Anatomopathological changes of the cardiac conduction system in sudden cardiac death, particularly in infants: advances over the last 25 years.

Abstract

Sudden cardiac death (SCD) is defined as the unexpected death without an obvious noncardiac cause that occurs within 1 h of witnessed symptom onset (established SCD) or within 24 h of unwitnessed symptom onset (probable SCD). In the United States, its incidence is 69/100,000 per year. Dysfunctions of the cardiac conduction and autonomic nervous systems are known to contribute to SCD pathogenesis, even if most clinicians and cardiovascular pathologists lack experience with detailed examination of the cardiac conduction system and fail to recognize lesions that are crucial to explain the SCD itself. In this review, we sought to describe the advances over the last 25 years in the study of the anatomopathological changes of the conducting tissue, in SCD, in mature hearts and particularly in sudden infant death syndrome (SIDS) and sudden intrauterine unexpected death syndrome (SIUDS), through the articles published in our journal Cardiovascular Pathology (CVP). We carried out an extensive Medline search to retrieve and review all articles published in CVP in which the sudden unexpected death of one or more subjects believed healthy was reported, especially if associated with lesions of the conducting tissue in settings that revealed no other explained causes of death, particularly in infants and fetuses. The cardiac conduction findings of resorptive degeneration, His bundle dispersion, Mahaim fibers, cartilaginous meta-hyperplasia, persistent fetal dispersion, left-sided His bundle, septation of the bifurcation, atrioventricular node dispersion, sinus node hypoplasia, Zahn node, His bundle hypoplasia, atrioventricular node, and His bundle dualism were similarly detected in SIDS and SIUDS victims.