How Often Do Inherited Cardiomyopathies Explain Sudden Cardiac Death in Young People?

Sudden cardiac death (SCD) in the young, though tragic, is rare because young people have a low prevalence of acquired cardiomyopathies (e.g., ischemic and nonischemic dilated cardiomyopathies). Registry studies of young people document an array of SCD etiologies, perhaps related to genetic variations among populations or to incomplete ascertainment of possible causes. A new prospective registry has done a more precise accounting, by tracking all SCDs in patients aged 1 to 35 years in Australia and New Zealand.

Annual SCD incidence was similar to that among young patients in other registries: 1.3 cases (1.8 in males vs. 0.7 in females) per 100,000 people. Most SCDs occurred during sleep or at rest. After a predefined and thorough autopsy, co…