athletes were screened 22 athletes diagnosed with HCM 3 identified on basis of family Hx 16 identified by abnormal ECG 2 identified on basis of murmur (14%) (9%) 73%

Does Cardiac Screening Save Lives?

TIME-TREND OF SUDDEN CARDIAC DEATH INCIDENCE IN ATHLETES VS NON-ATHLETES Veneto Region of Italy 1979-2002 Corrado. JAMA 2006; 296:1593-1601

Concerns Relating to ECG Screening Low incidence of sudden cardiac death High number of false positives Concerns relating to false negatives Cost Other issues

Prevalence of Young Athletes with Conditions Predisposing to SCD Reference Population Prevalence AHA (2007) Competitive athletes (U.S.) 0.3% Fuller (1997) 5,617 high school athletes (U.S) 0.4% Corrado (2006) 42,386 athletes age 12-35 (Italy) 0.2% Wilson (2008) 2,720 athletes /children age 10-17 0.3% Bessem (2009) 428 athletes age 12-35 (Netherlands) 0.7% Baggish (2010) 510 collegiate athletes 0.6% Sheikh (2015) 5000 British elite athletes 0.3%

Concerns Relating to ECG Screening Low incidence of sudden cardiac death High number of false positives Concerns relating to false negatives Cost Other issues

ECG Interpretation in an Athlete

TWI in a Black Athletes 12.4%

53% 6%

Differences from the ESC 2010 Following included in the normal category: T wave inversion in V1-V4 in black athletes. T wave inversions in V1-V2 in all athletes. QTc < 470 M and < 480 F. Criteria for RVH also include RAD

Evidence Based ECG Interpretation: 2004-2014

Sensitivity for all conditions 60% Sensitivity for serious conditions 100% Specificity 94% in Caucasians 84% in Black athletes

The Athlete s Heart - Overlap with Cardiomyopathy Physiology Pathology vs. Electrical Sinus bradycardia Voltage for chamber enlargement Repolarisation anomalies Functional Increase in stroke vol. Structural Increased cavity size Increased wall.thickness Overlap with cardiomyopathy. Differentiation between the 2 entities is crucial.

Overlap With Disease Anabolic drug abuse Long standing endurance athlete Black athletes Juvenile EKG pattern T-Wave Inversion Cardiomyopathy

Concerns Low incidence of sudden cardiac death High number of false positives Cost Concerns relating to false negatives

4,925 athletes (age 14-35 years) Mandatory requirement Previously un-screened 83% Male 85% White 26 Sporting disciplines Screening protocol H+P (12-point AHA) (1.6%) 12-lead ECG (2010 recommendations) (21.8%) Further investigations following positive screen Prospective follow-up Workload and cost implications Contemporary criteria applied retrospectively

Screened cohort Impact of Refined criteria >50% in positive screens 50% 8% No compromise in sensitivity 4,925 athletes 12% 18% Dhutia et al. JACC. 2016

Potential use of savings 2010 ESC recommendations Cost per athlete: $110 Cost per diagnosis: $35,993 Seattle criteria Cost per athlete: $92 Cost per diagnosis: $30,251 Screen an additional 2,120 athletes Refined criteria Cost per athlete: $87 Cost per diagnosis: $28,510 21% cost reduction Purchase an additional 95 AEDs Dhutia et al. JACC. 2016

Concerns Low incidence of sudden cardiac death High number of false positives Cost Concerns relating to false negatives

Deaths Despite Screening with ECG

Alternative Strategies

Exercise related cardiac arrest Incidence in the general population France (2005 2010) Mean age 46.1 ± 15.8. 93% Male. Survival 15%

Exercise related cardiac arrest Incidence in the general population Netherlands (2006 2009) Mean age 58.8 ± 13.6. 95% Male. Survival 45%

Exercise related cardiac arrest Country Netherlands France Age, years 58.8 ± 13.6 46.1 ± 15.8 Men 93% 95% Success rate 45% 15% Bystander witnessed arrest 89% 94% Bystander CPR 87% 31% AED use 36% 1% Shockable initial rhythm 80% 47% Time to first shock (min) 9.8 (6.4 12.5) 12.5 (10.5 15.5)

Kim et al NEJM 2012 10.9 million runs 59 deaths. 29 % survival death rate FACTOR ODDS RATIO By stander CPR 3.73 CI 2.19-6.39 Time of collapse to CPR 1.32 CI 1.08-1.61 Initial use of AED 3.71 CI 2.07-6.64

Drezner 2009 Report of 1710 US high schools with an on-site AED program. Survey relating to sudden cardiac arrest (SCA) between Jan 2006- July 2007 36 cases of SCA Prompt CPR 94% AED shock 83% 14 (high school) 22 older non students Mean age 16 Mean age 57 64% survived to hospital discharge in each group Higher survival rates may have been to the onsite AED (79%) and smaller number of cases of hypertrophic cardiomyopathy (21%)

Delay Intervals Mean time from collapse to CPR 1.5 Minutes Mean time from SCA to first shock 3.6 Minutes

The Emergency Response Plan Personnel Communication system Emergency response plan Location of the AED Practice and review of emergency response plan

120 100 90 97 % 80 60 40 20 72 64 41 65 26 0

Premier League March 2012

Key Messages Sudden cardiac death (SCD) in young athletes is rare. Exercise is a trigger for SCD in predisposed athletes. Syncope is relatively common in athletes. Most cases are benign but may represent serious cardiac disease. The history (including witness reports) is an important discriminator. Pre-participation screening with ECG identifies athletes with electrical disease and cardiomyopathy. The ECG cannot identify all athletes at risk of SCD. Early CPR and AEDs save lives in sport.

Assessing and Managing the Health of a Young Athlete Predisposed to Myoneural Dysfunction Professor Sanjay Sharma St George s University of London sasharma@sgul.ac.uk @SSharmacardio