Home LiteratureArticle Details
PMID: 17237131 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't

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

Heart (British Cardiac Society) ·Vol. 93 ·No. 5 ·2007-05-00 ·Pages 601-5

Behr ER, Casey A, Sheppard M, Wright M, Bowker TJ, Davies MJ, McKenna WJ, Wood DA

Abstract

To describe the characteristics of sudden arrhythmic death syndrome (SADS) and compare its incidence with official national mortality statistics for unascertained deaths. 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. 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. 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. 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.

MeSH Terms
Adolescent Adult Arrhythmias, Cardiac/complications,mortality Cause of Death Child Child, Preschool Death, Sudden, Cardiac/epidemiology England/epidemiology Female Humans Male Middle Aged Prospective Studies Socioeconomic Factors
Authors & Affiliations
8 authors, click to expand affiliations / ORCID
Behr E R
Cardiological Sciences, St George's University of London, London, UK.
Casey A
Sheppard M
Wright M
Bowker T J
Davies M J
McKenna W J
Wood D A
References (33)
33 references, click to expand
  1. Sudden unexpected nontraumatic death in 54 young adults: a 30-year population-based study.
    Am J Cardiol. 1995 Jul 15;76(3):148-52 PMID: 7611149
  2. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study.
    J Am Coll Cardiol. 1997 Nov 15;30(6):1512-20 PMID: 9362410
  3. Genetic basis and molecular mechanism for idiopathic ventricular fibrillation.
    Nature. 1998 Mar 19;392(6673):293-6 PMID: 9521325
  4. Cardiovascular causes of sudden death in young individuals including athletes.
    Cardiol Rev. 1999 May-Jun;7(3):127-35 PMID: 10423663
  5. Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death.
    Am Heart J. 1957 Jul;54(1):59-68 PMID: 13435203
  6. A NEW FAMILIAL CARDIAC SYNDROME IN CHILDREN.
    J Ir Med Assoc. 1964 Apr;54:103-6 PMID: 14136838
  7. [RARE CARDIAC ARRYTHMIAS OF THE PEDIATRIC AGE. II. SYNCOPAL ATTACKS DUE TO PAROXYSMAL VENTRICULAR FIBRILLATION. (PRESENTATION OF 1ST CASE IN ITALIAN PEDIATRIC LITERATURE)].
    Clin Pediatr (Bologna). 1963 Sep;45:656-83 PMID: 14158288
  8. Targeted mutational analysis of the RyR2-encoded cardiac ryanodine receptor in sudden unexplained death: a molecular autopsy of 49 medical examiner/coroner's cases.
    Mayo Clin Proc. 2004 Nov;79(11):1380-4 PMID: 15544015
  9. Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives.
    Circulation. 2005 Jul 12;112(2):207-13 PMID: 15998675
  10. Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2.
    Circulation. 2000 Sep 5;102(10):1178-85 PMID: 10973849
  11. Clinical and genetic heterogeneity of right bundle branch block and ST-segment elevation syndrome: A prospective evaluation of 52 families.
    Circulation. 2000 Nov 14;102(20):2509-15 PMID: 11076825
  12. Mutations of the cardiac ryanodine receptor (RyR2) gene in familial polymorphic ventricular tachycardia.
    Circulation. 2001 Jan 30;103(4):485-90 PMID: 11157710
  13. Mutations in Kir2.1 cause the developmental and episodic electrical phenotypes of Andersen's syndrome.
    Cell. 2001 May 18;105(4):511-9 PMID: 11371347
  14. A missense mutation in a highly conserved region of CASQ2 is associated with autosomal recessive catecholamine-induced polymorphic ventricular tachycardia in Bedouin families from Israel.
    Am J Hum Genet. 2001 Dec;69(6):1378-84 PMID: 11704930
  15. Hypertrophic cardiomyopathy: management, risk stratification, and prevention of sudden death.
    Heart. 2002 Feb;87(2):169-76 PMID: 11796562
  16. Ankyrin-B mutation causes type 4 long-QT cardiac arrhythmia and sudden cardiac death.
    Nature. 2003 Feb 6;421(6923):634-9 PMID: 12571597
  17. Sudden, unexpected cardiac or unexplained death in England: a national survey.
    QJM. 2003 Apr;96(4):269-79 PMID: 12651971
  18. Short QT Syndrome: a familial cause of sudden death.
    Circulation. 2003 Aug 26;108(8):965-70 PMID: 12925462
  19. Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome.
    Lancet. 2003 Nov 1;362(9394):1457-9 PMID: 14602442
  20. Sudden death associated with short-QT syndrome linked to mutations in HERG.
    Circulation. 2004 Jan 6;109(1):30-5 PMID: 14676148
  21. Postmortem molecular screening in unexplained sudden death.
    J Am Coll Cardiol. 2004 May 5;43(9):1625-9 PMID: 15120823
  22. A cardiac arrhythmia syndrome caused by loss of ankyrin-B function.
    Proc Natl Acad Sci U S A. 2004 Jun 15;101(24):9137-42 PMID: 15178757
  23. Mutations in the cardiac ryanodine receptor gene (hRyR2) underlie catecholaminergic polymorphic ventricular tachycardia.
    Circulation. 2001 Jan 16;103(2):196-200 PMID: 11208676
  24. Ca(V)1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism.
    Cell. 2004 Oct 1;119(1):19-31 PMID: 15454078
  25. Sudden death in young athletes.
    Circulation. 1980 Aug;62(2):218-29 PMID: 6446987
  26. Hypertrophic cardiomyopathy: an important cause of sudden death.
    Arch Dis Child. 1984 Oct;59(10):971-5 PMID: 6541894
  27. Cardiac involvement in myotonic muscular dystrophy.
    Medicine (Baltimore). 1985 Nov;64(6):371-87 PMID: 4058303
  28. Community study of the causes of "natural" sudden death.
    BMJ. 1988 Dec 3;297(6661):1453-6 PMID: 3147014
  29. Sudden unexpected death in persons less than 40 years of age.
    Am J Cardiol. 1991 Nov 15;68(13):1388-92 PMID: 1951130
  30. Unexplained death in fit young people.
    BMJ. 1992 Sep 5;305(6853):538-9 PMID: 1294088
  31. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report.
    J Am Coll Cardiol. 1992 Nov 15;20(6):1391-6 PMID: 1309182
  32. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.
    Br Heart J. 1994 Mar;71(3):215-8 PMID: 8142187
  33. A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome.
    Cell. 1995 Mar 10;80(5):795-803 PMID: 7889573
Article Info
Journal
Heart (British Cardiac Society)
Abbr.
Heart
ISSN
1468-201X
Published
2007-05-00
Epub
2007-00-19
Pages
601-5
Language
English
Region
England
NLM ID
9602087
PMCID
PMC1955564
Subset
IM
Corrections
CommentIn
Analysis Services
Analysis Services

Contact

No. 2 Wenbo Road, Zhangqiu District, Jinan, Shandong

Qilu Normal University · Genelibs Bioinformatics Lab

750 Shunhua Rd, Jinan

2F, Bldg F, University Science Park

Tel: 0531-88819269

WeChat Official Account

Follow our WeChat subscription account for real-time updates and the latest in medical and biological research.


Business Email

E-mail: [email protected]