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Sudden death whilst swimming the cardiac channelopathies Dr Anthony J Handley Medical Adviser, International Lifesaving Federation of Europe Director, European Resuscitation Council

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An 11-year old moderate swimmer in a supervised lesson in the shallow end of the pool He dived under water to pick up an object but failed to surface

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A swimming instructor pulled him from the pool and lifeguards commenced CPR within 2-3 minutes In VF when paramedics arrived could not be resuscitated

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The findings in the lungs at autopsy were fully consistent with drowning The underlying reason as to why he ran into difficulties is not apparent from the post mortem

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The coroner concluded: After considering the expert medical evidence, and on the balance of probability, we were unable to identify the primary event that led to Nathan Scott Matthews drowning

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Causes of sudden death in the water Sudden cardiac death Heart attack or stroke Trauma causing unconsciousness Diagnosed at autopsy

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Causes of sudden death in the water Seizures, including epilepsy No autopsy abnormalities Diving response or cold shock Shallow water blackout Sudden cardiac death

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Sudden cardiac death (SCD) is defined as: ... death due to cardiac causes... within one hour of the onset of acute symptoms... Myerburg & Castellanos A. In Braunwald Heart Disease 1997: 74279

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Priori et al. Eur Heart J 2001; 22: 13741450 General population 1 : 1000 pa Borjesson & Pelliccia. Br J Sports Med 2009; 43: 644-648 Young people (< 35yrs) 0.3-3.6 (1) : 100,000 pa

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Corrado et al. J Am Coll Cardiol 2003; 42: 1959-1963 3 x the risk Non-athletes (< 35yrs) 0.7 : 100,000 pa Athletes (< 35yrs) 2.1 : 100,000 pa

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Kenny & Martin. Arch Dis Child 2011; 96: 5-8 Estimated 1.5% 2.5% SCD occur during swimming 1 : 100,000 young people at risk swimming-related SCD

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Ischaemic heart disease 51% ARVC 8% Myocarditis 4% Aortic stenosis 3% Coronary abnormalities 4% HCM 7% Unexplained 11% Surez-Mier et al Forensic Sci Int 2013; 226: 188-196 SCD during sport

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Ischaemic heart disease 14% ARVC 15% Myocarditis 5% Aortic stenosis 4% Coronary abnormalities 6% HCM 18% Unexplained 23% Surez-Mier et al Forensic Sci Int 2013; 226: 188-196 SCD during sport aged < 35 years

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Tester & Ackerman Pediatr 2012; 33: 461-470 Using strict criteria autopsy-negative SCD in younger subjects - 8%

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Nearly 30% of under-35-year-olds who suffer autopsy-negative SUD have a cardiac channelopathy Kenny & Martin. Arch Dis Child 2011; 96: 5-8

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An ion channel is the route that the ions (sodium, potassium, calcium) take in and out of the heart muscle cells to allow the movement of electricity

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Catecholaminergic polymorphic ventricular tachycardia (CPVT) Long QT Syndrome (LQTS) Brugada Syndrome PCCD (progressive cardiac conduction defect) Short QT syndrome (SQTS) Familial atrial fibrillation Sodium channel disease Ion channelopathies

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Catecholaminergic polymorphic ventricular tachycardia (CPVT) Long QT Syndrome (LQTS) Brugada Syndrome PCCD (progressive cardiac conduction defect) Short QT syndrome (SQTS) Familial atrial fibrillation Sodium channel disease Ion channelopathies Associated with swimming SCD

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Normal LQTS

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Swimming appears to be a gene-specific (KVLQT1)... trigger for LQTS... and unexplained drowning... may have a genetic basis Ackerman et al. Mayo Clin Proc 1999; 74: 1088-1094

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Pre-participation screening

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Young Competitive Athletes Family & Personal History Physical Examination ECG Negative FindingsPositive Findings Eligible for CompetitionFurther Investigations No cardiac Disease Cardiac Disease Treatment as necessary Corrado et al. Eur Heart J 2005; 26: 516-524

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False-positive results 2% Papadakis & Sharma. Br J Sports Med 2009; 43; 663-668

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30 Corrado et al. Eur Heart 2005; 26: 516-524 Europe $ 75 Maron et al. Circulation 2007; 115: 1643-1655 USA Cost of preventing each death would be $ 3.4 million

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14,220 Corrado et al. Abstract presented at American Heart Association 2004 37,750 Cost per year per life saved X

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1997-2009 Millennium Mathematics Project, University of Cambridge http://www.plus.maths.org/latestnews/jan-apr10/qaly/ NICE: National Institute of Clinical Excellence NICE's threshold for cost-effective treatment lies between 20,000 and 30,000 per QALY

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Hope et al. Medical Ethics and Law 2003. Churchill Livingstone: Chapter 13 Kidney dialysis 45,000 Coronary artery bypass graft 26,000 Breast cancer screening 6,000 Cervical cancer screening 200 Pre-participation screening 12,350

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The best strategy is to combine pre- participation identification of athletes affected and secondary prevention with back-up defibrillation on the athletic field. Corrado et al. Eur J Cardiovascular Prev & Rehab 2011; 18: 197-208 Screening of athletes / participants

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Genetic testing Accurate screening test Can trace others within the family Can make diagnosis after death BUT expensive Bai et al. Circ Arrhythmia Electrophysiol 2009; 2: 6-15 Benefits

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Genetic testing Bai et al. Circ Arrhythmia Electrophysiol 2009; 2: 6-15 $ 45,000 if cases of SUD $ 71,500 if screening family of SCD Winlel et al. J Cardiovasc Electrophysiol 2012; 23: 1092-1098

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Implications for lifeguards Ensure that lifeguards are aware of the conditions Consider provision of AEDs at swimming pools Refer funny turns for a medical opinion

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Remember that these conditions are hard or impossible to diagnose without previous event or screening Implications for lifeguards

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Support a lifeguard involved in such an incident Implications for lifeguards