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The long QT syndrome and seizures in childhood
1Department of Cardiology, Royal Children's Hospital, Monash Medical Centre, Melbourne, Victoria, Australia. davisa@cryptic.rch.unimelb.edu.au
Insights
Long QT syndrome (LQTS) in children can cause dangerous arrhythmias, often misdiagnosed as seizures or fainting. Early diagnosis and treatment significantly improve survival rates for these children.
Area of Science:
- Pediatric Cardiology
- Clinical Electrophysiology
- Genetics
Background:
- Long QT syndrome (LQTS) predisposes children to life-threatening ventricular arrhythmias.
- Arrhythmias in LQTS can manifest as syncope or seizures, frequently misdiagnosed.
- Untreated symptomatic LQTS carries a high mortality rate.
Purpose of the Study:
- To raise pediatrician awareness of LQTS presentations in children.
- To highlight the importance of recognizing LQTS to prevent misdiagnosis.
- To provide recommendations for screening for LQTS in pediatric patients.
Main Methods:
- Review of clinical presentations of LQTS in children.
- Analysis of diagnostic challenges and misdiagnosis patterns.
- Formulation of screening recommendations for pediatricians.
Main Results:
- Symptomatic LQTS in children is associated with significant mortality without treatment.
- Effective therapies exist that substantially improve outcomes.
- Awareness of LQTS clinical features is crucial for timely diagnosis.
Conclusions:
- Pediatricians must be vigilant for LQTS presentations, differentiating them from seizure disorders or syncope.
- Prompt diagnosis and initiation of therapy dramatically reduce mortality in pediatric LQTS.
- Implementing screening protocols can improve early detection and management of LQTS.
Abstract:
Children with the long QT syndrome (LQTS) are prone to life threatening ventricular arrhythmias. These arrhythmias may result in syncope and seizures that are often attributed incorrectly to a seizure disorder or to common fainting. The untreated mortality for symptomatic children with the LQTS is high but is improved significantly with therapy. Paediatricians should be aware of the presentations of the syndrome. Recommendations for screening for the syndrome are given.