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Sudden cardiac death in children: case for screening grows stronger

More than half of all victims of sudden cardiac death in children (SCDc) – defined as SCD in those 1–18 years old – experienced antecedent and/or prodromal symptoms prior to death, according to a nationwide study published recently in the European Heart Journal.1

The authors argue that cardiac symptoms in young persons should warrant clinical work-up, with an autopsy performed in all cases of sudden unexpected death to diagnose and treat potential inherited cardiac diseases in family members.

In a nationwide setting all deaths in children aged 1–18 years in Denmark in 2000–06 were examined, with death certificates and autopsy reports collected and read to chart causes of death and incidence rates. By additional use of the extensive healthcare registries in Denmark, the authors were also able to investigate prior disease and symptoms. During the 7-year study period there was an average of 1.11 million persons aged 1–18 years. There were a total of 1,504 deaths (214 deaths per year) from 7.78 million person-years. A total of 114 (7.5%) were sudden and unexpected. A cardiac disease was known prior to death in 18% of all sudden unexpected death cases. In two-thirds of all sudden unexpected death cases no previous medical history was registered. Causes of death in autopsied cases were cardiac or unknown in 70%.

Unexplained deaths, presumed to be a primary cardiac arrhythmia, accounted for 28% of autopsied sudden unexpected death cases. Autopsy rate was 77%. There were a total of 87 cases of SCDc (5.8% of all deaths). Prodromal symptoms were noted in 26% and antecedent symptoms in 45% of SCDc cases. The most frequent antecedent symptoms were seizures, dyspnoea, and syncope. In total, 61% of SCDc were not known with any prior disease; 23% were known with congenital or other heart disease prior to death. In total, 43 (49%) of all sudden unexpected deaths died of a potential inherited cardiac disease. The incidence rate of sudden unexpected death was 1.5 per 100,000 person-years. The highest possible incidence rate of SCDc was 1.1 per 100,000 person-years.

…population screening is feasible

photo (5)

Dr Rajay Narain (St George's University of London)

Despite fears over cost, the wide-scale screening of young people to detect risk of sudden cardiac death (SCD) is feasible and cost effective, according to a study presented recently at EuroPRevent 2014.2 More than 12,000 people aged between 14 and 35 were screened at a cost of £35 (40 euro) each; rates of subsequent referral for further investigation were low and considered of “a relative low additional cost” to health services.

The study was reported by Dr Rajay Narain (St George’s University of London). As background to the study Dr Narain explained that the most publicised cases of SCD in young people occur in elite sports players and athletes. Yet the majority of cases occur as a result of inherited cardiac conditions, a large proportion of which can be detected during life.

Speaking to BJC Arrhythmia Watch, Dr Narain said: “The unexpected deaths of young people playing sport or going about their daily lives have prompted calls from health campaigners for routine tests to check for any heart abnormalities, but concerns about costs have also been raised…heart screening could be cost-effective and a way to save lives.”

“To prevent such tragedies,” said Dr Narain, “sporting and scientific bodies recommend pre-participation screening in young athletes. However, this approach is controversial because of cost – and most SCDs in the young are likely to occur in non-competitive athletes. It was thus our aim to see if population screening was feasible in this age group.”

The study involved the screening of 12,000 young people irrespective of their athletic ability. Only 13% were considered elite athletes. Screening was performed at a cost of £35 per individual and comprised a health questionnaire, 12-lead ECG and consultation with a cardiologist. Those with abnormalities had an echocardiogram on the day or were referred for further evaluation. Follow-up data were obtained through self-reported patient questionnaires.

Results showed that almost one-in-ten of the population  (9.4%) were sent for echocardiography on the day, and 2.7% were referred for further assessment. Of these who responded to the questionnaire and completed their follow-up investigations (189), a cardiac pathology (or findings necessitating regular follow-up) were identified in 31 (16%). The most common were heart block (10), irregular heart rhythm (9) and valvular heart disease (6). Different cardiomyopathies were evident in 11 cases.

Commenting on the results, Dr Narain said that the concept of early identification of potential victims is still a highly controversial issue. “Those opposed question the cost effectiveness of pre-participation screening and the need for multiple investigations to identify so many disease processes implicated in sudden cardiac death,” he said, adding that there are also concerns about the medical and legal implications of false positive and negative results.

However, by applying such a screening programme as the one investigated here, Dr Nairn said that many of the sudden deaths from these conditions, which number around 15 per week in the UK, can be prevented.

“Fortunately,” said Dr Narain, “the incidence of SCD, especially in people doing sports, is low, so several thousand athletes have to be assessed to identify the one who might die suddenly. But despite their rarity, such events are highly visible, particularly when high-profile athletes are involved. The cardiac arrest of the footballer Fabrice Muamba last year is just one example of the societal impact of such tragedies.”

Population screening to prevent SCD in young people is “possible and achievable” said Dr Narain, who added: “Most developed countries have the potential for creating an infrastructure in high schools similar to established immunisation programmes. There is evidence that teachers, coaches and even volunteering parents could be trained in ECGs. The incentive is prudent – 25–30% of the population in the Western world is now aged 18 or under.”

References

1. Winkel BG, Risgaard B, Sadjadieh G, Bundgaard H, Haunsø S, Tfelt-Hansen J. Sudden cardiac death in children (1–18 years): symptoms and causes of death in a nationwide setting. Eur Heart J 2014;35:868–75. http://dx.doi.org/10.1093/eurheartj/eht509

2. Narain R, Dhutia H, Merghani A, et al. Preventing sudden cardiac death in the young: Results form a population-based screening program in the UK. EuroPRevent 2014. For the details of this session, please visit: http://media.ne.cision.com/l/afzngujk/spo.escardio.org/SessionDetails.aspx?eevtid=66&sessId=13564

Published on: May 28, 2014

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