1. Sudden Cardiac Death (SCD)

2. SCD is natural death from cardiac causes, heralded by abrupt loss of consciousness within 1 hour of the onset of an acute change in cardiovascular status. Preexisting heart disease may or may not have been known to be present but the time and mode of death are unexpected. Prodromes, occurring weeks or months before an event, are not sensitive or specific predictors of an impending event. Definition

3. Sudden cardiac death Sudden, irreversible cessation of all biologic functions Cardiac arrest Abrupt cessation of cardiac mechanical function, which may be reversible by a prompt intervention but will lead to death in its absence Cardiovascular collapse Sudden loss of effective blood flow due to cardiac and/or peripheral vascular factors that may reverse spontaneously (e.g. vasovagal syncope) or require interventions (e.g., cardiac arrest)

4. Definition

5. SCD accounts for 5-15% of the total mortality in the US. In absolute terms, the estimated number of SCD in the US is 350,000/yr Epidemiology

6. RISK FACTORS Cigarette smoking Like most of risk factors for CHD Exercise Transiently increased during and up to 30 minutes after. Family history of SCD 1.5 to 1.8-fold increased risk of SCD Serum CRP Chronic inflammation Excess alcohol intake Heavy alcohol consumption (6 or more drinks per day) or binge drinking Psychosocial factors Acutely stressful situations (earthquakes, war) Caffeine No significant association

7. Major causes of SCD 70%10%

8. Major causes of sudden death

9. מקרה 1 בת 30 פנתה לרופא המשפחה בשל חום (38.7) ושיעול ליחתי.

10. מקרה 1 יומיים לאחר התחלת טיפול אנטיביוטי התמוטטה בביתה. Polymorphic VT Torsade de pointes (TdP)

11. מקרה 1

13. Acquired LQTS Electrolyte imbalance Hypokalemia, Hypocalcaemia, Hypomagnesemia Metabolic disturbance Hypothermia, Starvation Diets, Exposure to Organophosphates Drug Therapy Antiarrhythmics, Cisapride, Fluconazole, Phenothiazines, Tricyclic Antidepressants, CNC stimulants: Concerta/Ritaline (methylphenidate), Strattera (Atomoxetine)

14. Inherited LQTS

16. מקרה 2 בן 30 – עבר החייאה לאחר שהתמוטט בביתו.

17. An autosomal dominant genetic disorder. Characterized by abnormal findings on ECG in conjunction with an increased risk of ventricular tachyarrhythmias and SCD. Genetic analysis has led to the identification of causative mutations in the SCN5A gene, encoding subunits of a cardiac sodium channel. Sudden cardiac arrest may be the initial presentation (30%) Arrhythmic events generally occur between the ages 22 - 65 More common at night than in the day and during sleep than while awake. Brugada Syndrome

18. Brugada pattern Vs. Brugada syndrome – A 10% event rate at 2.5 years Typical ECG plus one of the following: – Documented ventricular fibrillation (VF) – Polymorphic ventricular tachycardia (VT) – Family history of SCD at less than 45 years of age – Inducible VT during electrophysiology study – Unexplained syncope suggestive of a tachyarrhythmia Brugada Syndrome

19. מקרה 3 בן 60 התלונן על כאבים בחזה והתמוטט.

20. מקרה 3 עבר החייאה מוצלחת ( כ 10 דקות ) והובא למיון.

21. מקרה 4 בן 70 – התלונן על חולשה ולאחר כשעה התמוטט. עבר החייאה והובא למיון.

22. מקרה 4 בן 70 – התלונן על חולשה ולאחר כשעה התמוטט. עבר החייאה והובא למיון.

23. מקרה 4 בן 70 – התלונן על חולשה ולאחר כשעה התמוטט. עבר החייאה והובא למיון.

24. Conduction system

25. מקרה 5 בן 18 התמוטט במהלך משחק כדורגל. עבר החייאה והובא למיון.

26. מקרה 5 NormalHCM

27. מקרה 5

28. Hypertrophic cardiomyopathy (HCM) is a genetically determined (AD) heart muscle disease. Most often (60-70%) caused by mutations in one of several sarcomere genes. Symptoms (particularly during or immediately following exertion) – Dyspnea – Fatigue – Chest pain – Presyncope and syncope – Palpitation – SCD HCM

29. מקרה 6 בן 25 התמוטט במהלך ריצה. עבר החייאה והובא למיון. epsilon wave

30. Arrythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease, typically inherited as an autosomal dominant trait. SCD can be the first presentation – 10% of SCD – 75% during routine daily activities – 10% during the perioperative period – 3.5% while participating in sports ARVC

31. מקרה 7 בת 68 התמוטטה במהלך ריצה. עברה החייאה והובאה למיון. בבדיקה – א " ס, גסה, בנקודה אאורטלית קורנת לקרוטידים. קול שני לא נשמע.

32. Identification and treatment of acute reversible causes (cath, electrolytes etc.) Evaluation for structural heart disease In patients without obvious arrhythmic triggers or cardiac structural abnormalities, an evaluation for primary electrical diseases Neurologic and psychologic assessment In selected patients with a suspected or confirmed heritable syndrome, evaluation of family members Management

33. Secondary prevention – An implantable cardioverter-defibrillator (ICD) therapy Management