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- W3204172065 abstract "<h3>Background</h3> The congenital long QT syndrome is a disorder characterized by Q-T interval prolongation on electrocardiogram that predisposes the heart to ventricular tachycardia and fibrillation. Children with long QT are prone to life threatening ventricular arrhythmia. The condition may remain unrecognized for a long time in children who present with recurrent episodes of syncope and seizures. We report a case of congenital Long QT Syndrome being thought to have vasovagal syncope and discuss the importance of having conversations about how early recognition of this condition could be promoted. <h3>Objectives</h3> To study the clinical profile of a patient with long QT syndrome presenting as seizures and syncope. We aim to evaluate the patient with genetically confirmed long QT syndrome to establish the frequency of delayed recognition. We aim to raise the awareness of the possibility of Long QT syndrome in cases of unexplained recurrent syncope with seizures. <h3>Methods</h3> We report a 9 year old girl who presented since 2 years of age with history of intermittent syncope and seizures. The girl had multiple presentations to ED and one presentation to the paediatric ward with either syncopal episodes or short seizure like episodes secondary to painful stimuli which were thought to be vasovagal. She did have previous ECGs which were reported as being normal however retrospective analysis of one of the available ECG showed a QTc of 463 milliseconds. She had background of PUJ obstruction and came to the hospital last year for DMSA (dimercaptosuccinic acid scan). During the cannulation she had a syncopal episode with quick recovery. And an ECG done showed bradycardia with a QTc of 460 milliseconds. She was referred to the inherited cardiac care team and further genetic analysis confirmed a pathogenic KCNQ1 mutation (long QT1). Her mother (who was suffering with seizures) and brother were also diagnosed with long QT syndrome. She was commenced on Nadolol and is currently stable and doing well. <h3>Results</h3> There are several case reports of unrecognised long QT syndrome in literature and this case is another example of the same. The case illustrates that more needs to be done by the PEC (Paediatricians with expertise in cardiology) and Paediatric cardiologists groups to ensure these are recognised early to avoid morbidity and mortality. An index case often results in diagnosis of other members in the family so has significant preventative implications for avoiding sudden cardiac arrests. Suggestions would include more education for colleagues in Emergency department, Primary care and general paediatricians about manual QTc calculations and pertinent family history, all children with syncope having ECGs vetted by PECs and more discussions in specialist group committees. <h3>Conclusions</h3> The presence of a prolonged QT interval and sinus bradycardia along with documented ventricular tachycardia during the ‘seizures’ confirms the diagnosis of the Long QT syndrome. This possibility should be considered by paediatricians who see a child with seizures or recurrent syncope. This experience emphasizes the importance of obtaining an electrocardiogram in all children with syncope or seizure disorder of unknown origin." @default.
- W3204172065 created "2021-10-11" @default.
- W3204172065 creator A5060996184 @default.
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- W3204172065 date "2021-09-30" @default.
- W3204172065 modified "2023-10-14" @default.
- W3204172065 title "1447 Unrecognised long QTc syndrome- can we do more?" @default.
- W3204172065 doi "https://doi.org/10.1136/archdischild-2021-rcpch.648" @default.
- W3204172065 hasPublicationYear "2021" @default.
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