1. Sudden death in young athletes Rare event - actual rate uncertain Rare event - actual rate uncertain USA - retrospective analysis in High School and College athletes – less than 1 : 100 000 participants per year USA - retrospective analysis in High School and College athletes – less than 1 : 100 000 participants per year Italy – incidence 3 : 100 000 athletes less than 35 years Italy – incidence 3 : 100 000 athletes less than 35 years Male/female ratio - 2-10 : 1 Male/female ratio - 2-10 : 1 Men-higher prevalence of cardiac disease at the age12 - 35 years (probably phenotypic expression) Men-higher prevalence of cardiac disease at the age12 - 35 years (probably phenotypic expression) Men-higher participation rate in competitive sports (football, basketball, ice-hockey) Men-higher participation rate in competitive sports (football, basketball, ice-hockey)

2. Causes of sudden death in athletes less than 35 years Nonpenetrating strike to the chest wall Commotio cordis (trigger of abrupt ventricular fibrillation in interval 15-30 ms before T-wave peak on ECG) Commotio cordis (trigger of abrupt ventricular fibrillation in interval 15-30 ms before T-wave peak on ECG) Competitive sports increase likelihood of cardiac arrest Competitive sports increase likelihood of cardiac arrest Most fatalities-in basketball, football, ice-hockey (USA), soccer, ice hockey (Europe) Most fatalities-in basketball, football, ice-hockey (USA), soccer, ice hockey (Europe) Italy - young adults involved in sports - 25-fold greater risk of sudden death than their non-athletic counterparts Italy - young adults involved in sports - 25-fold greater risk of sudden death than their non-athletic counterparts

3. Causes of sudden death in athletes less than 35 years Hypertrophic cardiomyopathy - more than 1/3 of cases Hypertrophic cardiomyopathy - more than 1/3 of cases Cardiac congenital anomalies Cardiac congenital anomalies arrhytmogenic right ventricle cardiomyopathy arrhytmogenic right ventricle cardiomyopathy premature atherosclerotic coronary artery disease premature atherosclerotic coronary artery disease dilated cardiomyopathy dilated cardiomyopathy mitral valve prolaps mitral valve prolaps conduction system diseases - WPW-syndrome, prolonged QT - interval, Brugada syndrome, Lenégre disease, A-V block, sudden unexplained nocturnal death syndrome (SUNDS) conduction system diseases - WPW-syndrome, prolonged QT - interval, Brugada syndrome, Lenégre disease, A-V block, sudden unexplained nocturnal death syndrome (SUNDS) aortic rupture (bicuspid aortic valve, Marfan syndrome) aortic rupture (bicuspid aortic valve, Marfan syndrome)

4. Causes of sudden death in athletes less than 35 years Non-cardiac Non-cardiac exercise induced bronchoconstriction-BHR exercise induced bronchoconstriction-BHR cerebral aneurysma rupture cerebral aneurysma rupture use of performance - enhancing drugs (deleterious health problems-heart attacks) use of performance - enhancing drugs (deleterious health problems-heart attacks) vigorous exercise (sudden death due to to undiagnosed heart disease) vigorous exercise (sudden death due to to undiagnosed heart disease) London marathon runners – 650 000 completed runs - 5 deaths, 6 resuscitations – 1 death per 80 000 runners London marathon runners – 650 000 completed runs - 5 deaths, 6 resuscitations – 1 death per 80 000 runners

5. Extreme sport activities decrease of heart function and its fatigue decrease of heart function and its fatigue Ashley - echocardiographic evaluation before and after a 480 km competition (running, swimming, bicycling) - lasting about 90 hours - 13 % decrease of cardiac contractility Ashley - echocardiographic evaluation before and after a 480 km competition (running, swimming, bicycling) - lasting about 90 hours - 13 % decrease of cardiac contractility

6. Assessment of the risk for sudden death in young poeple in competitive sports AHA-1996, ESC-2005 Consensus Guidelanes Family history: of sudden death, heart disease before 50 years, cardiomyopathy, arrhythmias, Marfan syndrome Family history: of sudden death, heart disease before 50 years, cardiomyopathy, arrhythmias, Marfan syndrome Athletes medical history: chest pain, palpitations, syncope, tachycardia, dyspnoea, fatigue, heart murmur, hypertension Athletes medical history: chest pain, palpitations, syncope, tachycardia, dyspnoea, fatigue, heart murmur, hypertension Physical examination: heart murmurs, arrhythmias, femoral artery puls (coarctation of aorta), Marfan syndrome Physical examination: heart murmurs, arrhythmias, femoral artery puls (coarctation of aorta), Marfan syndrome

7. Italy - addition of 12-lead ECG-identification of up to 60% of the potential lethal condition of sudden death Italy - addition of 12-lead ECG-identification of up to 60% of the potential lethal condition of sudden death All investigations - marginal use in detecting heart disorders and preventing fatalities All investigations - marginal use in detecting heart disorders and preventing fatalities Echocardiography + tissue Doppler echocardiography – differentiation pathologic from adaptational changes of the heart muscle Echocardiography + tissue Doppler echocardiography – differentiation pathologic from adaptational changes of the heart muscle In 2 - 5% of sudden cardiac deaths cases - no definitive cause even after autopsy detected In 2 - 5% of sudden cardiac deaths cases - no definitive cause even after autopsy detected

8. Guidelines for management of athletes with identified cardiovascular disease are available withdrawal of competitive sport activities - reduced risk of sudden death withdrawal of competitive sport activities - reduced risk of sudden death suggestion to the type and degree of sport activity that can be done safely suggestion to the type and degree of sport activity that can be done safely

9. Examples of sudden death in young athletes Pheidipphides (490 BC) A greek soldier and conditioned runner, Pheidippides ran from Marathon to Athens to announce military victory over Persia. He delivered his message, then collapsed and died. A greek soldier and conditioned runner, Pheidippides ran from Marathon to Athens to announce military victory over Persia. He delivered his message, then collapsed and died. Flo Hyman (1986) Flo Hyman was captain of the 1984 US Women´s Olympic volleyball team that won silver. She died aged 30 years of congenital heart disorder Marfan´s syndrome while playing a Japanese league game. Flo Hyman was captain of the 1984 US Women´s Olympic volleyball team that won silver. She died aged 30 years of congenital heart disorder Marfan´s syndrome while playing a Japanese league game. Daniel Yorath (1992) A 15-year-old football player who had just been signed by the UK team Leeds United, Daniel Yorath died from hypertrophic cardiomyopathy while playing football with his father in the garden. A 15-year-old football player who had just been signed by the UK team Leeds United, Daniel Yorath died from hypertrophic cardiomyopathy while playing football with his father in the garden.

10. Examples of sudden death in young athletes Reggie Lewis (1993) Boston Celtic´s star and sixth captain Reggie Lewis was 27 year old when he died while shooting baskets at an off-seasons practice. Boston Celtic´s star and sixth captain Reggie Lewis was 27 year old when he died while shooting baskets at an off-seasons practice. Sergei Grinkov (1995) An Olympic gold medal skater, Sergei Grinkov collapsed and died from a heart attack at age 28 years while training at an ice rink in Lake Placid, NY, USA. An autopsy showed that he had arteries of a 70-year-old man, though had never mentioned any chest pain. An Olympic gold medal skater, Sergei Grinkov collapsed and died from a heart attack at age 28 years while training at an ice rink in Lake Placid, NY, USA. An autopsy showed that he had arteries of a 70-year-old man, though had never mentioned any chest pain.

11. Case report History 16 years old athlete-attacks of tachycardia (220/min) during long distance running - faintness, disappearing immediately after stopping exercise 16 years old athlete-attacks of tachycardia (220/min) during long distance running - faintness, disappearing immediately after stopping exercise Physical examination Physical examination Signs of anemia on oral mucosa, lips, conjunctives Signs of anemia on oral mucosa, lips, conjunctives Systolic murmur 3/6 in praecordium, irradiating into carotides Systolic murmur 3/6 in praecordium, irradiating into carotides Ergometry: Rest Exercise Ergometry: Rest Exercise HR 97/min 240/min BP 145/110 mmHg 200/110 mmHg

12. Klidové EKG – tachykardie, jinak normální nález

13. Při výkonu 90 W se začaly objevovat ojedinělé SVES.

14. Jejich počet se v první minutě výkonu 150 W zvětšil.

15. Ve 2. minutě výkonu 150 W vznikl náhle záchvat SV tachykardie (s frekvencí 240 tepů/min) a vyšetřovaný začal pociťovat mdlobu.

16. V době uklidňování SV tachykardie opět náhle vymizela.

17. Ve 3. minutě uklidňování již vidíme sinusový rytmus s výraznou dechovou arytmií.

18. Laboratory results HB-79 g/l; Htc – 0.25; Ery - 4,01; FW - 25/34 Laboratory results HB-79 g/l; Htc – 0.25; Ery - 4,01; FW - 25/34 Cardiologic evaluation Echocardiography - hypertrophy and dilatation of left ventricle, a mild decrease of ejection fraction of left ventricle, interventricular septum thickeness at high normal limit (1.1 cm) Right and left heart catheterisation - normal pressure, normal flow values, no gradient even after Isuprel application Cardiologic evaluation Echocardiography - hypertrophy and dilatation of left ventricle, a mild decrease of ejection fraction of left ventricle, interventricular septum thickeness at high normal limit (1.1 cm) Right and left heart catheterisation - normal pressure, normal flow values, no gradient even after Isuprel application Treatment Ferronat p.os, no exercise until next control examination will be done Treatment Ferronat p.os, no exercise until next control examination will be done

19. Follow up 3 weeks after medical examination while playing football sudden syncope, resuscitation, defibrillation, sudden death. Follow up 3 weeks after medical examination while playing football sudden syncope, resuscitation, defibrillation, sudden death. Autopsy finding - Hypertrophic cardiomyopathy - interventricular septum thickeness -18 mm, Autopsy finding - Hypertrophic cardiomyopathy - interventricular septum thickeness -18 mm, A thickenning of posterior left ventricle wall – 3 x 3 cm. A thickenning of posterior left ventricle wall – 3 x 3 cm.

20. Výsledek pitvy: mezikomorové septum 18 mm, na zadní stěně LK je ztluštění 3x3 cm, histologický nález odpovídá hypertrofické kardiomyopatii.