Key result
HCM accounts for 35% of SCD in young US athletes despite rising alternative cardiac diagnoses.
Why the study?
The true prevalence and causes of sudden cardiac death in young individuals and athletes, particularly the role of hypertrophic cardiomyopathy, remain uncertain due to varying findings across populations.
Contemporary studies challenge the traditional view that hypertrophic cardiomyopathy is the predominant cause of sudden cardiac death in young athletes, highlighting ARVC and ion-channelopathies as increasingly recognized causes.
May inform SCD risk models in HCM; hypothesis-generating and requires prospective validation before clinical use.
Clinical characteristics and circumstances of sudden cardiac death in hypertrophic cardiomyopathy Sudden cardiac death (SCD) can be the first manifestation of genetic heart disease affecting apparently healthy individuals.1 A diverse spectrum of diseases are implicated and the prevalence of specific aetiologies depends on the demographics of the victims.2–5 The primary cardiomyopathies and the ion channelopathies are the predominant causes of SCD in the young (<35 years).1,6,7 Post-mortem examination is an essential first diagnostic step which steers the clinical evaluation of surviving relatives towards inherited structural diseases or primary arrhythmogenic syndromes.8 Hypertrophic cardiomyopathy (HCM) is a genetically transmitted cardiomyopathy, characterized by unexplained left ventricular (LV) hypertrophy.9 It is one of the leading causes of SCD in young athletic individuals.4 The combination of the pathological substrate characterized by myocardial disarray, fibrosis, and ischaemia with electrolyte abnormalities, acid–base disturbances, and mechanical stressors may trigger fatal arrhythmias during intensive exercise.10,11 Contemporary studies have cast doubt relating to the true prevalence of HCM as a cause of SCD in young athletes. Although HCM was reported in 35% of young athletes who died suddenly in the USA,4,12 recent studies in Italy,13 the UK,14 and college athletes in the USA,15 reported a higher prevalence of other cardiac conditions such as arrhythmogenic right ventricular cardiomyopathy (ARVC) and a normal heart at post-mortem implying sudden arrhythmic death syndrome (SADS) secondary to an ion-channelopathy.
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Finocchiaro et al. (2017) conducted a review in Sudden cardiac death in hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy was reported as the cause of sudden cardiac death in 35% of young athletes in the USA, though recent studies suggest a higher prevalence of other cardiac conditions.
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