ABSTRACT
PURPOSE OF REVIEW: Is to summarise the new contributions toward the understanding of the broad spectrum of manifestations of Brugada syndrome (BrS) during the first years of life. The review encompasses the screening of the asymptomatic patient referred due to family history in one extreme of the spectrum, and also the rare child with early clinical expression of the disease on the opposite side. RECENT FINDINGS: Involve specific features of pediatric BrS including the risk related to a positive family history of sudden cardiac death, the risk of presenting with syncope and the multiple diagnostic challenges of the disease. We included some of the most controversial aspects of the diagnosis and risk stratification, encompassing noninvasive studies (Holter monitors, exercise test, implantable loop recorders, and provocative tests), as well as invasive stratification during the first years of life. Finally, the role and concerns of genetic testing in this age group are commented upon. SUMMARY: The main key to minimize overdiagnosis and overtreatment in the young population with a personal and/or family diagnosis of BrS is to perform a systematic but also individualized assessment. Appropriate diagnostic guidelines need to be created and age-specific risk stratification algorithms built for the young patient both with suspected and confirmed BrS.
Subject(s)
Brugada Syndrome , Brugada Syndrome/diagnosis , Brugada Syndrome/genetics , Brugada Syndrome/therapy , Child , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Electrocardiography , Humans , Overdiagnosis , Overtreatment , Risk AssessmentABSTRACT
BACKGROUND: Literature reports 5% of recurrence/failure in paediatric accessory pathway ablations. Our aim was to investigate the reasons underlying this finding and share techniques to obtain long-term success. METHODS: Thirty-nine paediatric patients referred for a repeat procedure were analysed: characteristics of the pathways and the initial and redo procedures were identified. RESULTS: Mean age was 11.9 ± 3.3 years (59% males). Three patients (8%) had multiple accessory pathways. The most frequent location was left lateral (26%). Left sided pathway recurrence was caused mainly by poor contact (60%) and inadequate mapping (40%). For right lateral accessory pathways, poor contact accounted for 70% of failures. For antero-septal and para-Hisian locations, the use of cryoablation and choice of low radiofrequency energy delivery accounted for > 75% of failures. Long-term success strategies included choice of contact force catheters and radiofrequency applications at the ventricular insertion of the pathway and in the aortic coronary cusps. In postero-septal substrates, the main reason accounting for failure was deep or epicardial location of the pathway (37%), solved by using an irrigated tip catheter or applying lesions within the coronary sinus, or applications from both right and left postero-septal areas. CONCLUSION: Acute failure and post-procedure recurrence in paediatric accessory pathway ablations have multiple reasons related to the characteristics of the pathway and the technology available. Accurate understanding of the anatomy, careful mapping and pacing manoeuvers, and incorporation of new technologies contribute to achieve a definitive success in > 98% of procedures.
