In a society where disease prevention is becoming increasingly important, young people should have the opportunity to be tested. Annually, tens of thousands of people in the UK will be personally affected by young sudden deaths and may require reassurance. Proactive testing is usually the only way a person can learn if they are at risk of sudden death. When screening is not provided – or even discouraged (as is the current case) – our society is being condemned to accept that every year a significant group of young people will die of these conditions.
The 2015 UK National Screening Committee (UK NSC) review does little to advocate progress. They state, “The conditions that lead to sudden cardiac death are poorly understood and there is no evidence to guide clinicians regarding treatment or lifestyle advice when such a problem is found in a family member or when detected at a screening examination.” The false premise and misleading conclusions of their report are a cause for great concern; they depict the field of inherited cardiac diseases and sports cardiology as void of any evidence to guide the investigation and management of individuals suspected to have or diagnosed with conditions associated with YSCD.
Their position is in conflict with Chapter Eight of the National Service Framework for Coronary Heart Disease, aimed at facilitating early identification of individuals at risk of YSCD: “Quality Requirement Three” states; “When sudden cardiac death occurs, NHS services have systems in place to identify family members at risk and provide personally tailored, sensitive and expert support, diagnosis, treatment, information and advice to close relatives… Sudden cardiac death in younger people is often indicative of inherited cardiac disease. There is real potential to prevent further tragedies by the appropriate care of family members in these cases.”
Furthermore, the UK NSC is dismissive of the testing process; “There have been no assessments of the accuracy of these tests”. What is happening is that the fact, the ECG is a simple, safe and validated test as required by the World Health Organization (WHO) and UK NSC criteria. Moreover, the ECG has been studied extensively as a screening tool, particularly in the UK, for young individuals of different genders, ethnicities, levels of exercise and sporting disciplines. The position of the UK NSC directly contradicts the National Institute for Health and Care Excellence (NICE) guidelines for transient loss of consciousness, which state that; “A 12 lead ECG is an important initial diagnostic test for identifying the likely cause of transient loss of consciousness in some people, and especially in predicting adverse events (for example, ECG abnormalities that are ‘red flag’ signs or symptoms may suggest structural heart disease or potential for arrhythmic syncope).”
It is important to acknowledge that screening to reduce YSCD is a challenge, looking for more than one condition, some of which are fairly novel and lack evidence-based management. However, it is unacceptable for policy to fall back on the lack of randomised controlled trials (RCTs) as the basis for insufficient evidence. In principle RCTs are the ideal standard in research to inform policy and practice. However, the ideal standard is not always achievable in many aspects of science (and life, in general). It would be technically and ethically challenging to perform a large scale randomised controlled study, particularly in light of the results of the 2006 Corrado et al. study (1) and the many studies relating to the effective management of individuals identified with an inherited cardiac disease.
As part of evaluating any screening programme it is first essential to understand point one of the UK NSC Programme Appraisal Criteria: “The condition should be an important health problem”. For this there must be an accurate appraisal of the incidence of young sudden death. The latest screening report makes no reference to the data published by CRY (Papadakis et al. (2)) that informs CRY’s “12 a week” figure. This is the most important data published to date on understanding the incidence of young sudden cardiac deaths in the UK.
The current UK national policy is based on a gross underestimation of the incidence of YSCD, the tests used to identify people at risk, and the management strategies to reduce risks. Current policy is failing to prevent young sudden cardiac deaths in the UK.