Jennifer Hendry*, Valentina Giudici**, A Spanaki*, Sarah Mead-Reagan*, Ella Field*, Dominic Abrams ***, Martin Lowe*, Kaski Juan Pablo****
*Inherited Cardiovascular Diseases Unit, Department of Cardiology, Great Ormond Street Hospital, London, UK, **Institute of Cardiovascular Science, University College London, UK, ***Department of Cardiology, Boston Childrens Hospital, Boston MA, USA, **Department of Paediatrics, Ospedale Luigi Sacco, University of Milan, Italy
|Address for Correspondence: Jennifer Hendry, University of Edinburgh. Email: email@example.com
Background: Sudden arrhythmic death syndrome (SADS) describes the sudden death of a previously healthy individual, with no cause identified on post-mortem. A large proportion of SADS cases are thought to be attributable to inherited cardiac disease. Previous studies of adult populations, have identified evidence of a heritable cardiac condition in up to 53% of families, however, prevalence data within paediatrics is more limited. The aim of this study was to determine the yield of extensive clinical screening in children attending Great Ormond Street Hospital, referred due to a history of SADS or aborted cardiac arrest (ACA) in a first-degree relative.
Methods: Retrospective evaluation of children attending family screening after sudden cardiac death or ACA in a family member was performed and strict inclusion criteria employed. Comprehensive assessment of patients included clinical examination, family history, electrocardiogram, echocardiogram, 24-hour tape and signal averaged electrocardiogram; older children also underwent exercise testing, cardiac MRI and ajmaline-provocation test.
Results: The study included 110 children in total from 63 families. In 10 children from 9 families, an inherited cardiac disease was diagnosed (14.3%). Specifically, 7 patients were diagnosed with Brugada Syndrome, 2 with Long QT Syndrome and 1 with catecholinergic Polymorphic Ventricular Tachycardia.
Conclusion: The results demonstrate a high prevalence of heritable cardiovascular disease after screening of children at risk due to a history of SADS in first-degree relatives. This emphasises the importance of ongoing screening in this population, to ensure early diagnosis, appropriate management and prevention of sudden cardiac death.
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|Hendry J, Giudici V, Spanaki A, Mead-Reagan S, Field E, Abrams D, Lowe M, Pablo J K..Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=8611&type=C&tid=0&imgid=0&reportid=811&tbltype=|