SUDDEN CARDIAC DEATH: WHO? WHEN? WHY? Michael Christiansen, M.D., FRCPath Statens Serum Institut, University of Copenhagen Copenhagen, Denmark [email protected]

WHAT? Sudden Death is non-traumatic, unexpected fatal event occurring within one hour of the onset of symptoms in an apparently healthy subject. If death is not witnessed the definition applies when the victim was in good health 24 hours before the event. Sudden Cardiac Death (SCD) is used when: 1.  A congenital acquired, potentially fatal cardiac condition was known to be present during life; OR 2.  Autopsy has identified a cardiac or vascular anomaly as the probable cause of the event; OR 3. No obvious extra-cardiac causes have been identified by post-mortem examination and therefore an arrhythmic event is a likely cause of death. 2015 ESC Guidelines, Eur Heart J, doi: 10.1093/eurheartj/ehv316

MAJOR CAUSES OF DEATH

Global Atlas on cardiovascular disease prevention and control; WHO 2011.

CAUSES OF CARDIOVASCULAR DEATHS

MEN

WOMEN

Global Atlas on cardiovascular disease prevention and control; WHO 2011.

FAMILY WITH SCD LQTS2, KCNH2: p.G572R

Larsen LA, Svendsen IH, Jensen AM, Kanters JK, Andersen PS, Møller M, Sørensen SA, Sandøe E, Jacobsen JR, Vuust J, Christiansen M. Clin Genet 57: 125-130, 2000.

BURDEN AND RISK OF SUDDEN DEATH

HOW MANY?

SCD: In the western world: 50-100 per 100.000 per year. SCD: 300.000 – 400.000 per year in the US SCD: 70.000 in 2010 in the UK Normal autopsy and heart pathology: Sudden arrhythmia death syndrome (SADS): 1.38 per 100.000 per year in the UK. SCD in the young (