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Long QT syndrome in children
1Department of Pediatrics, National Cheng Kung University Hospital, Tainan, Taiwan, R.O.C. jingming@mail.ncku.edu.tw
Insights
Long QT syndrome in children can lead to serious events like seizures and sudden death. Early diagnosis and beta-blocker treatment show promise for managing this condition, though some patients experience recurrent symptoms.
Area of Science:
- Pediatric Cardiology
- Clinical Electrophysiology
- Genetics
Background:
- Long QT syndrome (LQTS) is a cardiac channelopathy associated with risk of syncope, seizures, and sudden cardiac death.
- Pediatric LQTS presents unique diagnostic and management challenges due to varying clinical manifestations and potential for severe outcomes.
Purpose of the Study:
- To describe the clinical characteristics, electrocardiographic findings, and outcomes of 11 children diagnosed with Long QT syndrome.
- To evaluate the effectiveness of beta-blocker therapy in managing pediatric LQTS.
Main Methods:
- Retrospective review of 11 pediatric patients diagnosed with LQTS between June 1990 and June 1996.
- Analysis of clinical presentation, corrected QT intervals (QTc), electrocardiogram (ECG) abnormalities, and treatment responses.
- Follow-up assessment of symptom recurrence and mortality.
Main Results:
- The study included 11 children (7 males, 4 females) aged 1 day to 13 years with QTc intervals ranging from 0.46-0.59 sec.
- Presenting symptoms included seizures (6), syncope (3), and sudden death (2). ECG abnormalities noted were torsades de pointes (7) and sinus bradycardia (4).
- After treatment with beta-blockers, 6 patients were symptom-free, 2 had recurrent syncope, and 1 died from ventricular tachycardia during a 0.5-6 year follow-up.
Conclusions:
- Long QT syndrome in children presents with diverse symptoms and ECG findings, including life-threatening arrhythmias.
- Beta-blocker therapy is a cornerstone in managing pediatric LQTS, leading to symptom resolution in a majority of cases.
- Despite treatment, a subset of pediatric LQTS patients remain at risk for recurrent syncope and mortality, necessitating ongoing monitoring.
Abstract:
Long QT syndrome is a disease that can cause syncope, seizures and sudden death. From June 1990 to June 1996, 11 children (male/female: 714; ages: 1 day-13 years with a median of 5.4 years) from different families were found to have long QT syndrome. Their corrected QT intervals (QTc) were 0.46-0.59 sec (median: 0.53 sec). All patients had normal hearing. One patient had Marfan syndrome with mitral valve prolapse. The presentation symptoms were: sudden death (2), seizures (6) and syncope (3). Their electrocardiogram abnormalities included: torsades de pointes (7), sinus bradycardia (4), T wave abnormalities (4), monomorphic ventricular tachycardia (2) and congenital complete atrioventricular block (1). All patients were treated with beta-blockers and one had pacemaker implantation. In a follow-up period of 0.5-6 years, 6 were symptom free, 1 died of ventricular tachycardia and 2 had recurrent syncope.