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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 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 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 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 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 Matthew’s drowning Causes of sudden death in the water Diagnosed at autopsy Heart attack or stroke Trauma causing unconsciousness Sudden cardiac death Causes of sudden death in the water No autopsy abnormalities Seizures, including epilepsy Diving response or cold shock Shallow water blackout Sudden cardiac death 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: 742–79 General population 1 : 1000 pa Priori et al. Eur Heart J 2001; 22: 1374–1450 Young people (< 35yrs) 0.3-3.6 (1) : 100,000 pa Borjesson & Pelliccia. Br J Sports Med 2009; 43: 644-648 Non-athletes (< 35yrs) Athletes (< 35yrs) 0.7 : 100,000 pa 2.1 : 100,000 pa 3 x the risk Corrado et al. J Am Coll Cardiol 2003; 42: 1959-1963 Estimated 1.5% – 2.5% SCD occur during swimming 1 : 100,000 young people at risk swimming-related SCD Kenny & Martin. Arch Dis Child 2011; 96: 5-8 SCD during sport HCM 7% Ischaemic heart disease 51% Coronary abnormalities 4% ARVC 8% Myocarditis 4% Unexplained 11% Aortic stenosis 3% Suárez-Mier et al Forensic Sci Int 2013; 226: 188-196 SCD during sport aged < 35 years HCM 18% Ischaemic heart disease 14% Coronary abnormalities 6% ARVC 15% Myocarditis 5% Unexplained 23% Aortic stenosis 4% Suárez-Mier et al Forensic Sci Int 2013; 226: 188-196 Using strict criteria autopsy-negative SCD in younger subjects - 8% Tester & Ackerman Pediatr 2012; 33: 461-470 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 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 Ion channelopathies 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 Catecholaminergic polymorphic ventricular tachycardia (CPVT) Long QT Syndrome (LQTS) Associated Brugada Syndromewith swimming SCD PCCD (progressive cardiac conduction defect) Short QT syndrome (SQTS) Familial atrial fibrillation Sodium channel disease Normal LQTS 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 Pre-participation screening Young Competitive Athletes Family & Personal History Physical Examination ECG Negative Findings Eligible for Competition Positive Findings No cardiac Disease Further Investigations Cardiac Disease Treatment as necessary Corrado et al. Eur Heart J 2005; 26: 516-524 False-positive results Papadakis & Sharma. Br J Sports Med 2009; 43; 663-668 2% Europe € 30 USA $ 75 ‘Cost of preventing each… death would be $ 3.4 million’ Maron et al. Circulation 2007; 115: 1643-1655 Corrado et al. Eur Heart 2005; 26: 516-524 Cost per year per life saved € 14,220 X € 37,750 Corrado et al. Abstract presented at American Heart Association 2004 NICE: National Institute of Clinical Excellence NICE's threshold for cost-effective treatment lies between £20,000 and £30,000 per QALY 1997-2009 Millennium Mathematics Project, University of Cambridge http://www.plus.maths.org/latestnews/jan-apr10/qaly/ Kidney dialysis £ 45,000 Coronary artery bypass graft £ 26,000 Breast cancer screening £ 6,000 Cervical cancer screening £ Pre-participation screening £ 12,350 Hope et al. Medical Ethics and Law 2003. Churchill Livingstone: Chapter 13 200 Screening of athletes / participants ‘The best strategy is to combine … preparticipation 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 Genetic testing Benefits 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 Genetic testing $ 45,000 if cases of SUD $ 71,500 if screening family of SCD Winlel et al. J Cardiovasc Electrophysiol 2012; 23: 1092-1098 Bai et al. Circ Arrhythmia Electrophysiol 2009; 2: 6-15 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 Implications for lifeguards Remember that these conditions are hard or impossible to diagnose without previous event or screening Implications for lifeguards ‘Support’ a lifeguard involved in such an incident …