Jennifer Hendry1, Valentina Giudici2, A Spanaki1, Sarah Mead-Reagan1, Ella Field1, Dominic Abrams3, Martin Lowe1, Kaski Juan Pablo4.
1Inherited Cardiovascular Diseases Unit,Department of Cardiology, Great Ormond Street Hospital, London, UK,
2Institute of Cardiovascular Science, University College London, UK,
3Department of Cardiology, Boston Children’s Hospital, Boston MA, USA,
4Department of Paediatrics, Ospedale Luigi Sacco, University of Milan, Italy. | | Abstract | Address for Correspondence: Jennifer Hendry, University of Edinburgh. Email: s1103537@sms.ed.ac.uk
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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DOI No: https://doi.org/10.7199/ped.oncall.2014.5S
| Cite this article as: | | Hendry J, Giudici V, Spanaki A, Mead-Reagan S, Field E, Abrams D, Lowe M, Pablo K J. Sudden arrhythmic death syndrome (SADS): Diagnostic yield of comprehensive clinical evaluation of paediatric first-degree relatives. Pediatr Oncall J. 2014;11. doi: 10.7199/ped.oncall.2014.5S |
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