Key result
Sudden arrhythmic death syndrome in children and young adults without structural heart disease is a complex condition potentially triggered by miscommunication between the heart and central nervous system.
Why the study?
The causes of over half of sudden arrhythmic death syndrome cases remain unexplained despite postmortem genetic testing, and the contribution of abnormal central nervous system function to these unexpected lethal events warrants exploration.
This review highlights the complex pathogenesis of sudden arrhythmic death syndrome in the young, suggesting a critical role of central nervous system dysfunction in addition to primary cardiac disorders.
Pediatric SCD risk assessment remains incomplete; leaves open need for prospective genetic and CNS-focused studies before refined prevention strategies.
Sudden cardiac death (SCD) is a rare clinical encounter in pediatrics, but its social impact is immense because of its unpredicted and catastrophic nature in previously healthy individuals. Unlike in adults where the primary cause of SCD is related to ischemic heart disease, the etiology is diverse in young SCD victims. Although certain structural heart diseases may be identified during autopsy in some SCD victims, autopsy-negative SCD is more common in pediatrics, which warrants the diagnosis of sudden arrhythmic death syndrome (SADS) based upon the assumption that the usual heart rhythm is abruptly replaced by lethal ventricular arrhythmia. Despite current advances in molecular genetics, the causes of more than half of SADS cases remain unanswered even after postmortem genetic testing. Moreover, the majority of these deaths occur at rest or during sleep even in the young. Recently, sudden unexpected death in epilepsy (SUDEP) has emerged as another etiology of SCD in children and adults, suggesting critical involvement of the central nervous system (CNS) in SCD. Primary cardiac disorders may not be solely responsible for SCD; abnormal CNS function may also contribute to the unexpected lethal event. In this review article, we provide an overview of the complex pathogenesis of SADS and its diverse clinical presentation in the young and postulate that SADS is, in part, induced by unfortunate miscommunication between the heart and CNS via the autonomic nervous system.
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Tsuda et al. (2020) conducted a review in Sudden cardiac death (SCD) and sudden arrhythmic death syndrome (SADS). Sudden arrhythmic death syndrome in children and young adults without structural heart disease is a complex condition potentially triggered by miscommunication between the heart and central nervous system.
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