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Prolonged QT syndrome in children: an uncommon but potentially fatal entity
Marla J Friedman1, Colette C Mull, Ghazala Q Sharieff
1Division of Emergency Medicine, A.I. duPont Hospital for Children, Wilmington, Delaware 19899, USA.
Insights
Long QT syndrome (LQTS) poses risks like syncope and sudden death in children. Early recognition through history and ECG, followed by family screening and treatment, can prevent fatal outcomes.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Prolonged QT syndrome (LQTS) is a cardiac disorder with congenital or acquired causes.
- Affected children face risks including syncope, seizures, arrhythmias, and sudden cardiac death.
- LQTS encompasses genetic conditions like Jervell and Lange-Nielsen and Romano-Ward syndromes.
Purpose of the Study:
- To emphasize the importance of considering LQTS in pediatric patients presenting with syncope.
- To highlight key diagnostic elements including personal and family history.
- To underscore the necessity of electrocardiogram evaluation and family-wide screening.
Main Methods:
- Clinical assessment focusing on patient history, including syncopal episodes, exercise-induced symptoms, and family history of sudden death or deafness.
- Electrocardiogram (ECG) with manual QT interval calculation for patients with suggestive symptoms.
- Family member evaluation upon diagnosis of LQTS.
Main Results:
- Syncope, especially recurrent or exercise-induced, is a critical indicator for LQTS.
- A detailed personal and family history is crucial for identifying at-risk individuals.
- ECG findings and family screening are essential for diagnosis and management.
Conclusions:
- Prompt recognition of LQTS in children presenting with syncope is vital.
- Comprehensive evaluation, including ECG and family history, aids diagnosis.
- Timely treatment and family screening can mitigate the life-threatening risks associated with LQTS.
Abstract:
Prolonged QT syndrome may be either congenital, as in Jervell and Lange-Nielsen or Romano-Ward syndromes, or acquired in nature. Affected children are at risk for syncope, seizures, dysrhythmias and sudden death. Physicians should consider long QT syndrome (LQTS) in all patients who present with syncope. A thorough personal and family history should be documented, with particular attention to prior syncopal episodes, congenital deafness, and unexplained sudden death. Syncope that is either recurrent or induced by exercise or stress is concerning and also should be noted. An electrocardiogram with manual calculation of the QT interval should be performed on all patients with a suggestive history. Furthermore, the diagnosis of LQTS warrants evaluation of all other family members. With recognition and appropriate treatment of affected patients, the potentially fatal consequences of LQTS may be prevented.