Sudden Arrhythmic Death Syndrome (SADS) - a national survey of sudden unexplained cardiac death

Elijah R Behr ¹, Mary Sheppard ², Melissa Wright ², Timothy J Bowker ², Michael J Davies ¹, William J McKenna ³ and David A Wood ^2*

¹ Cardiological Sciences, St George's University of London, United Kingdom
² Clinical Epidemiology and Pathology, National Heart & Lung Institute, United Kingdom
³ The Heart Hospital, University College Hospital, London, United Kingdom

^* To whom correspondence should be addressed. E-mail: d.wood{at}imperial.ac.uk.

Accepted 19 September 2006

Abstract

Objective To describe the characteristics of 'Sudden Arrhythmic Death Syndrome' (SADS) and compare its incidence with official national mortality statistics for unascertained deaths.

Design and Setting Sudden unexplained deaths were prospectively surveyed through 117 Coroners' jurisdictions in England. Consecutive cases meeting the following criteria were included: white Caucasian, aged 4-64 years, no history of cardiac disease, last seen alive within 12 hours of death, normal coroner's autopsy, cardiac pathologist's confirmation of a normal heart and negative toxicology.

Main Outcome Measures The estimated SADS mortality was calculated and the official mortality statistics for unascertained causes of deaths in 4 to 64 year olds was identified for the same time period.

Results 115 coroner's cases were reported and 56 (49 %) SADS victims were identified: mean age 32, range 7-64 years and 35 (63 %) were male. Seven out of 39 cases (18 %) had a family history of other premature sudden deaths (<45). The estimated SADS mortality rate was 0.16/100,000 p.a. (95 % CI = 0.12-0.21), compared to an official mortality rate of 0.10/100,000 p.a. for ICD 798.1 (Sudden death, cause unknown - instantaneous death) or 1.34/100,000 p.a. for 'unascertained causes of death'.

Conclusions SADS deaths occur predominantly in young males. When compared to official mortality rates the SADS incidence may be up to eight times higher than estimated: over 500 potential SADS cases p.a. in England. SADS families carry genetic cardiac disease placing them at risk of further sudden deaths. SADS should therefore be a certifiable cause of death prompting specialised cardiological evaluation of families.

Keywords: SADS, epidemiology, long QT syndrome, sudden arrhythmic death syndrome SADS, sudden unexplained death

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