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• W2106549666 abstract "This editorial refers to ‘Family-based cardiac screening in relatives of victims of sudden arrhythmic death syndrome’ by C. McGorrian et al ., on page 1050. Sudden arrhythmic death syndrome (SADS) defines a sudden unexpected death that remains unexplained after comprehensive postmortem examination, histology, and toxicology studies. It accounts for up to 500 deaths in the UK every year, corresponding to an annual incidence of 1.34/100 000 population.1,2 International estimates vary partly due to different populations and inclusion criteria. The incidence of SADS in other Caucasian populations ranges from 0.81/100 000 (Danish) to 1.2/100 000 (USA). South-east Asia demonstrates the highest annual incidence of unexplained sudden death with 38 per 100 000 men.3A significant proportion of SADS cases have been demonstrated to be due to inherited channelopathies and cardiomyopathies. These have been identified through studies on familial cardiological evaluation and/or the mutation analysis of SADS victims' DNA, the ‘molecular autopsy’, and have laid the groundwork for much of our current clinical practice.4–8In this issue of the Journal , McGorrian et al .9 report on their experience at a single tertiary referral centre in the Republic of Ireland of familial evaluation following SADS-death. They studied 262 relatives from 73 families over 4 years from 2007 to 2011 and employed a standard screening protocol similar to that reported by our group in 2008; beginning with a review of clinical and family history followed by first-line investigations consisting a 12-lead electrocardiogram (ECG), 24 h Holter monitor, exercise stress test, and echocardiogram. Further testing with a signal-averaged ECG, cardiac magnetic resonance imaging, and ajmaline provocation were performed if deemed necessary.4They identified an inheritable cardiac condition in 36 relatives (13.7%), leading to diagnoses in 22 families—representing a yield of 30.1% from the family screening. Channelopathies …" @default.
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• W2106549666 date "2013-03-20" @default.
• W2106549666 modified "2023-09-26" @default.
• W2106549666 title "Familial cardiological evaluation in sudden arrhythmic death syndrome: essential but challenging" @default.
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• W2106549666 doi "https://doi.org/10.1093/europace/eut060" @default.
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