Sudden arrhythmic death syndrome: a national survey of sudden unexplained cardiac death

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 h of death, normal coroner's autopsy, cardiac pathologist's confirmation of a normal heart and negative toxicology. Main outcome measures: The estimated mortality from SADS was calculated and the official mortality statistics for unascertained causes of deaths in 4-64-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 years, range 7-64 years and 35 (63%) male. 7 of 39 cases (18%) had a family history of other premature sudden deaths (,45). The estimated mortality from SADS was 0.16/100 000 per annum (95% CI 0.12 to 0.21), compared with an official mortality of 0.10/100 000 per annum for International Classification of Diseases 798.1 (sudden death, cause unknown-instantaneous death) or 1.34/100 000 per annum for unascertained causes of death. Conclusions: Deaths from SADS occur predominantly in young males. When compared with official mortality, the incidence of SADS may be up to eight times higher than estimated: more than 500 potential SADS cases per annum in England. Families with SADS 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.

Cardiac Evaluation of Pediatric Relatives in Sudden Arrhythmic Death Syndrome

“…1 It accounts for ≈500 deaths in the United Kingdom every year, corresponding to an annual incidence of 1.38/100 000 population. 2 International estimates vary partly because of different populations and inclusion criteria. The incidence of SADS in other white populations ranges from 0.81/100 000 (Danish) to 1.2/100 000 (United States).…”

Genetic aspects and investigation of sudden death in young people

“…In the UK, the NSF for Coronary Disease has acknowledged that effective evaluation of relatives, guided by genetic testing, can prevent further deaths in the family 1 Although genetic testing forms a part of this process, its role varies in different conditions. Importantly, genetic results must be carefully interpreted in the context of the clinical phenotype.…”

2016 AHA/ACC Clinical Performance and Quality Measures for Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Performance Measures

“…[54][55][56][57][58] For instance, compared with 10.6% of referents, 18.6% of individuals in the Paris Prospective Study who experienced SCD in follow-up reported a parental history of SCD at baseline. The adjusted relative risk of a parental history of SCD was 1.80 (95% confidence interval: 1.11-2.88; P=0.01).…”