Nationwide study of sudden cardiac death in persons aged 1–35 years

Winkel, Bo Gregers; Holst, Anders G.; Theilade, Juliane; Kristensen, Ingrid Bayer; Thomsen, Jørgen Lange; Ottesen, Gyda Lolk; Bundgaard, Henning; Svendsen, Jesper Hastrup; Haunsø, Stig; Tfelt-Hansen, Jacob

doi:10.1093/eurheartj/ehq428

Cited by 340 publications

(306 citation statements)

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“…A nationwide retrospective study of sudden cardiac death in an unselected population of persons 1 to 35 years of age in Denmark showed an incidence of 2.8 per 100,000 person-years, or 1.9 per 100,000 person-years when only autopsied cases were considered. 8 A similar incidence of 1.8 per 100,000 per year was found after a review of death certificates in England and Wales. 9 In up to one third of cases of sudden cardiac death among children and young adults, a cause of death is not found after a comprehensive autopsy examination that includes toxicologic and histologic studies; these deaths are termed unexplained sudden cardiac deaths.…”

supporting

confidence: 57%

“…9 In up to one third of cases of sudden cardiac death among children and young adults, a cause of death is not found after a comprehensive autopsy examination that includes toxicologic and histologic studies; these deaths are termed unexplained sudden cardiac deaths. 3,8,[10][11][12] Unexplained sudden cardiac death is often attributed to cardiac arrhythmia caused by cardiac ionchannel dysfunction, which is undetectable in a conventional autopsy. Noncardiac conditions may also cause sudden death that is clinically indistinguishable from sudden cardiac death.…”

mentioning

confidence: 99%

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A Prospective Study of Sudden Cardiac Death among Children and Young Adults

et al. 2016

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supporting

confidence: 57%

mentioning

confidence: 99%

A Prospective Study of Sudden Cardiac Death among Children and Young Adults

et al. 2016

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“…In an analysis of the ECGs from the same cohort we found a lower prevalence of the Brugada sign than that reported from the majority of other studies [24]. Another recent study from Denmark also reported a lower number of HCM and ARVC than that reported in other studies in persons who had suffered SCD before the age of 35 [18].…”

Section: Open Accesssupporting

confidence: 52%

“…This is not surprising as even in studies, in which the age limit has been 35 -40 years, coronary heart disease has been one of the most prevalent etiologies of SCD. [2][3][4][5]18] The higher age limit in our study was dictated by the low numbers of SCD in the younger participants. In our cohort, in the age groups younger than 40 we only found two persons with SCD.…”

Section: Discussion 3 Resultsmentioning

confidence: 99%

ECG is not a reliable predictor of sudden cardiac death in the general population

Theilade¹,

Pecini²,

Marott³

et al. 2013

WJCD

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Objectives: To determine the predictive value of the ECG for sudden death in the general population. Design: In the Copenhagen City Heart Study, a randomly selected population sample in Copenhagen, Denmark has been followed prospectively since 1976. From this population sample, we analyzed ECGs of individuals who had suffered sudden cardiac death (SCD) before the age of 50 years and compared them with ECGs of a randomly selected control individuals from the same population sample. Specific ECG signs that could point toward a condition associated with a risk of SCD were noted. Results: From a total of 18,974 individuals in the cohort, 207 had died at an age younger than 50 years. Among these, 24 persons with SCD were identified. The most prevalent ECG abnormality was QRS fragmentation. We found no ECGs with long or short QTc, Brugada sign or WPW. The prevalence of signs of left ventricular hypertrophy, early repolarization, or fragmentation was not different from the prevalence of these signs in the control group. Conclusion: In the Copenhagen City Heart Study, the ECG failed to predict SCD in persons who died before the age of 50 years.

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“…The prevalence of SCD is 50,000-100,000 pa in the UK, of which up to 4% may be 'unexplained' (1). The finding of a morphologically normal heart on autopsy is common in most series of young SCD (2)(3)(4)(5)(6)(7)(8). Unexplained sudden death of an individual older than 1 year of age with negative pathological and toxicological assessment on autopsy is referred to as sudden arrhythmic death syndrome (SADS) (9).…”

Section: Introductionmentioning

confidence: 99%

Sudden death and cardiac arrest without phenotype: the utility of genetic testing

Wijeyeratne

Behr

2017

Trends in Cardiovascular Medicine

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Approximately 4% of sudden cardiac deaths are unexplained (the sudden arrhythmic death syndrome: SADS), and up to 6-10% of survivors of cardiac arrest do not have an identifiable cardiac abnormality after comprehensive clinical evaluation (idiopathic ventricular fibrillation: IVF). Genetic testing may be able to play a role in diagnostics and can be targeted to an underlying phenotype present in family members following clinical evaluation. Alternatively post-mortem genetic testing (the 'molecular autopsy') may diagnose the underlying cause if a clearly pathogenic rare variant is found. Limitations include a modest yield, and the high probability of finding a variant of unknown significance (VUS) leading to a low signal-to-noise ratio. Next generation sequencing enables costefficient high throughput screening of a larger number of genes but at the expense of increased genetic noise. The yield from genetic testing is even lower in IVF in the absence of any suggestion of another phenotype in the index case or his/her family, and should be actively discouraged at this time. Future improvements in diagnostic utility include optimisation of the use of variant-calling pipelines and shared databases as well as patient-specific models of disease to more accurately assign pathogenicity of variants. Studying 'trios' of parents and the index case may better assess the yield of sporadic and recessive disease.

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Nationwide study of sudden cardiac death in persons aged 1–35 years

Abstract: A total of 7% of all deaths in the young can be attributed to SCD, when including non-autopsied cases (autopsy ratio 75%). The incidence rate of SCD in the young of 2.8 per 100 000 person-years is higher than previously reported.

Cited by 340 publications

References 25 publications

A Prospective Study of Sudden Cardiac Death among Children and Young Adults

A Prospective Study of Sudden Cardiac Death among Children and Young Adults

ECG is not a reliable predictor of sudden cardiac death in the general population

Sudden death and cardiac arrest without phenotype: the utility of genetic testing

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