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Advance in the understanding of vasovagal syncope in children and adolescents. BACKGROUND: Vasovagal syncope (VVS) accounts for 60-80% of cases of neurally mediated syncope. VVS results from acute orthostatic intolerance and recurrent syncopal attacks, which can seriously affect an individual's quality of life. In addition, some children even experience trauma during attacks. Therefore, it is particularly important to clarify the pathogenesis of VVS. The aim of our study is to reveal the latest research progress of VVS. DATA SOURCES: Literature that involved the pathogenesis of VVS were selected from Cochrane Library (1990-2019), EMBASE (1991-2019) and PubMed (1968-2019) databases. RESULTS: Hypovolemia, autonomic dysfunction, vasomotor dysfunction, baroreceptor reflex abnormalities, endothelial dysfunction, serotonin surges, and gut microbiota were involved in the underlying mechanism of VVS. CONCLUSIONS: VVS is not always a benign prognosis. Various aspects were involved in its pathogenesis. Bezold-Jarish reflex, dysfunction of the autonomic nervous system, genetic factors and so on played important roles in VVS; however, the mechanism remains unclear.